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1. Mood Disorder Questionnaire Positivity in Systemic Lupus Erythematosus and Other Chronic Diseases including Screen Bipolar Disorders or Rhythm and Energy Dysregulation Syndromes (DYMERS)

Author

Primavera, D, Fornaro, M, Carra, G, Romano, F, Gonzales, C, Preti, A, Sancassiani, F, Cossu, G, Nardi, A, Scano, A, Orru, G, Chessa, E, Floris, A, Piga, M, Cauli, A, Carta, M, Primavera D., Fornaro M., Carra G., Romano F., Gonzales C. I. A., Preti A., Sancassiani F., Cossu G., Nardi A. E., Scano A., Orru G., Chessa E., Floris A., Piga M., Cauli A., Carta M. G., Primavera, D, Fornaro, M, Carra, G, Romano, F, Gonzales, C, Preti, A, Sancassiani, F, Cossu, G, Nardi, A, Scano, A, Orru, G, Chessa, E, Floris, A, Piga, M, Cauli, A, Carta, M, Primavera D., Fornaro M., Carra G., Romano F., Gonzales C. I. A., Preti A., Sancassiani F., Cossu G., Nardi A. E., Scano A., Orru G., Chessa E., Floris A., Piga M., Cauli A., and Carta M. G.

Abstract

Introduction: This study explores the issue of paper-and-pencil screening tests for bipolar disorder, often leading to false positives. It discusses hypotheses that connect MDQ positivity with sleep disorders, a decline in health-related quality of life, and the impact of the COVID-19 pandemic on mood disorders. The study proposes that MDQ identifies a "Dysregulation of Mood, Energy, and Social Rhythms Syndrome" (DYMERS), indicating a stress-related condition. It aims to investigate the association between MDQ positivity and systemic lupus erythematosus (SLE) in comparison to other chronic disorders. Methods: This case-control study, conducted from April 2019 to February 2020, investigated MDQ positivity in patients with SLE. Ethical approvals were obtained, and statistical analysis was used for data assessment. Results: This is a case-controlled study where MDQ positivity was significantly higher in systemic lupus erythematosus cases than controls. The analysis compared gender, age, and the presence of depressive episodes between MDQ-positive and MDQ-negative cases, revealing some differences but no significant variations. Interestingly, no association with high prednisone or biologics use was observed. The frequency of MDQ positivity in systemic lupus erythematosus was compared to other chronic pathologies, revealing varying associations with each condition. Conclusion: This study reveals a high rate of (MDQ) positivity in systemic lupus erythematosus (SLE), associated with the risk of bipolar disorder in SLE. Notable discrepancies in MDQ positivity risk factors between SLE and bipolar disorder are observed. The study emphasizes the ability of MDQ to identify a distinct syndrome characterized by rhythm dysregulation, posing a risk for bipolar disorder and other disorders.

Published
2024

2. Gene Expression Profiling of Monozygotic Twins Affected by Psoriatic Arthritis

Author

Angioni MM, Floris A, Cangemi I, Congia M, Chessa E, Orrù S, Piga M, and Cauli A

Subjects
psoriatic arthritis, gene expression profiling, diseases in twin, genetic, environment-gene interaction, joint erosions, Diseases of the musculoskeletal system, RC925-935
Abstract

Maria Maddalena Angioni,1 Alberto Floris,1 Ignazio Cangemi,1 Mattia Congia,1 Elisabetta Chessa,1 Sandro Orrù,2 Matteo Piga,1 Alberto Cauli1 1Rheumatology Unit, Department of Medical Sciences and Public Health, University of Cagliari, University Clinic AOU, Cagliari, 09042, Italy; 2Medical Genetics, Department of Medical Sciences and Public Health, University of Cagliari, Cagliari, 09042, ItalyCorrespondence: Maria Maddalena AngioniRheumatology Unit, Department of Medical Sciences and Public Health, University of Cagliari, University Clinic AOU Cagliari, S.S. 554, Bivio per Sestu, Monserrato, Cagliari, 09042, ItalyTel +39 07051093340Email m.maddalena.angioni@gmail.comIntroduction: Psoriatic Arthritis (PsA) is a multifactorial disease, where the relative burden of genetic, epigenetic and environmental factors in clinical course and damage accrual is not yet definitively clarified. In clinical practice, there is a real need for useful candidate biomarkers in PsA diagnosis and disease progression, by exploring its underlying transcriptomic and epigenomic mechanisms. This work aims to profile the transcriptome in monozygotic (MZ) twins with psoriatic arthritis (PsA) highly concordant for clinical presentation, but discordant for the radiographic outcomes’ severity.Methods: We describe i) the clinical case of two MZ twins; ii) their comparative gene expression profiling (HTA 2.0 Affymetrix) and iii) signal pathways and pathophysiological processes in which differentially expressed genes are involved (in silico analysis by the IPA software, QIAGEN).Results: One hundred sixty-three transcripts and 36 coding genes (28 up and 8 down) were differentially expressed between twins, and in the brother with the most erosive form, the transcriptomic profiling highlights the overexpression of genes known to be involved in immunomodulatory processes and on a broad spectrum of PsA manifestations.Discussion: Twins’ clinical cases are still a gold mine in medical research: twin brothers are ideal experimental models in estimating the relative importance of genetic versus nongenetic components as determinants of complex phenotypes, non-Mendelian and multifactorial diseases as PsA.Keywords: psoriatic arthritis, gene expression profiling, diseases in twin, genetic, environment–gene interaction, joint erosions

Published
2021

3. Similarities and differences between younger and older disease onset patients with newly diagnosed systemic lupus erythematosus

Author

Prevete, I, Iuliano, A, Cauli, A, Piga, M, Iannone, F, Coladonato, L, Bortoluzzi, A, Silvagni, E, Tani, C, Elefante, E, Doria, A, Iaccarino, L, Franceschini, F, Fredi, M, Conti, F, Spinelli, F, Frediani, B, Garcia, E, Scire, C, Zanetti, A, Rozza, D, Carrara, G, Sebastiani, G, Prevete I., Iuliano A., Cauli A., Piga M., Iannone F., Coladonato L., Bortoluzzi A., Silvagni E., Tani C., Elefante E., Doria A., Iaccarino L., Franceschini F., Fredi M., Conti F., Spinelli F. R., Frediani B., Garcia E. G., Scire C. A., Zanetti A., Rozza D., Carrara G., Sebastiani G. D., Prevete, I, Iuliano, A, Cauli, A, Piga, M, Iannone, F, Coladonato, L, Bortoluzzi, A, Silvagni, E, Tani, C, Elefante, E, Doria, A, Iaccarino, L, Franceschini, F, Fredi, M, Conti, F, Spinelli, F, Frediani, B, Garcia, E, Scire, C, Zanetti, A, Rozza, D, Carrara, G, Sebastiani, G, Prevete I., Iuliano A., Cauli A., Piga M., Iannone F., Coladonato L., Bortoluzzi A., Silvagni E., Tani C., Elefante E., Doria A., Iaccarino L., Franceschini F., Fredi M., Conti F., Spinelli F. R., Frediani B., Garcia E. G., Scire C. A., Zanetti A., Rozza D., Carrara G., and Sebastiani G. D.

Abstract

Objective Several studies show that age at onset has an impact on the clinical-serological presentation, comorbidities and disease course of patients with systemic lupus erythematosus (SLE). We evaluated whether, in patients with recent onset SLE, the age at onset correlates with clinical-serological manifestations and with comorbidities. Methods We analysed 171 patients with a SLE diagnosis obtained within 12 months of diagnosis enrolled in the Early Lupus project. Based on the age of onset of the first disease symptom, they were stratified into 2 groups: early onset (18–45 years) and late onset (>45 years). The analysis was replicated by stratifying patients based on age at diagnosis (fulfillment of ACR classification criteria). Each comparison was made at baseline and at 36 months of follow-up. Results Baseline: patients with late onset displayed comorbidities (hypertension, dyslipidaemia and osteoporosis) more frequently than early onset group. 11.4% of late onset patients had a malignancy in medical history, not recorded in the early onset cohort. The two groups differed neither in organ involvement (domain BILAG) nor in disease activity (ECLAM). Patients with early onset showed a disease with signs of higher serologic activity (higher frequency of anti-dsDNA positivity and lower mean C3 and C4 levels) and had malar rash more frequently than the late onset group (36.2% vs. 18.2%, p=0.042). Similar results were obtained by stratifying patients by age of diagnosis (18-45 years and >45 years), except for the higher frequency of discoid rash in the group with age at diagnosis >45 years (18% vs. 6.6%, p=0.045). 36 months: the 2 groups of patients independently of the stratification applied did not differ in the accumulation of damage, but showed a different pattern of 8 organ involvement. Musculoskeletal involvement was more frequent both in the late onset group (18.6% vs. 7.3%, p=0.043) and in the group with age at diagnosis >45 years (20.4% vs. 5.9%, p

Published
2023

4. Musculoskeletal ultrasound may narrow the gap between patients and physicians in the assessment of rheumatoid arthritis disease activity

Author

Floris, A, Rozza, D, Zanetti, A, Carrara, G, Bellis, E, Cauli, A, Iagnocco, A, Scire, C, Piga, M, Batticciotto, A, Bortoluzzi, A, Gabba, A, Gattamelata, A, Adinolfi, A, Raffeiner, B, Vinghitti, C, Mastaglio, C, Scioscia, C, Russi, D, Luccioli, F, Cavatorta, F, Ceccarelli, F, Sakellariou, G, Filippou, G, Cagnotto, G, Farina, I, Menza, L, Idolazzi, L, Canzoni, M, Massarotti, M, Focherini, M, Caprioli, M, Gutierrez, M, Draghessi, A, Muratore, M, De Lucia, O, Rossini, P, Macchioni, P, Ramonda, R, Rossi, S, Parisi, S, Sabatino, V, Picerno, V, Floris A., Rozza D., Zanetti A., Carrara G., Bellis E., Cauli A., Iagnocco A., Scire C. A., Piga M., Batticciotto A., Bortoluzzi A., Gabba A., Gattamelata A., Adinolfi A., Raffeiner B., Vinghitti C., Mastaglio C., Scioscia C., Russi D., Luccioli F., Cavatorta F., Ceccarelli F., Sakellariou G., Filippou G., Cagnotto G., Farina I., Menza L., Idolazzi L., Canzoni M., Massarotti M., Focherini M., Caprioli M., Gutierrez M., Draghessi A., Muratore M., De Lucia O., Rossini P., MacChioni P., Ramonda R., Rossi S., Parisi S., Sabatino V. D., Picerno V., Floris, A, Rozza, D, Zanetti, A, Carrara, G, Bellis, E, Cauli, A, Iagnocco, A, Scire, C, Piga, M, Batticciotto, A, Bortoluzzi, A, Gabba, A, Gattamelata, A, Adinolfi, A, Raffeiner, B, Vinghitti, C, Mastaglio, C, Scioscia, C, Russi, D, Luccioli, F, Cavatorta, F, Ceccarelli, F, Sakellariou, G, Filippou, G, Cagnotto, G, Farina, I, Menza, L, Idolazzi, L, Canzoni, M, Massarotti, M, Focherini, M, Caprioli, M, Gutierrez, M, Draghessi, A, Muratore, M, De Lucia, O, Rossini, P, Macchioni, P, Ramonda, R, Rossi, S, Parisi, S, Sabatino, V, Picerno, V, Floris A., Rozza D., Zanetti A., Carrara G., Bellis E., Cauli A., Iagnocco A., Scire C. A., Piga M., Batticciotto A., Bortoluzzi A., Gabba A., Gattamelata A., Adinolfi A., Raffeiner B., Vinghitti C., Mastaglio C., Scioscia C., Russi D., Luccioli F., Cavatorta F., Ceccarelli F., Sakellariou G., Filippou G., Cagnotto G., Farina I., Menza L., Idolazzi L., Canzoni M., Massarotti M., Focherini M., Caprioli M., Gutierrez M., Draghessi A., Muratore M., De Lucia O., Rossini P., MacChioni P., Ramonda R., Rossi S., Parisi S., Sabatino V. D., and Picerno V.

Abstract

Objectives: To investigate the association between patient-physician discordance in the assessment of disease activity and residual US synovitis/tenosynovitis in a cohort of patients with RA in clinical remission. Methods: A post hoc analysis of the STARTER study, promoted by the Musculoskeletal-US (MSUS) Study Group of the Italian Society for Rheumatology, was performed using data from 361 consecutive patients with RA in clinical remission. The global assessment of disease activity by each patient (PGA) and evaluator/physician (EGA) was recorded on a 100-mm visual analogue scale. The PGA-EGA discordance was classified as positive (PGA>EGA) or negative (PGA

Published
2023

5. Relationship between the prevalence of subclinical tenosynovitis and treatment in patients with RA in clinical remission: STARTER study

Author

Parisi, S, Zanetti, A, Carrara, G, Scire, C, Iagnocco, A, Filippou, G, Batticciotto, A, Floris, A, Bortoluzzi, A, Gabba, A, Gattamelata, A, Adinolfi, A, Raffeiner, B, Venditti, C, Mastaglio, C, Scioscia, C, Rossi, D, Bellis, E, Luccioli, F, Cavatorta, F, Ceccarelli, F, Sakellariou, G, Cagnotto, G, Farina, I, Menza, L, Idolazzi, L, Canzoni, M, Massarotti, M, Focherini, M, Caprioli, M, Gutierrez, M, Draghessi, A, Piga, M, Muratore, M, De Lucia, O, Rossini, P, Macchioni, P, Ramonda, R, Rossi, S, Di Sabatino, V, Picerno, V, Parisi S., Zanetti A., Carrara G., Scire C. A., Iagnocco A., Filippou G., Batticciotto A., Floris A., Bortoluzzi A., Gabba A., Gattamelata A., Adinolfi A., Raffeiner B., Venditti C., Mastaglio C., Scioscia C., Rossi D., Bellis E., Luccioli F., Cavatorta F. P., Ceccarelli F., Sakellariou G., Cagnotto G., Farina I., Menza L., Idolazzi L., Canzoni M., Massarotti M., Focherini M. C., Caprioli M., Gutierrez M., Draghessi A., Piga M., Muratore M., De Lucia O., Rossini P., MacChioni P., Ramonda R., Rossi S., Di Sabatino V., Picerno V., Parisi, S, Zanetti, A, Carrara, G, Scire, C, Iagnocco, A, Filippou, G, Batticciotto, A, Floris, A, Bortoluzzi, A, Gabba, A, Gattamelata, A, Adinolfi, A, Raffeiner, B, Venditti, C, Mastaglio, C, Scioscia, C, Rossi, D, Bellis, E, Luccioli, F, Cavatorta, F, Ceccarelli, F, Sakellariou, G, Cagnotto, G, Farina, I, Menza, L, Idolazzi, L, Canzoni, M, Massarotti, M, Focherini, M, Caprioli, M, Gutierrez, M, Draghessi, A, Piga, M, Muratore, M, De Lucia, O, Rossini, P, Macchioni, P, Ramonda, R, Rossi, S, Di Sabatino, V, Picerno, V, Parisi S., Zanetti A., Carrara G., Scire C. A., Iagnocco A., Filippou G., Batticciotto A., Floris A., Bortoluzzi A., Gabba A., Gattamelata A., Adinolfi A., Raffeiner B., Venditti C., Mastaglio C., Scioscia C., Rossi D., Bellis E., Luccioli F., Cavatorta F. P., Ceccarelli F., Sakellariou G., Cagnotto G., Farina I., Menza L., Idolazzi L., Canzoni M., Massarotti M., Focherini M. C., Caprioli M., Gutierrez M., Draghessi A., Piga M., Muratore M., De Lucia O., Rossini P., MacChioni P., Ramonda R., Rossi S., Di Sabatino V., and Picerno V.

Abstract

Objective: This study is a sub-analysis from the patient cohort of the STARTER (Sonographic Tenosynovitis Assessment in RheumaToid arthritis patiEnts in Remission) study. The aim was to evaluate differences in ultrasound-detected joint and/or tendon involvement between patients receiving therapies based on a combination of conventional synthetic DMARDs (csDMARDs) and biologic DMARDs (bDMARDs) and those who were treated with either csDMARDs or bDMARDs in monotherapy. Material and methods: Four hundred and twenty-seven consecutive patients with a diagnosis of RA were recruited between October 2013 and June 2014. They were divided into three subgroups based on their therapy at baseline: patients with bDMARD in monotherapy, patients with csDMARD in monotherapy and patients in combination therapy (csDMARD + bDMARD). At baseline, 6 months and 12 months, a clinical examination (28 joint count) and an ultrasound evaluation were performed in each patient. A score of grey-scale (GS) and power Doppler (PD) synovitis and tenosynovitis was calculated based on the OMERACT scoring systems. Results: Two hundred and fifty-six patients completed the observation period: 48 patients from the bDMARD group (18.75%), 152 patients from the csDMARD group (59.38%) and 56 patients from csDMARD + bDMARD group (21.88%). The analysis showed that GS tenosynovitis and PD tenosynovitis are better controlled in combination therapy than they are ith csDMARD alone (P=0.025 and P=0.047, respectively); for PD synovitis, there was a better response in those who were treated with the combination therapy when compared with the patients receiving csDMARD (P=0.01) or bDMARD (P=0.02) alone. Conclusions: The analysis showed a lower prevalence of subclinical inflammatory manifestations detected with ultrasound imaging in those patients treated with the combination therapy than in those in monotherapy.

Published
2023

6. Assessment Of Circulating Endothelial Cells And Their Progenitors As Potential Biomarkers Of Disease Activity And Damage Accrual In Behçet’s Syndrome

Author

Floris A, Piga M, Pinna S, Angioni MM, Congia M, Mascia P, Chessa E, Cangemi I, Mathieu A, and Cauli A

Subjects
Circulating Endothelial Cells (CECs), Endothelial Progenitor Cells (EPCs), Behçet’s Syndrome., Diseases of the musculoskeletal system, RC925-935
Abstract

Alberto Floris, Matteo Piga, Silvia Pinna, Maria Maddalena Angioni, Mattia Congia, Piero Mascia, Elisabetta Chessa, Ignazio Cangemi, Alessandro Mathieu, Alberto Cauli Rheumatology Unit, AOU University Clinic and University of Cagliari, Cagliari, ItalyCorrespondence: Matteo PigaRheumatology Unit, University Clinic AOU of Cagliari, SS 554, Monserrato, CA 09042, ItalyTel +390706754069Fax +39070513157Email matteopiga@unica.itPurpose: To explore the potential role of circulating endothelial cells (CECs) and their progenitors (EPCs) as biomarkers of disease activity and damage accrual in patients with Behçet’s syndrome (BS), by using a standardised and reliable flow cytometry protocol.Patients and methods: CECs and EPCs were assessed in 32 BS patients and 11 gender/age/smoking habits matched healthy controls (HC). They were identified by flow cytometry as alive/nucleated/CD45-negative/CD34-bright/CD146-positive and alive/nucleated/CD45-negative/CD34-bright/CD309-positive events, respectively. In BS patients, demographic and clinical features, including disease activity (assessed by Behçet’s disease current disease activity form, BDCAF) and irreversible damage accrual (by the vasculitis damage index, VDI) were recorded. Uni- and multivariate analysis were performed to compare the CECs and EPCs concentrations in BS vs HC and to identify potential associations with demographic or clinical features.Results: The CECs concentration was significantly higher in the BS patients than HCs [median (IQR) 15.0 (7.5–23.0) vs 6.0 (2.0–13.0) CECs/mL, p=0.024]. In BS patients, no significant associations were found between CECs and demographic features, present and past clinical manifestations, BDCAF score and ongoing treatment. A significant association was observed between CECs and organ damage, as assessed by the VDI (rho 0.356, p=0.045). Higher levels of CECs were especially associated with vascular damage [median (IQR) 23.0 (14.0–47.0) vs 13.0 (6.0–19.0) CECs/mL, p=0.011], including arterial aneurysm and stenosis, complicated venous thrombosis, cerebrovascular accident. The concentration of EPCs did not significantly differ between the BS and HC [median 26.5 (13.0–46.0) vs 19.0 (4.0–42.0) EPCs/mL, p=0.316] and no significant associations were observed between their levels and any clinical characteristic.Conclusion: Our study suggests that the CECs concentration is significantly higher in BS than healthy subjects, and it mainly correlates with vascular damage. A longitudinal extension of the present study on a wider cohort would be useful to validate the potential role of CECs as a marker or, hopefully, predictor of vascular damage in BS.Keywords: circulating endothelial cells, CECs, endothelial progenitor cells, EPCs, Behçet’s syndrome

Published
2019

7. Musculoskeletal ultrasound may narrow the gap between patients and physicians in the assessment of rheumatoid arthritis disease activity

Author

Floris A., Rozza D., Zanetti A., Carrara G., Bellis E., Cauli A., Iagnocco A., Scire C. A., Piga M., Batticciotto A., Bortoluzzi A., Gabba A., Gattamelata A., Adinolfi A., Raffeiner B., Vinghitti C., Mastaglio C., Scioscia C., Russi D., Luccioli F., Cavatorta F., Ceccarelli F., Sakellariou G., Filippou G., Cagnotto G., Farina I., Menza L., Idolazzi L., Canzoni M., Massarotti M., Focherini M., Caprioli M., Gutierrez M., Draghessi A., Muratore M., De Lucia O., Rossini P., MacChioni P., Ramonda R., Rossi S., Parisi S., Sabatino V. D., Picerno V., Floris, A, Rozza, D, Zanetti, A, Carrara, G, Bellis, E, Cauli, A, Iagnocco, A, Scire, C, Piga, M, Batticciotto, A, Bortoluzzi, A, Gabba, A, Gattamelata, A, Adinolfi, A, Raffeiner, B, Vinghitti, C, Mastaglio, C, Scioscia, C, Russi, D, Luccioli, F, Cavatorta, F, Ceccarelli, F, Sakellariou, G, Filippou, G, Cagnotto, G, Farina, I, Menza, L, Idolazzi, L, Canzoni, M, Massarotti, M, Focherini, M, Caprioli, M, Gutierrez, M, Draghessi, A, Muratore, M, De Lucia, O, Rossini, P, Macchioni, P, Ramonda, R, Rossi, S, Parisi, S, Sabatino, V, and Picerno, V

Subjects
rheumatoid arthritis, remission, Rheumatology, patient–physician discordance, patient global assessment, Pharmacology (medical), patient-physician discordance, ultrasonography, RA
Abstract

Objectives To investigate the association between patient–physician discordance in the assessment of disease activity and residual US synovitis/tenosynovitis in a cohort of patients with RA in clinical remission. Methods A post hoc analysis of the STARTER study, promoted by the Musculoskeletal-US (MSUS) Study Group of the Italian Society for Rheumatology, was performed using data from 361 consecutive patients with RA in clinical remission. The global assessment of disease activity by each patient (PGA) and evaluator/physician (EGA) was recorded on a 100-mm visual analogue scale. The PGA-EGA discordance was classified as positive (PGA>EGA) or negative (PGA Results The mean (s.d.) PGA and EGA scores were 6.1 (7.1) and 8.8 (12) mm, respectively, with a median (IQR) absolute difference of 4 (0–10) mm. Positive and negative discordances were recorded in 39 (10.8%) and 65(18.0%) patients, respectively. The GS-S (adjOR 1.099) and PD-S (adjOR 1.167) scores were associated with positive discordance (P Conclusions Patient–physician discordance is associated with the lack of US remission in patients with RA and may represent a further indication for MSUS.

Published
2022

8. The AutoInflammatory Diseases Alliance Registry of monogenic autoinflammatory diseases

Author

Gaggiano, C, Vitale, A, Tufan, A, Ragab, G, Aragona, E, Wiesik-Szewczyk, E, Ait-Idir, D, Conti, G, Iezzi, L, Maggio, Mc, Cattalini, M, La Torre, F, Lopalco, G, Verrecchia, E, de Paulis, A, Sahin, A, Insalaco, A, Sfikakis, Pp, Marino, A, Frassi, M, Ogunjimi, B, Opris-Belinski, D, Parronchi, P, Emmi, G, Shahram, F, Ciccia, F, Piga, M, Hernández-Rodríguez, J, Pereira, Rmr, Alessio, M, Naddei, R, Olivieri, An, Giudice, Ed, Sfriso, P, Ruscitti, P, Gobbi, Fl, Kucuk, H, Sota, J, Hussein, Ma, Malizia, G, Jahnz-Różyk, K, Sari-Hamidou, R, Romeo, M, Ricci, F, Cardinale, F, Iannone, F, Casa, Fd, Natale, Mf, Laskari, K, Giani, T, Franceschini, F, Sabato, V, Yildirim, D, Caggiano, V, Hegazy, Mt, Marzo, Rd, Kucharczyk, A, Khellaf, G, Tarsia, M, Almaghlouth, Ia, Laymouna, Ah, Mastrorilli, V, Dotta, L, Benacquista, L, Grosso, S, Crisafulli, F, Parretti, V, Giordano, Hf, Mahmoud, Aaa, Nuzzolese, R, Musso, M, Chighizola, Cb, Gentileschi, S, Morrone, M, Cola, Id, Spedicato, V, Giardini, Ham, Vasi, I, Renieri, A, Fabbiani, A, Mencarelli, Ma, Frediani, B, Balistreri, A, Tosi, Gm, Fabiani, C, Lidar, M, Rigante, D, Cantarini, L, Gaggiano C, Vitale A, Tufan A, Ragab G, Aragona E, Wiesik-Szewczyk E, Ait-Idir D, Iezzi L, Maggio MC, Cattalini M, La Torre F, Lopalco G, Verrecchia E, de Paulis A, Sahin A, Insalaco A, Sfikakis PP, Marino A, Frassi M, Ogunjimi B, Opris-Belinski D, Parronchi P, Emmi G, Shahram F, Ciccia F, Piga M, Hernández-Rodríguez J, Pereira RMR, Alessio M, Naddei R, Olivieri AN, Giudice ED, Sfriso P, Ruscitti P, Gobbi FL, Kucuk H, Sota J, Hussein MA, Malizia G, Jahnz-Różyk K, Sari-Hamidou R, Romeo M, Ricci F, Cardinale F, Iannone F, Casa FD, Natale MF, Laskari K, Giani T, Franceschini F, Sabato V, Yildirim D, Caggiano V, Hegazy MT, Marzo RD, Kucharczyk A, Khellaf G, Tarsia M, Almaghlouth IA, Laymouna AH, Mastrorilli V, Dotta L, Benacquista L, Grosso S, Crisafulli F, Parretti V, Giordano HF, Mahmoud AAA, Nuzzolese R, Musso M, Chighizola CB, Gentileschi S, Morrone M, Cola ID, Spedicato V, Giardini HAM, Vasi I, Renieri A, Fabbiani A, Mencarelli MA, Frediani B, Balistreri A, Tosi GM, Fabiani C, Lidar M, Rigante D (ORCID:0000-0001-7032-7779), Cantarini L, Conti G, Gaggiano, C, Vitale, A, Tufan, A, Ragab, G, Aragona, E, Wiesik-Szewczyk, E, Ait-Idir, D, Conti, G, Iezzi, L, Maggio, Mc, Cattalini, M, La Torre, F, Lopalco, G, Verrecchia, E, de Paulis, A, Sahin, A, Insalaco, A, Sfikakis, Pp, Marino, A, Frassi, M, Ogunjimi, B, Opris-Belinski, D, Parronchi, P, Emmi, G, Shahram, F, Ciccia, F, Piga, M, Hernández-Rodríguez, J, Pereira, Rmr, Alessio, M, Naddei, R, Olivieri, An, Giudice, Ed, Sfriso, P, Ruscitti, P, Gobbi, Fl, Kucuk, H, Sota, J, Hussein, Ma, Malizia, G, Jahnz-Różyk, K, Sari-Hamidou, R, Romeo, M, Ricci, F, Cardinale, F, Iannone, F, Casa, Fd, Natale, Mf, Laskari, K, Giani, T, Franceschini, F, Sabato, V, Yildirim, D, Caggiano, V, Hegazy, Mt, Marzo, Rd, Kucharczyk, A, Khellaf, G, Tarsia, M, Almaghlouth, Ia, Laymouna, Ah, Mastrorilli, V, Dotta, L, Benacquista, L, Grosso, S, Crisafulli, F, Parretti, V, Giordano, Hf, Mahmoud, Aaa, Nuzzolese, R, Musso, M, Chighizola, Cb, Gentileschi, S, Morrone, M, Cola, Id, Spedicato, V, Giardini, Ham, Vasi, I, Renieri, A, Fabbiani, A, Mencarelli, Ma, Frediani, B, Balistreri, A, Tosi, Gm, Fabiani, C, Lidar, M, Rigante, D, Cantarini, L, Gaggiano C, Vitale A, Tufan A, Ragab G, Aragona E, Wiesik-Szewczyk E, Ait-Idir D, Iezzi L, Maggio MC, Cattalini M, La Torre F, Lopalco G, Verrecchia E, de Paulis A, Sahin A, Insalaco A, Sfikakis PP, Marino A, Frassi M, Ogunjimi B, Opris-Belinski D, Parronchi P, Emmi G, Shahram F, Ciccia F, Piga M, Hernández-Rodríguez J, Pereira RMR, Alessio M, Naddei R, Olivieri AN, Giudice ED, Sfriso P, Ruscitti P, Gobbi FL, Kucuk H, Sota J, Hussein MA, Malizia G, Jahnz-Różyk K, Sari-Hamidou R, Romeo M, Ricci F, Cardinale F, Iannone F, Casa FD, Natale MF, Laskari K, Giani T, Franceschini F, Sabato V, Yildirim D, Caggiano V, Hegazy MT, Marzo RD, Kucharczyk A, Khellaf G, Tarsia M, Almaghlouth IA, Laymouna AH, Mastrorilli V, Dotta L, Benacquista L, Grosso S, Crisafulli F, Parretti V, Giordano HF, Mahmoud AAA, Nuzzolese R, Musso M, Chighizola CB, Gentileschi S, Morrone M, Cola ID, Spedicato V, Giardini HAM, Vasi I, Renieri A, Fabbiani A, Mencarelli MA, Frediani B, Balistreri A, Tosi GM, Fabiani C, Lidar M, Rigante D (ORCID:0000-0001-7032-7779), Cantarini L, and Conti G

Abstract

Objective: The present manuscript aims to describe an international, electronic-based, user-friendly and interoperable patient registry for monogenic autoinflammatory diseases (mAIDs), developed in the contest of the Autoinflammatory Diseases Alliance (AIDA) Network. Methods: This is an electronic platform, based on the Research Electronic Data Capture (REDCap) tool, used for real-world data collection of demographics, clinical, laboratory, instrumental and socioeconomic data of mAIDs patients. The instrument has flexibility, may change over time based on new scientific acquisitions, and communicate potentially with other similar registries; security, data quality and data governance are corner stones of the platform. Results: AIDA project will share knowledge and expertise on mAIDs. Since its start, 118 centers from 24 countries and 4 continents have joined the AIDA project. Fifty-nine centers have already obtained the approval from their local Ethics Committees. Currently, the platform counts 337 users (122 Principal Investigators, 210 Site Investigators, 2 Lead Investigators, and 3 data managers). The Registry collects baseline and follow-up data using 3,748 fields organized into 21 instruments, which include demographics, patient history, symptoms, trigger/risk factors, therapies, and healthcare information for mAIDs patients. Conclusions: The AIDA mAIDs Registry, acts both as a research tool for future collaborative real-life studies on mAIDs and as a service to connect all the figures called to participate. On this basis, the registry is expected to play a pivotal role in generating new scientific evidence on this group of rare diseases, substantially improving the management of patients, and optimizing the impact on the healthcare system. NCT 05200715 available at https://clinicaltrials.gov

Published
2022

9. Glucocorticoid tapering and associated outcome in patients with newly diagnosed systemic lupus erythematosus: the real-world GULP prospective observational study

Author

Floris, A, Chessa, E, Sebastiani, G, Prevete, I, Iannone, F, Coladonato, L, Govoni, M, Bortoluzzi, A, Mosca, M, Tani, C, Doria, A, Iaccarino, L, Franceschini, F, Fredi, M, Conti, F, Spinelli, F, Bellisai, F, D'Alessandro, R, Zanetti, A, Carrara, G, Scire, C, Cauli, A, Piga, M, Floris A., Chessa E., Sebastiani G. D., Prevete I., Iannone F., Coladonato L., Govoni M., Bortoluzzi A., Mosca M., Tani C., Doria A., Iaccarino L., Franceschini F., Fredi M., Conti F., Spinelli F. R., Bellisai F., D'Alessandro R., Zanetti A., Carrara G., Scire C. A., Cauli A., Piga M., Floris, A, Chessa, E, Sebastiani, G, Prevete, I, Iannone, F, Coladonato, L, Govoni, M, Bortoluzzi, A, Mosca, M, Tani, C, Doria, A, Iaccarino, L, Franceschini, F, Fredi, M, Conti, F, Spinelli, F, Bellisai, F, D'Alessandro, R, Zanetti, A, Carrara, G, Scire, C, Cauli, A, Piga, M, Floris A., Chessa E., Sebastiani G. D., Prevete I., Iannone F., Coladonato L., Govoni M., Bortoluzzi A., Mosca M., Tani C., Doria A., Iaccarino L., Franceschini F., Fredi M., Conti F., Spinelli F. R., Bellisai F., D'Alessandro R., Zanetti A., Carrara G., Scire C. A., Cauli A., and Piga M.

Abstract

Objective A subanalysis of the multicentre Early Lupus inception cohort was performed to investigate the real-world Glucocorticoids (GCs) Use in newly diagnosed systemic lupus erythematosus (SLE) Patients (GULP). Methods Patients starting prednisone (PDN) ≥5 mg/day and concomitant hydroxychloroquine or immunosuppressant within 12 months of SLE classification were enrolled. Core set variables were recorded at baseline and every 6 months, including changes in PDN dose, European Consensus Lupus Activity Measurement (ECLAM) and Systemic Lupus International Collaborating Clinics damage index. Regression models analysed predictors of tapering PDN<5 mg/day at any time and outcomes associated with different patterns of GCs tapering. Results The GULP study included 127 patients with SLE; 73 (57.5%) tapered and maintained PDN <5 mg/ day, and 17 (13.4%) discontinued PDN within a 2-year follow-up. Renal involvement (HR: 0.41; p=0.009) and lower C3 serum levels (HR: 1.04; p=0.025) predicted a lack of PDN tapering below 5 mg/day. High ECLAM scores were associated with a greater probability of increasing PDN dose (OR: 1.6; p=0.004), independently of daily intake. Disease relapse rate did not statistically differ (p=0.706) between patients tapering PDN <5 mg/day (42/99, 42.4%) and those tapering PDN without dropping below 5 mg/day (13/28, 46.4%). Every month on PDN <5 mg/day associated with lower damage accrual (IRR: 0.96; p=0.007), whereas never tapering PDN <5 mg/day associated with a higher risk of developing GC-related damage (OR 5.9; p=0.014). Conclusion Tapering PDN <5 mg/day was achieved and maintained in half of newly diagnosed patients with SLE and may represent a good balance between the need to prevent damage accrual and the risk of disease relapse.

Published
2022

10. Clinical spectrum time course in non-Asian patients positive for anti-MDA5 antibodies

Author

Cavagna, L, Meloni, F, Meyer, A, Sambataro, G, Belliato, M, De Langhe, E, Cavazzana, I, Pipitone, N, Triantafyllias, K, Mosca, M, Barsotti, S, Zampogna, G, Biglia, A, Emmi, G, De Visser, M, Van Der Kooi, A, Parronchi, P, Hirschi, S, da Silva, J, Scire, C, Furini, F, Giannini, M, Martinez Gonzalez, O, Damian, L, Piette, Y, Smith, V, Mera-Valera, A, Bachiller-Corral, J, Cabezas Rodriguez, I, Brandy-Garcia, A, Maurier, F, Perrin, J, Gonzalez-Moreno, J, Drott, U, Delbruck, C, Schwarting, A, Arrigoni, E, Sebastiani, G, Iuliano, A, Nannini, C, Quartuccio, L, Rodriguez Cambron, A, Blazquez Canamero, M, Villa Blanco, I, Cagnotto, G, Pesci, A, Luppi, F, Dei, G, Romero Bueno, F, Franceschini, F, Chiapparoli, I, Zanframundo, G, Lettieri, S, De Stefano, L, Cutolo, M, Mathieu, A, Piga, M, Prieto-Gonzalez, S, Moraes-Fontes, M, Fonseca, J, Jovani, V, Riccieri, V, Santaniello, A, Montfort, S, Bilocca, D, Erre, G, Bartoloni, E, Gerli, R, Monti, M, Lorenz, H, Sambataro, D, Bellando Randone, S, Schneider, U, Valenzuela, C, Lopez-Mejias, R, Cifrian, J, Mejia, M, Gonzalez Perez, M, Wendel, S, Fornaro, M, De Luca, G, Orsolini, G, Rossini, M, Dieude, P, Knitza, J, Castaneda, S, Voll, R, Rojas-Serrano, J, Valentini, A, Vancheri, C, Matucci-Cerinic, M, Feist, E, Codullo, V, Iannone, F, Distler, J, Montecucco, C, Gonzalez-Gay, M, Cavagna L., Meloni F., Meyer A., Sambataro G., Belliato M., De Langhe E., Cavazzana I., Pipitone N., Triantafyllias K., Mosca M., Barsotti S., Zampogna G., Biglia A., Emmi G., De Visser M., Van Der Kooi A., Parronchi P., Hirschi S., da Silva J. A. P., Scire C. A., Furini F., Giannini M., Martinez Gonzalez O., Damian L., Piette Y., Smith V., Mera-Valera A., Bachiller-Corral J., Cabezas Rodriguez I., Brandy-Garcia A. M., Maurier F., Perrin J., Gonzalez-Moreno J., Drott U., Delbruck C., Schwarting A., Arrigoni E., Sebastiani G. D., Iuliano A., Nannini C., Quartuccio L., Rodriguez Cambron A. B., Blazquez Canamero M. A., Villa Blanco I., Cagnotto G., Pesci A., Luppi F., Dei G., Romero Bueno F. I., Franceschini F., Chiapparoli I., Zanframundo G., Lettieri S., De Stefano L., Cutolo M., Mathieu A., Piga M., Prieto-Gonzalez S., Moraes-Fontes M. F., Fonseca J. E., Jovani V., Riccieri V., Santaniello A., Montfort S., Bilocca D., Erre G. L., Bartoloni E., Gerli R., Monti M. C., Lorenz H. M., Sambataro D., Bellando Randone S., Schneider U., Valenzuela C., Lopez-Mejias R., Cifrian J., Mejia M., Gonzalez Perez M. -I., Wendel S., Fornaro M., De Luca G., Orsolini G., Rossini M., Dieude P., Knitza J., Castaneda S., Voll R. E., Rojas-Serrano J., Valentini A., Vancheri C., Matucci-Cerinic M., Feist E., Codullo V., Iannone F., Distler J. H., Montecucco C., Gonzalez-Gay M. A., Cavagna, L, Meloni, F, Meyer, A, Sambataro, G, Belliato, M, De Langhe, E, Cavazzana, I, Pipitone, N, Triantafyllias, K, Mosca, M, Barsotti, S, Zampogna, G, Biglia, A, Emmi, G, De Visser, M, Van Der Kooi, A, Parronchi, P, Hirschi, S, da Silva, J, Scire, C, Furini, F, Giannini, M, Martinez Gonzalez, O, Damian, L, Piette, Y, Smith, V, Mera-Valera, A, Bachiller-Corral, J, Cabezas Rodriguez, I, Brandy-Garcia, A, Maurier, F, Perrin, J, Gonzalez-Moreno, J, Drott, U, Delbruck, C, Schwarting, A, Arrigoni, E, Sebastiani, G, Iuliano, A, Nannini, C, Quartuccio, L, Rodriguez Cambron, A, Blazquez Canamero, M, Villa Blanco, I, Cagnotto, G, Pesci, A, Luppi, F, Dei, G, Romero Bueno, F, Franceschini, F, Chiapparoli, I, Zanframundo, G, Lettieri, S, De Stefano, L, Cutolo, M, Mathieu, A, Piga, M, Prieto-Gonzalez, S, Moraes-Fontes, M, Fonseca, J, Jovani, V, Riccieri, V, Santaniello, A, Montfort, S, Bilocca, D, Erre, G, Bartoloni, E, Gerli, R, Monti, M, Lorenz, H, Sambataro, D, Bellando Randone, S, Schneider, U, Valenzuela, C, Lopez-Mejias, R, Cifrian, J, Mejia, M, Gonzalez Perez, M, Wendel, S, Fornaro, M, De Luca, G, Orsolini, G, Rossini, M, Dieude, P, Knitza, J, Castaneda, S, Voll, R, Rojas-Serrano, J, Valentini, A, Vancheri, C, Matucci-Cerinic, M, Feist, E, Codullo, V, Iannone, F, Distler, J, Montecucco, C, Gonzalez-Gay, M, Cavagna L., Meloni F., Meyer A., Sambataro G., Belliato M., De Langhe E., Cavazzana I., Pipitone N., Triantafyllias K., Mosca M., Barsotti S., Zampogna G., Biglia A., Emmi G., De Visser M., Van Der Kooi A., Parronchi P., Hirschi S., da Silva J. A. P., Scire C. A., Furini F., Giannini M., Martinez Gonzalez O., Damian L., Piette Y., Smith V., Mera-Valera A., Bachiller-Corral J., Cabezas Rodriguez I., Brandy-Garcia A. M., Maurier F., Perrin J., Gonzalez-Moreno J., Drott U., Delbruck C., Schwarting A., Arrigoni E., Sebastiani G. D., Iuliano A., Nannini C., Quartuccio L., Rodriguez Cambron A. B., Blazquez Canamero M. A., Villa Blanco I., Cagnotto G., Pesci A., Luppi F., Dei G., Romero Bueno F. I., Franceschini F., Chiapparoli I., Zanframundo G., Lettieri S., De Stefano L., Cutolo M., Mathieu A., Piga M., Prieto-Gonzalez S., Moraes-Fontes M. F., Fonseca J. E., Jovani V., Riccieri V., Santaniello A., Montfort S., Bilocca D., Erre G. L., Bartoloni E., Gerli R., Monti M. C., Lorenz H. M., Sambataro D., Bellando Randone S., Schneider U., Valenzuela C., Lopez-Mejias R., Cifrian J., Mejia M., Gonzalez Perez M. -I., Wendel S., Fornaro M., De Luca G., Orsolini G., Rossini M., Dieude P., Knitza J., Castaneda S., Voll R. E., Rojas-Serrano J., Valentini A., Vancheri C., Matucci-Cerinic M., Feist E., Codullo V., Iannone F., Distler J. H., Montecucco C., and Gonzalez-Gay M. A.

Abstract

Objective To define the clinical spectrum time-course and prognosis of non-Asian patients positive for anti-MDA5 antibodies. Methods We conducted a multicentre, international, retrospective cohort study. Results 149 anti-MDA5 positive patients (median onset age 53 years, median disease duration 18 months), mainly females (100, 67%), were included. Dermatomyositis (64, 43%) and amyopathic dermatomyositis (47, 31%), were the main diagnosis; 15 patients (10%) were classified as interstitial pneumonia with autoimmune features (IPAF) and 7 (5%) as rheumatoid arthritis. The main clinical findings observed were myositis (84, 56%), interstitial lung disease (ILD) (108, 78%), skin lesions (111, 74%), and arthritis (76, 51%). The onset of these manifestations was not concomitant in 74 cases (50%). Of note, 32 (21.5%) patients were admitted to the intensive care unit for rapidly progressive-ILD, which occurred in median 2 months from lung involvement detection, in the majority of cases (28, 19%) despite previous immunosuppressive treatment. One-third of patients (47, 32% each) was ANA and anti-ENA antibodies negative and a similar percentage was anti-Ro52 kDa antibodies positive. Non-specific interstitial pneumonia (65, 60%), organising pneumonia (23, 21%), and usual interstitial pneumonia-like pattern (14, 13%) were the main ILD patterns observed. Twenty-six patients died (17%), 19 (13%) had a rapidly progressive-ILD. Conclusion The clinical spectrum of the anti-MDA5 antibodies-related disease is heterogeneous. Rapidly-progressive ILD deeply impacts the prognosis also in non-Asian patients, occurring early during the disease course. Anti-MDA5 antibody positivity should be considered even when baseline autoimmune screening is negative, anti-Ro52 kDa antibodies are positive, and radiology findings show a NSIP pattern.

Published
2022

11. Relevant domains and outcome measurement instruments in neuropsychiatric systemic lupus erythematosus: a systematic literature review

Author

Silvagni, E, Chessa, E, Bergossi, F, D'Amico, M, Furini, F, Guerrini, G, Cauli, A, Scire, C, Bertsias, G, Govoni, M, Piga, M, Bortoluzzi, A, Silvagni E., Chessa E., Bergossi F., D'Amico M. E., Furini F., Guerrini G., Cauli A., Scire C. A., Bertsias G., Govoni M., Piga M., Bortoluzzi A., Silvagni, E, Chessa, E, Bergossi, F, D'Amico, M, Furini, F, Guerrini, G, Cauli, A, Scire, C, Bertsias, G, Govoni, M, Piga, M, Bortoluzzi, A, Silvagni E., Chessa E., Bergossi F., D'Amico M. E., Furini F., Guerrini G., Cauli A., Scire C. A., Bertsias G., Govoni M., Piga M., and Bortoluzzi A.

Abstract

Objectives: Although neuropsychiatric involvement in SLE (NPSLE) is one of the most complex and troubling manifestations of the disease, validated outcome instruments to be used as sensitive endpoints in controlled clinical trials are lacking. We performed a systematic literature review (SLR) to identify outcome measurement instruments and domains used to assess NPSLE. Methods: The Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines were used. Articles available in English (1967-2020), listed in PubMed, Embase, PsycINFO, Cochrane Library and the EULAR outcome measures library were screened. All domains and outcome measurement instruments were characterized according to the OMERACT Filter 2.1, considering core areas (manifestations/abnormalities, life impact, death/lifespan, societal/resource use) and contextual factors. Results: Of 3392 abstracts evaluated, 83 studies were included in the SLR (15 974 patients, females 89.9%). Eligible studies included domains and instruments pertinent to all core areas defined by the OMERACT, except for 'societal/resource use'. The most common core areas were 'manifestations/abnormalities', covering 10 domains pertinent to laboratory and instrumental markers, indexes and neuropsychiatric dimension (cognitive, neurologic and psychiatric field), and 'life impact', covering 7 domains related to physical function (from both the perspective of the patient and the physician), pain and quality of life. Conclusion: Our study revealed great heterogeneity in the instruments derived from populations with NPSLE and none of these had high-quality evidence. This supports the need to develop and further validate a core domain set and outcome measurement instruments to promote clinical research in this field, enhancing comparability across studies.

Published
2022

12. Severe neuropsychiatric systemic lupus erythematosus successfully treated with rituximab: an alternative to standard of care

Author

Chessa E, Piga M, Floris A, Mathieu A, and Cauli A

Subjects
Systemic lupus erythematosus, neuropsychiatric lupus, rituximab, demyelinating syndrome, brain MRI., Diseases of the musculoskeletal system, RC925-935
Abstract

Elisabetta Chessa, Matteo Piga, Alberto Floris, Alessandro Mathieu, Alberto Cauli Rheumatology Unit, University Clinic AOU of Cagliari, Cagliari, Italy Abstract: Demyelinating syndrome secondary to systemic lupus erythematosus (DS-SLE) is a rare encephalomyelitis burden with a high risk of disability and death. We report on a 49-year-old Caucasian woman with systemic lupus erythematosus (SLE) complicated by severe cognitive dysfunction, brainstem disease, cranial nerve palsies, weakness and numbness in limbs and multiple discrete magnetic resonance imaging (MRI) areas of damage within the white matter of semioval centers, temporal lobe, external capsule, claustrum, subinsular regions and midbrain. She also had multiple mononeuritis diagnosed through sensory and motor nerve conduction study. She was diagnosed with severe DS-SLE prominently involving the brain and was treated with 500 mg methylprednisolone (PRE) pulses for 3 consecutive days, followed by one single pulse of 500 mg cyclophosphamide, and 1 g rituximab, which was then repeated 14 days later. PRE 25 mg/day, rapidly tapered to 7.5 mg/day in 6 months, and mycophenolate mofetil 1 g/day were prescribed as maintenance therapy. She had progressive and sustained improvement in neurological symptoms with almost complete resolution of brain MRI lesions after 1 year. B-cell depleting therapy could be considered as a possible alternative to standard of care in the management of severe inflammatory neuropsychiatric SLE but it should be associated with a conventional immunosuppressant as maintenance treatment to reduce the risk of flare and reduce corticosteroids dose. Keywords: systemic lupus erythematosus, neuropsychiatric lupus, rituximab, demyelinating syndrome, brain MRI

Published
2017

13. A patient-driven registry on Behçet's disease: the AIDA for patients pilot project

Author

Gaggiano, C, Del Bianco, A, Sota, J, Gentileschi, S, Ruscitti, P, Giacomelli, R, Piga, M, Crisafulli, F, Monti, S, Emmi, G, De Paulis, A, Vitale, A, Tarsia, M, Caggiano, V, Nuzzolese, R, Parretti, V, Fabiani, C, Lopalco, G, Maier, A, Cattalini, M, Rigante, Donato, Govoni, M, Li Gobbi, F, Guiducci, S, Parronchi, P, Marino, A, Ciccia, F, Maggio, Mc, Aragona, E, Bartoloni, E, Iagnocco, A, Viapiana, O, Sebastiani, Gd, Guerriero, S, Insalaco, A, Del Giudice, E, Conti, G, Barone, P, Olivieri, An, Brucato, A, Carubbi, F, Triggianese, P, Mauro, A, Tosi, Gm, Fonollosa, A, Giardini, Ham, Ragab, G, Tharwat, S, Hernández-Rodríguez, J, Sfikakis, Pp, Laskari, K, Karamanakos, A, Espinosa, G, Shahram, F, Direskeneli, H, Hinojosa-Azaola, A, Opris-Belinski, D, Almaghlouth, Ia, Hatemi, G, Eksin, Ma, Önen, F, Więsik-Szewczyk, E, Akkoç, N, Tufan, A, Şahin, A, Erten, Ş, Ozen, S, Batu, Ed, Frediani, B, Balistreri, A, Cantarini, L, Rigante D (ORCID:0000-0001-7032-7779), Gaggiano, C, Del Bianco, A, Sota, J, Gentileschi, S, Ruscitti, P, Giacomelli, R, Piga, M, Crisafulli, F, Monti, S, Emmi, G, De Paulis, A, Vitale, A, Tarsia, M, Caggiano, V, Nuzzolese, R, Parretti, V, Fabiani, C, Lopalco, G, Maier, A, Cattalini, M, Rigante, Donato, Govoni, M, Li Gobbi, F, Guiducci, S, Parronchi, P, Marino, A, Ciccia, F, Maggio, Mc, Aragona, E, Bartoloni, E, Iagnocco, A, Viapiana, O, Sebastiani, Gd, Guerriero, S, Insalaco, A, Del Giudice, E, Conti, G, Barone, P, Olivieri, An, Brucato, A, Carubbi, F, Triggianese, P, Mauro, A, Tosi, Gm, Fonollosa, A, Giardini, Ham, Ragab, G, Tharwat, S, Hernández-Rodríguez, J, Sfikakis, Pp, Laskari, K, Karamanakos, A, Espinosa, G, Shahram, F, Direskeneli, H, Hinojosa-Azaola, A, Opris-Belinski, D, Almaghlouth, Ia, Hatemi, G, Eksin, Ma, Önen, F, Więsik-Szewczyk, E, Akkoç, N, Tufan, A, Şahin, A, Erten, Ş, Ozen, S, Batu, Ed, Frediani, B, Balistreri, A, Cantarini, L, and Rigante D (ORCID:0000-0001-7032-7779)

Abstract

Introduction: This paper describes the creation and preliminary results of a patient-driven registry for the collection of patient-reported outcomes (PROs) and patient-reported experiences (PREs) in Behçet’s disease (BD). Methods: The project was coordinated by the University of Siena and the Italian patient advocacy organization SIMBA (Associazione Italiana Sindrome e Malattia di Behçet), in the context of the AIDA (AutoInflammatory Diseases Alliance) Network programme. Quality of life, fatigue, socioeconomic impact of the disease and therapeutic adherence were selected as core domains to include in the registry. Results: Respondents were reached via SIMBA communication channels in 167 cases (83.5%) and the AIDA Network affiliated clinical centers in 33 cases (16.5%). The median value of the Behçet’s Disease Quality of Life (BDQoL) score was 14 (IQR 11, range 0–30), indicating a medium quality of life, and the median Global Fatigue Index (GFI) was 38.7 (IQR 10.9, range 1–50), expressing a significant level of fatigue. The mean Beliefs about Medicines Questionnaire (BMQ) necessity-concern differential was 0.9 ± 1.1 (range – 1.8–4), showing that the registry participants prioritized necessity belief over concerns to a limited extent. As for the socioeconomic impact of BD, in 104 out of 187 cases (55.6%), patients had to pay from their own pocket for medical exams required to reach the diagnosis. The low family socioeconomic status (p < 0.001), the presence of any major organ involvement (p < 0.031), the presence of gastro-intestinal (p < 0.001), neurological (p = 0.012) and musculoskeletal (p = 0.022) symptoms, recurrent fever (p = 0.002), and headache (p < 0.001) were associated to a higher number of accesses to the healthcare system. Multiple linear regression showed that the BDQoL score could significantly predict the global socioeconomic impact of BD (F = 14.519, OR 1.162 [CI 0.557–1.766], p < 0.001). Discussion: Preliminary results from the AID

Published
2023

14. Musculoskeletal manifestations in children with Behçet's syndrome: data from the AIDA Network Behçet's Syndrome Registry

Author

Gaggiano, C, Maselli, A, Sfikakis, Pp, Laskari, K, Ragab, G, Hegazy, Mt, Laymouna, Ah, Lopalco, G, Almaghlouth, Ia, Asfina, Kn, Alahmed, O, Giardini Mayrink, Ha, Parente de Brito Antonelli, I, Cattalini, M, Piga, M, Sota, J, Gentileschi, S, Maggio, Mc, Opris-Belinski, D, Hatemi, G, Insalaco, A, Olivieri, An, Tufan, A, Karadeniz, H, Kardaş, Rc, La Torre, F, Cardinale, F, Marino, A, Guerriero, S, Ruscitti, P, Tarsia, M, Vitale, A, Caggiano, V, Telesca, S, Iannone, F, Parretti, V, Frassi, M, Aragona, E, Ciccia, F, Wiesik-Szewczyk, E, Ionescu, R, Şahin, A, Akkoç, N, Hinojosa-Azaola, A, Tharwat, S, Hernández-Rodríguez, J, Espinosa, G, Conti, G, Del Giudice, E, Govoni, M, Emmi, G, Fabiani, C, Balistreri, A, Frediani, B, Rigante, Donato, Cantarini, L, Rigante D (ORCID:0000-0001-7032-7779), Gaggiano, C, Maselli, A, Sfikakis, Pp, Laskari, K, Ragab, G, Hegazy, Mt, Laymouna, Ah, Lopalco, G, Almaghlouth, Ia, Asfina, Kn, Alahmed, O, Giardini Mayrink, Ha, Parente de Brito Antonelli, I, Cattalini, M, Piga, M, Sota, J, Gentileschi, S, Maggio, Mc, Opris-Belinski, D, Hatemi, G, Insalaco, A, Olivieri, An, Tufan, A, Karadeniz, H, Kardaş, Rc, La Torre, F, Cardinale, F, Marino, A, Guerriero, S, Ruscitti, P, Tarsia, M, Vitale, A, Caggiano, V, Telesca, S, Iannone, F, Parretti, V, Frassi, M, Aragona, E, Ciccia, F, Wiesik-Szewczyk, E, Ionescu, R, Şahin, A, Akkoç, N, Hinojosa-Azaola, A, Tharwat, S, Hernández-Rodríguez, J, Espinosa, G, Conti, G, Del Giudice, E, Govoni, M, Emmi, G, Fabiani, C, Balistreri, A, Frediani, B, Rigante, Donato, Cantarini, L, and Rigante D (ORCID:0000-0001-7032-7779)

Abstract

This study aims to describe musculoskeletal manifestations (MSM) in children with Behçet’s syndrome (BS), their association with other disease manifestations, response to therapy, and long-term prognosis. Data were retrieved from the AIDA Network Behçet’s Syndrome Registry. Out of a total of 141 patients with juvenile BS, 37 had MSM at disease onset (26.2%). The median age at onset was 10.0 years (IQR 7.7). The median follow-up duration was 21.8 years (IQR 23.3). Recurrent oral (100%) and genital ulcers (67.6%) and pseudofolliculitis (56.8%) were the most common symptoms associated with MSM. At disease onset, 31 subjects had arthritis (83.8%), 33 arthralgia (89.2%), and 14 myalgia (37.8%). Arthritis was monoarticular in 9/31 cases (29%), oligoarticular in 10 (32.3%), polyarticular in 5 (16.1%), axial in 7 (22.6%). Over time, arthritis became chronic-recurrent in 67.7% of cases and 7/31 patients had joint erosions (22.6%). The median Behçet's Syndrome Overall Damage Index was 0 (range 0–4). Colchicine was inefcacious for MSM in 4/14 cases (28.6%), independently from the type of MSM (p=0.46) or the concomitant therapy (p=0.30 for cDMARDs, p=1.00 for glucocorticoids); cDMARDs and bDMARDs were inefcacious for MSM in 6/19 (31.4%) and 5/12 (41.7%) cases. The presence of myalgia was associated with bDMARDs inefcacy (p=0.014). To conclude, MSM in children with BS are frequently associated with recurrent ulcers and pseudofolliculitis. Arthritis is mostly mono- or oligoarticular, but sacroiliitis is not unusual. Prognosis of this subset of BS is overall favorable, though the presence of myalgia negatively afects response to biologic therapies.

Published
2023

15. Current perspective on the role of the interleukin-23/interleukin-17 axis in inflammation and disease (chronic arthritis and psoriasis)

Author

Cauli A, Piga M, Floris A, and Mathieu A

Subjects
TH17, IL-17, IL-23, Psoriasis, Psoriatic Arthritis, Ankylosing Spondylitis, Immunologic diseases. Allergy, RC581-607
Abstract

Alberto Cauli, Matteo Piga, Alberto Floris, Alessandro Mathieu Rheumatology Unit, Department of Medical Sciences, Policlinico of the University of Cagliari, Monserrato, Cagliari, Italy Abstract: TH17 is a lymphocyte subset, which is characterized by its polarization to secrete interleukin (IL)-17. IL-23 is the pivotal mediator responsible for TH17 differentiation and the IL-23/IL-17 axis has been strongly implicated in the pathogenesis of several immune mediated diseases, in particular chronic arthritis and skin psoriasis. This review will summarize the basic immunology and the new monoclonal antibodies, which antagonize this pathway allowing a new therapeutic approach. Keywords: TH17, IL-17, IL-23, psoriasis, psoriatic arthritis, ankylosing spondylitis

Published
2015

17. Clinical potential of apremilast in the treatment of psoriatic arthritis

Author

Cauli A, Porru G, Piga M, Vacca A, Dessole G, and Mathieu A

Subjects
Psoriatic Arthritis, Apremilast, Therapy., Immunologic diseases. Allergy, RC581-607
Abstract

Alberto Cauli, Giovanni Porru, Matteo Piga, Alessandra Vacca, Grazia Dessole, Alessandro MathieuRheumatology Unit, Department of Medical Sciences, Policlinico of University of Cagliari, Monserrato, ItalyAbstract: Psoriatic arthritis (PsA) is a frequent chronic inflammatory disease characterized by joint and skin involvement, and by typical extra-articular manifestations. Although the pathogenesis of PsA is still under investigation, the available evidence suggests the importance of the patient's genetic background, microbial or environmental triggers, and an imbalance in the adaptive and acquired immune system, resulting in the production of inflammatory mediators. New therapeutic approaches have been proposed, among them the use of modulators of intracellular signals and gene transcription such as PDE4-inhibiting compounds, which are able to modulate the activity of transcription factors such as CREB and NF-κB and therefore the synthesis of inflammatory mediators, resulting in immunoregulation. This paper summarizes the mechanism of action of apremilast, a PDE4 inhibitor, and the clinical data available on its clinical efficacy and safety profile in the treatment of PsA patients.Keywords: psoriatic arthritis, apremilast, therapy

Published
2014

18. Risk factors of damage in early diagnosed systemic lupus erythematosus: results of the Italian multicentre Early Lupus Project inception cohort

Author

Piga, M, Floris, A, Sebastiani, G, Prevete, I, Iannone, F, Coladonato, L, Govoni, M, Bortoluzzi, A, Mosca, M, Tani, C, Doria, A, Iaccarino, L, Franceschini, F, Fredi, M, Conti, F, Spinelli, F, Galeazzi, M, Bellisai, F, Zanetti, A, Carrara, G, Scirè, C, Mathieu, A, Piga M, Floris A, Sebastiani GD, Prevete I, Iannone F, Coladonato L, Govoni M, Bortoluzzi A, Mosca M, Tani C, Doria A, Iaccarino L, Franceschini F, Fredi M, Conti F, Spinelli FR8, Galeazzi M, Bellisai F, Zanetti A, Carrara G, Scirè CA, Mathieu A, Piga, M, Floris, A, Sebastiani, G, Prevete, I, Iannone, F, Coladonato, L, Govoni, M, Bortoluzzi, A, Mosca, M, Tani, C, Doria, A, Iaccarino, L, Franceschini, F, Fredi, M, Conti, F, Spinelli, F, Galeazzi, M, Bellisai, F, Zanetti, A, Carrara, G, Scirè, C, Mathieu, A, Piga M, Floris A, Sebastiani GD, Prevete I, Iannone F, Coladonato L, Govoni M, Bortoluzzi A, Mosca M, Tani C, Doria A, Iaccarino L, Franceschini F, Fredi M, Conti F, Spinelli FR8, Galeazzi M, Bellisai F, Zanetti A, Carrara G, Scirè CA, and Mathieu A

Abstract

Objective: To investigate risk factors for damage development in a prospective inception cohort of early diagnosed SLE patients. Methods: The Early Lupus Project recruited an inception cohort of patients within 12 months of SLE classification (1997 ACR criteria). At enrolment and every 6 months thereafter, the SLICC/ACR Damage Index was recorded. The contribution of baseline and time-varying covariates to the development of damage, defined as any SLICC/ACR Damage Index increase from 0 to ≥1, was assessed using univariate analysis. Forward-backward Cox regression models were fitted with covariates with P < 0.05 to identify factors independently associated with the risk of damage development. Results: Overall, 230 patients with a mean (s.d.) age of 36.5 (14.4) years were eligible for this study; the mean number of visits per patient was 5.3 (2.7). There were 51 (22.2%) patients with SLICC/ACR Damage Index ≥1 after 12 months, 59 (25.6%) after 24 months and 67 (29.1%) after 36 months. Dyslipidaemia [P = 0.001; hazard ratio (HR) 2.9; 95% CI 1.5, 5.6], older age (P = 0.001; HR 3.0; 95% CI 1.6, 5.5), number of organs/systems involved (P = 0.002; HR 1.4; 95% CI 1.1, 1.8) and cardiorespiratory involvement (P = 0.041; HR 1.9; 95% CI 1.0, 3.7) were independently associated with an increased risk of developing damage. Risk profiles for damage development differed for glucocorticoid-related and-unrelated damage. HCQ use (P = 0.005; HR 0.4; 95% CI 0.2, 0.8) reduced the risk of glucocorticoid-unrelated damage. Conclusion: We identified risk factors of damage development, but little effect of glucocorticoids, in this early SLE cohort. Addressing modifiable risk factors from the time of SLE diagnosis might improve patient outcomes.

Published
2020

20. Development and implementation of the AIDA International Registry for patients with Behçet's disease

Author

Vitale, A, Della Casa, F, Ragab, G, Almaghlouth, Ia, Lopalco, G, Pereira, Rm, Guerriero, S, Govoni, M, Sfikakis, Pp, Giacomelli, R, Ciccia, F, Monti, S, Ruscitti, P, Piga, M, Lomater, C, Tufan, A, Opris-Belinski, D, Emmi, G, Hernández-Rodríguez, J, Şahin, A, Sebastiani, Gd, Bartoloni, E, Akkoç, N, Gündüz, Ö, Cattalini, M, Conti, Giorgio, Hatemi, G, Maier, A, Parronchi, P, Del Giudice, E, Erten, S, Insalaco, A, Li Gobbi, F, Maggio, Mc, Shahram, F, Caggiano, V, Hegazy, Mt, Asfina, Kn, Morrone, M, Prado, Ll, Dammacco, R, Ruffilli, F, Arida, A, Navarini, L, Pantano, I, Cavagna, L, Conforti, A, Cauli, A, Marucco, Em, Kucuk, H, Ionescu, R, Mattioli, I, Espinosa, G, Araújo, O, Karkaş, B, Canofari, C, Sota, J, Laymouna, Ah, Bedaiwi, Aa, Colella, S, Giardini, Ham, Albano, V, Lo Monaco, A, Fragoulis, Ge, Kardas, Rc, Berlengiero, V, Hussein, Ma, Ricci, F, La Torre, F, Rigante, Donato, Więsik-Szewczyk, E, Frassi, M, Gentileschi, S, Tosi, Gm, Dagostin, Ma, Mahmoud, Aaa, Tarsia, M, Alessio, G, Cimaz, R, Giani, T, Gaggiano, C, Iannone, F, Cipriani, P, Mourabi, M, Spedicato, V, Barneschi, S, Aragona, E, Balistreri, A, Frediani, B, Fabiani, C, Cantarini, L &amp, Autoinflammatory Diseases Alliance (AIDA), Network, Conti G (ORCID:0000-0002-8566-9365), Rigante D (ORCID:0000-0001-7032-7779), Vitale, A, Della Casa, F, Ragab, G, Almaghlouth, Ia, Lopalco, G, Pereira, Rm, Guerriero, S, Govoni, M, Sfikakis, Pp, Giacomelli, R, Ciccia, F, Monti, S, Ruscitti, P, Piga, M, Lomater, C, Tufan, A, Opris-Belinski, D, Emmi, G, Hernández-Rodríguez, J, Şahin, A, Sebastiani, Gd, Bartoloni, E, Akkoç, N, Gündüz, Ö, Cattalini, M, Conti, Giorgio, Hatemi, G, Maier, A, Parronchi, P, Del Giudice, E, Erten, S, Insalaco, A, Li Gobbi, F, Maggio, Mc, Shahram, F, Caggiano, V, Hegazy, Mt, Asfina, Kn, Morrone, M, Prado, Ll, Dammacco, R, Ruffilli, F, Arida, A, Navarini, L, Pantano, I, Cavagna, L, Conforti, A, Cauli, A, Marucco, Em, Kucuk, H, Ionescu, R, Mattioli, I, Espinosa, G, Araújo, O, Karkaş, B, Canofari, C, Sota, J, Laymouna, Ah, Bedaiwi, Aa, Colella, S, Giardini, Ham, Albano, V, Lo Monaco, A, Fragoulis, Ge, Kardas, Rc, Berlengiero, V, Hussein, Ma, Ricci, F, La Torre, F, Rigante, Donato, Więsik-Szewczyk, E, Frassi, M, Gentileschi, S, Tosi, Gm, Dagostin, Ma, Mahmoud, Aaa, Tarsia, M, Alessio, G, Cimaz, R, Giani, T, Gaggiano, C, Iannone, F, Cipriani, P, Mourabi, M, Spedicato, V, Barneschi, S, Aragona, E, Balistreri, A, Frediani, B, Fabiani, C, Cantarini, L &amp, Autoinflammatory Diseases Alliance (AIDA), Network, Conti G (ORCID:0000-0002-8566-9365), and Rigante D (ORCID:0000-0001-7032-7779)

Abstract

Objective: Purpose of the present paper is to point out the design, development and deployment of the AutoInflammatory Disease Alliance (AIDA) International Registry dedicated to pediatric and adult patients with Behçet’s disease (BD). Methods: The Registry is a clinical physician-driven population- and electronic-based instrument implemented for the retrospective and prospective collection of real-life data about demographics, clinical, therapeutic, laboratory, instrumental and socioeconomic information from BD patients; the Registry is based on the Research Electronic Data Capture (REDCap) tool, which is thought to collect standardised information for clinical real-life research, and has been realised to change over time according to future scientific acquisitions and potentially communicate with other existing and future Registries dedicated to BD. Results: Starting from January 31st to November 23rd, 2021, 99 centres from 20 countries in 4 continents have been involved. Forty-eight of these have already obtained the approval from their local Ethics Committees. Currently, the platform counts 265 users (99 Principal Investigators, 162 Site Investigators, 2 Lead Investigators, and 2 data managers). The Registry collects baseline and follow-up data using 5474 fields organised into 15 instruments, including patient’s demographics, history, clinical manifestations and symptoms, trigger/risk factors, therapies and healthcare access. Conclusions: The development of the AIDA International Registry for BD patients will facilitate the collection of standardised data leading to real-world evidence, enabling international multicentre collaborative research through data sharing, international consultation, dissemination of knowledge, inclusion of patients and families, and ultimately optimisation of scientific efforts and implementation of standardised care.

Published
2022

21. Development and implementation of the AIDA International Registry for patients with VEXAS syndrome

Author

Vitale, A, Caggiano, V, Della Casa, F, Hernández-Rodríguez, J, Frassi, M, Monti, S, Tufan, A, Telesca, S, Conticini, E, Ragab, G, Lopalco, G, Almaghlouth, I, Pereira, Rmr, Yildirim, D, Cattalini, M, Marino, A, Giani, T, La Torre, F, Ruscitti, P, Aragona, E, Wiesik-Szewczyk, E, Del Giudice, E, Sfikakis, Pp, Govoni, M, Emmi, G, Maggio, Mc, Giacomelli, R, Ciccia, F, Conti, Giorgio, Ait-Idir, D, Lomater, C, Sabato, V, Piga, M, Sahin, A, Opris-Belinski, D, Ionescu, R, Bartoloni, E, Franceschini, F, Parronchi, P, de Paulis, A, Espinosa, G, Maier, A, Sebastiani, Gd, Insalaco, A, Shahram, F, Sfriso, P, Minoia, F, Alessio, M, Makowska, J, Hatemi, G, Akkoç, N, Li Gobbi, F, Gidaro, A, Olivieri, An, Al-Mayouf, Sm, Erten, S, Gentileschi, S, Vasi, I, Tarsia, M, Mahmoud, Aaa, Frediani, B, Fares Alzahrani, M, Laymouna, Ah, Ricci, F, Cardinale, F, Jahnz-Rózyk, K, Tosi, Gm, Crisafulli, F, Balistreri, A, Dagostin, Ma, Ghanema, M, Gaggiano, C, Sota, J, Di Cola, I, Fabiani, C, Giardini, Ham, Renieri, A, Fabbiani, A, Carrer, A, Bocchia, M, Caroni, F, Rigante, Donato, Cantarini, L, Conti G (ORCID:0000-0002-8566-9365), Rigante D (ORCID:0000-0001-7032-7779), Vitale, A, Caggiano, V, Della Casa, F, Hernández-Rodríguez, J, Frassi, M, Monti, S, Tufan, A, Telesca, S, Conticini, E, Ragab, G, Lopalco, G, Almaghlouth, I, Pereira, Rmr, Yildirim, D, Cattalini, M, Marino, A, Giani, T, La Torre, F, Ruscitti, P, Aragona, E, Wiesik-Szewczyk, E, Del Giudice, E, Sfikakis, Pp, Govoni, M, Emmi, G, Maggio, Mc, Giacomelli, R, Ciccia, F, Conti, Giorgio, Ait-Idir, D, Lomater, C, Sabato, V, Piga, M, Sahin, A, Opris-Belinski, D, Ionescu, R, Bartoloni, E, Franceschini, F, Parronchi, P, de Paulis, A, Espinosa, G, Maier, A, Sebastiani, Gd, Insalaco, A, Shahram, F, Sfriso, P, Minoia, F, Alessio, M, Makowska, J, Hatemi, G, Akkoç, N, Li Gobbi, F, Gidaro, A, Olivieri, An, Al-Mayouf, Sm, Erten, S, Gentileschi, S, Vasi, I, Tarsia, M, Mahmoud, Aaa, Frediani, B, Fares Alzahrani, M, Laymouna, Ah, Ricci, F, Cardinale, F, Jahnz-Rózyk, K, Tosi, Gm, Crisafulli, F, Balistreri, A, Dagostin, Ma, Ghanema, M, Gaggiano, C, Sota, J, Di Cola, I, Fabiani, C, Giardini, Ham, Renieri, A, Fabbiani, A, Carrer, A, Bocchia, M, Caroni, F, Rigante, Donato, Cantarini, L, Conti G (ORCID:0000-0002-8566-9365), and Rigante D (ORCID:0000-0001-7032-7779)

Abstract

Objective: The aim of this paper is to present the AutoInflammatory Disease Alliance (AIDA) International Registry dedicated to Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic (VEXAS) syndrome, describing its design, construction, and modalities of dissemination. Methods: The present registry is a clinical, physician-driven, population- and electronic-based instrument designed for the retrospective and prospective collection of real-life data. Data gathering is based on the Research Electronic Data Capture (REDCap) tool and is intended to obtain real-world evidence for daily patients’ management; the registry may potentially communicate with other on-line tools dedicated to VEXAS syndrome, enhancing international collaboration and data sharing for research purposes. The registry is handy enough to be easily modified to meet future needs regarding VEXAS syndrome. Results: To date (April 2022), 105 Centers from 23 Countries in 4 continents have been involved; 287 users (106 Principal Investigators, 177 Site Investigators, 2 Lead Investigators, and 2 data managers) may already enter the registry for data collection and sharing. The registry includes 4950 fields organised into 18 instruments designed to fully describe patient’s details about demographics, clinical manifestations, symptoms, histologic details about skin and bone marrow biopsies and aspirate, laboratory features, complications, comorbidities, therapies, and healthcare access. Conclusions: This international Registry for patients with VEXAS syndrome will allow the achievement of a comprehensive knowledge about this new disease in a relatively short time with the final goal to obtain real-world evidence data for daily clinical practice. This project can be found on https://clinicaltrials.gov NCT 05200715

Published
2022

22. Estimated 10-year cardiovascular risk in a large Italian cohort of rheumatoid arthritis patients: Data from the Cardiovascular Obesity and Rheumatic DISease (CORDIS) Study Group

Author

Cacciapaglia, F., Spinelli, F. R., Piga, M., Erre, G. L., Sakellariou, G., Manfredi, A., Viapiana, O., Fornaro, M., Colella, S., Floris, A., Mangoni, A. A., Castagna, F., Vacchi, Caterina, Orsolini, G., Bugatti, S., Cafaro, G., Cauli, A., Gremese, Elisa, Atzeni, F., Bartoloni, E., Vacchi C., Gremese E. (ORCID:0000-0002-2248-1058), Cacciapaglia, F., Spinelli, F. R., Piga, M., Erre, G. L., Sakellariou, G., Manfredi, A., Viapiana, O., Fornaro, M., Colella, S., Floris, A., Mangoni, A. A., Castagna, F., Vacchi, Caterina, Orsolini, G., Bugatti, S., Cafaro, G., Cauli, A., Gremese, Elisa, Atzeni, F., Bartoloni, E., Vacchi C., and Gremese E. (ORCID:0000-0002-2248-1058)

Abstract

Background: Several cardiovascular (CV) risk algorithms are available to predict CV events in the general population. However, their performance in patients with rheumatoid arthritis (RA) might differ from the general population. This cross-sectional multicentre study aimed to estimate the 10-year CV risk using two different algorithms in a large RA cohort and in patients with osteoarthritis (OA). Methods: In a consecutive series of RA patients and matched OA controls without prior CV events, clinical and serologic data and traditional CV risk factors were recorded. The 10-year CV risk was assessed with the Systematic COronary Risk Evaluation (SCORE) and the “Progetto Cuore” algorithms. Results: 1,467 RA patients and 342 OA subjects were included. RA patients were more frequently diabetic (9.9% vs 6.4%; p=0.04) and smokers (20.4% vs 12.5%; p=0.002) but had lower prevalence of obesity (15% vs 21%; p=0.003). Dyslipidaemia was more prevalent in OA (32.5% vs 21.7%; p<0.0001). The 10-year estimated CV risk was 1.6% (95%CI 1.3-1.9) in RA and 1.4% (95%CI 1.3-1.6) in OA (p=0.002) according to SCORE and 6.5% (95%CI 6.1-6.9) in RA and 4.4% (95%CI 3.9-5.1) in OA (p<0.001) according to “Progetto Cuore”. Regardless of the score used, RA patients had a 3- to-4-fold increased 10-year risk of CV events compared to OA subjects. Conclusion: RA patients have a significantly higher 10-year risk of CV events than OA subjects. In addition to effective disease control and joint damage prevention, specific protective measures targeting modifiable traditional CV risk factors should be implemented in RA.

Published
2022

23. Risk of acute arterial and venous thromboembolic events in eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)

Author

Bettiol, A., Sinico, R. A., Schiavon, F., Monti, S., Bozzolo, E. P., Franceschini, F., Govoni, M., Lunardi, C., Guida, G., Lopalco, G., Paolazzi, G., Vacca, A., Gregorini, G., Leccese, P., Piga, M., Conti, F., Fraticelli, P., Quartuccio, L., Alberici, F., Salvarani, C., Bettio, S., Negrini, S., Selmi, C., Sciascia, S., Moroni, G., Colla, L., Manno, C., Urban, M. L., Vannacci, A., Pozzi, M. R., Fabbrini, P., Polti, S., Felicetti, M., Marchi, M. R., Padoan, R., Delvino, P., Caporali, R., Montecucco, C., Dagna, L., Cariddi, A., Toniati, P., Tamanini, S., Furini, F., Bortoluzzi, A., Tinazzi, E., Delfino, L., Badiu, I., Rolla, G., Venerito, V., Iannone, F., Berti, A., Bortolotti, R., Racanelli, V., Jeannin, G., Padula, A., Cauli, A., Priori, R., Gabrielli, A., Bond, M., Tedesco, M., Pazzola, G., Tomietto, P., Pellecchio, M., Marvisi, C., Maritati, F., Palmisano, A., Dejaco, C., Willeit, J., Kiechl, S., Olivotto, I., Willeit, P., Prisco, D., Vaglio, A., Emmi, G., Bargagli, E., Becatti, M., Beccalli, M., Bello, F., Bozzao, F., Canti, V., Cassia, M. A., Cassone, G., Catanoso, M., Chieco-Bianchi, F., Clari, R., Coladonato, L., De Santis, M., Di Scala, G., Fagni, F., Fenaroli, P., Fiorillo, C., Floris, A., Fornaro, M., Galli, E., Generali, E., Giliberti, M., Lascaro, N., Leccese, I., Mattioli, I., Olivieri, B., Osti, N., Peyronel, F., Radin, M., Righetti, G., Salvati, S., Silvestri, E., Susca, N., Tamburini, C., Taurisano, G., Trezzi, B., Trivioli, G., Bettiol, A, Sinico, R, Schiavon, F, Monti, S, Bozzolo, E, Franceschini, F, Govoni, M, Lunardi, C, Guida, G, Lopalco, G, Paolazzi, G, Vacca, A, Gregorini, G, Leccese, P, Piga, M, Conti, F, Fraticelli, P, Quartuccio, L, Alberici, F, Salvarani, C, Bettio, S, Negrini, S, Selmi, C, Sciascia, S, Moroni, G, Colla, L, Manno, C, Urban, M, Vannacci, A, Pozzi, M, Fabbrini, P, Polti, S, Felicetti, M, Marchi, M, Padoan, R, Delvino, P, Caporali, R, Montecucco, C, Dagna, L, Cariddi, A, Toniati, P, Tamanini, S, Furini, F, Bortoluzzi, A, Tinazzi, E, Delfino, L, Badiu, I, Rolla, G, Venerito, V, Iannone, F, Berti, A, Bortolotti, R, Racanelli, V, Jeannin, G, Padula, A, Cauli, A, Priori, R, Gabrielli, A, Bond, M, Tedesco, M, Pazzola, G, Tomietto, P, Pellecchio, M, Marvisi, C, Maritati, F, Palmisano, A, Dejaco, C, Willeit, J, Kiechl, S, Olivotto, I, Willeit, P, Prisco, D, Vaglio, A, and Emmi, G

Subjects
Pulmonary and Respiratory Medicine, Burden of disease, Humans, Churg-Strauss Syndrome, Granulomatosis with Polyangiitis, Venous Thromboembolism, Venous Thrombosis, Churg-strauss syndrome, Criminology, NO, 03 medical and health sciences, 0302 clinical medicine, Medicine, 030212 general & internal medicine, Vascular inflammation, business.industry, Conflict of interest, Cytoplasmic antibody, medicine.disease, 030228 respiratory system, Wegener granulomatosis, arterial and venous thromboembolic events, Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome), Organ involvement, business, Production team
Abstract

Eosinophilic Granulomatosis with Polyangiitis (EGPA, Churg-Strauss syndrome) is a rare anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) characterised by respiratory manifestations and systemic organ involvement [1]. Particularly, cardiac manifestations occur in 40–60% of patients, representing the leading cause of mortality [2]. Recent reports suggest that venous thromboembolic events might also represent a consistent burden of disease [3, 4], as already known for the other AAVs [5–7], possibly due to eosinophil-mediated vascular inflammation [5]. Nevertheless, the occurrence of arterial and venous thrombotic events (AVTE) has never been systematically explored in EGPA. Footnotes This manuscript has recently been accepted for publication in the European Respiratory Journal . It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article. Conflict of interest: Alessandra Bettiol Conflict of interest: Renato Alberto Sinico Conflict of interest: Franco Schiavon Conflict of interest: Sara Monti Conflict of interest: Enrica Paola Bozzolo Conflict of interest: Franco Franceschini Conflict of interest: Marcello Govoni Conflict of interest: Claudio Lunardi Conflict of interest: Giuseppe Guida Conflict of interest: Giuseppe Lopalco Conflict of interest: Giuseppe Paolazzi Conflict of interest: Angelo Vacca Conflict of interest: Gina Gregorini Conflict of interest: Pietro Leccese Conflict of interest: Matteo Piga Conflict of interest: Fabrizio Conti Conflict of interest: Paolo Fraticelli Conflict of interest: Luca Quartuccio Conflict of interest: Federico Alberici Conflict of interest: Carlo Salvarani Conflict of interest: Silvano Bettio Conflict of interest: Simone Negrini Conflict of interest: Carlo Selmi Conflict of interest: Savino Sciascia Conflict of interest: Gabriella Moroni Conflict of interest: Loredana Colla Conflict of interest: Carlo Manno Conflict of interest: Maria Letizia Urban Conflict of interest: Alfredo Vannacci Conflict of interest: Maria Rosa Pozzi Conflict of interest: Paolo Fabbrini Conflict of interest: Stefano Polti Conflict of interest: Mara Felicetti Conflict of interest: Maria Rita Marchi Conflict of interest: Roberto Padoan Conflict of interest: Paolo Delvino Conflict of interest: Roberto Caporali Conflict of interest: Carlomaurizio Montecucco Conflict of interest: Lorenzo Dagna Conflict of interest: Adriana Cariddi Conflict of interest: Paola Toniati Conflict of interest: Dr. Tamanini reports other from Glaxo Smith Kline, outside the submitted work. Conflict of interest: Federica Furini Conflict of interest: Alessandra Bortoluzzi Conflict of interest: Elisa Tinazzi Conflict of interest: Lorenzo Delfino Conflict of interest: Iuliana Badiu Conflict of interest: Giovanni Rolla Conflict of interest: Vincenzo Venerito Conflict of interest: Florenzo Iannone Conflict of interest: Alvise Berti Conflict of interest: Roberto Bortolotti Conflict of interest: Vito Racanelli Conflict of interest: Guido Jeannin Conflict of interest: Angela Padula Conflict of interest: Alberto Cauli Conflict of interest: Roberta Priori Conflict of interest: Armando Gabrielli Conflict of interest: Milena Bond Conflict of interest: Martina Tedesco Conflict of interest: Giulia Pazzola Conflict of interest: Paola Tomietto Conflict of interest: Marco Pellecchio Conflict of interest: Chiara Marvisi Conflict of interest: Federica Maritati Conflict of interest: Alessandra Palmisano Conflict of interest: Christian Dejaco Conflict of interest: Johann Willeit Conflict of interest: Stefan Kiechl Conflict of interest: Iacopo Olivotto Conflict of interest: Peter Willeit Conflict of interest: Domenico Prisco Conflict of interest: Augusto Vaglio Conflict of interest: Giacomo Emmi

Published
2020

24. Erratum: Management of adult-onset Still's disease with interleukin-1 inhibitors: Evidence- And consensus-based statements by a panel of Italian experts (Arthritis Res Ther (2019) 21:275 DOI: 10.1186/s13075-019-2021-9)

Author

Colafrancesco, S., Manara, M., Bortoluzzi, A., Serban, T., Bianchi, G., Cantarini, L., Ciccia, F., Dagna, L., Govoni, M., Montecucco, C., Priori, R., Ravelli, A., Sfriso, P., Sinigaglia, L., Alivernini, S., Baldissera, E., Bartoloni, E., Berti, A., Bugatti, S., Camellino, D., Cammelli, D., Caporali, R., Caso, F., Cavallaro, E., Cavalli, G., Colaci, M., Costa, L., Di Scala, G., Emmi, G., Frassi, M., Gerli, R., Giacomelli, R., Gremese, E., Iannone, F., Lapadula, G., Leveghi, L., Lopalco, G., Manna, R., Marotto, D., Mathieu, A., Neri, R., Patisso, I., Piga, M., Punzi, L., Romano, M., Ruscitti, P., Salvarani, C., Scarpa, R., Scrivo, R., Talarico, R., Verrecchia, E., Viapiana, O., Vitale, A., Vitiello, G., Colafrancesco, S., Manara, M., Bortoluzzi, A., Serban, T., Bianchi, G., Cantarini, L., Ciccia, F., Dagna, L., Govoni, M., Montecucco, C., Priori, R., Ravelli, A., Sfriso, P., Sinigaglia, L., Alivernini, S., Baldissera, E., Bartoloni, E., Berti, A., Bugatti, S., Camellino, D., Cammelli, D., Caporali, R., Caso, F., Cavallaro, E., Cavalli, G., Colaci, M., Costa, L., Di Scala, G., Emmi, G., Frassi, M., Gerli, R., Giacomelli, R., Gremese, E., Iannone, F., Lapadula, G., Leveghi, L., Lopalco, G., Manna, R., Marotto, D., Mathieu, A., Neri, R., Patisso, I., Piga, M., Punzi, L., Romano, M., Ruscitti, P., Salvarani, C., Scarpa, R., Scrivo, R., Talarico, R., Verrecchia, E., Viapiana, O., Vitale, A., and Vitiello, G.

Abstract

Following publication of the original article [1], it was brought to our attention that the AOSD Consensus Group was incorrectly tagged and therefore not searchable. The publishers apologize for this error.

Published
2020

25. Risk of acute arterial and venous thromboembolic events in Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)

Author

Bettiol, A, Sinico, R, Schiavon, F, Monti, S, Bozzolo, E, Franceschini, F, Govoni, M, Lunardi, C, Guida, G, Lopalco, G, Paolazzi, G, Vacca, A, Gregorini, G, Leccese, P, Piga, M, Conti, F, Fraticelli, P, Quartuccio, L, Alberici, F, Salvarani, C, Bettio, S, Negrini, S, Selmi, C, Sciascia, S, Moroni, G, Colla, L, Manno, C, Urban, M, Vannacci, A, Pozzi, M, Fabbrini, P, Polti, S, Felicetti, M, Marchi, M, Padoan, R, Delvino, P, Caporali, R, Montecucco, C, Dagna, L, Cariddi, A, Toniati, P, Tamanini, S, Furini, F, Bortoluzzi, A, Tinazzi, E, Delfino, L, Badiu, I, Rolla, G, Venerito, V, Iannone, F, Berti, A, Bortolotti, R, Racanelli, V, Jeannin, G, Padula, A, Cauli, A, Priori, R, Gabrielli, A, Bond, M, Tedesco, M, Pazzola, G, Tomietto, P, Pellecchio, M, Marvisi, C, Maritati, F, Palmisano, A, Dejaco, C, Willeit, J, Kiechl, S, Olivotto, I, Willeit, P, Prisco, D, Vaglio, A, Emmi, G, Bettiol, Alessandra, Sinico, Renato Alberto, Schiavon, Franco, Monti, Sara, Bozzolo, Enrica Paola, Franceschini, Franco, Govoni, Marcello, Lunardi, Claudio, Guida, Giuseppe, Lopalco, Giuseppe, Paolazzi, Giuseppe, Vacca, Angelo, Gregorini, Gina, Leccese, Pietro, Piga, Matteo, Conti, Fabrizio, Fraticelli, Paolo, Quartuccio, Luca, Alberici, Federico, Salvarani, Carlo, Bettio, Silvano, Negrini, Simone, Selmi, Carlo, Sciascia, Savino, Moroni, Gabriella, Colla, Loredana, Manno, Carlo, Urban, Maria Letizia, Vannacci, Alfredo, Pozzi, Maria Rosa, Fabbrini, Paolo, Polti, Stefano, Felicetti, Mara, Marchi, Maria Rita, Padoan, Roberto, Delvino, Paolo, Caporali, Roberto, Montecucco, Carlomaurizio, Dagna, Lorenzo, Cariddi, Adriana, Toniati, Paola, Tamanini, Silvia, Furini, Federica, Bortoluzzi, Alessandra, Tinazzi, Elisa, Delfino, Lorenzo, Badiu, Iuliana, Rolla, Giovanni, Venerito, Vincenzo, Iannone, Florenzo, Berti, Alvise, Bortolotti, Roberto, Racanelli, Vito, Jeannin, Guido, Padula, Angela, Cauli, Alberto, Priori, Roberta, Gabrielli, Armando, Bond, Milena, Tedesco, Martina, Pazzola, Giulia, Tomietto, Paola, Pellecchio, Marco, Marvisi, Chiara, Maritati, Federica, Palmisano, Alessandra, Dejaco, Christian, Willeit, Johann, Kiechl, Stefan, Olivotto, Iacopo, Willeit, Peter, Prisco, Domenico, Vaglio, Augusto, Emmi, Giacomo, Bettiol, A, Sinico, R, Schiavon, F, Monti, S, Bozzolo, E, Franceschini, F, Govoni, M, Lunardi, C, Guida, G, Lopalco, G, Paolazzi, G, Vacca, A, Gregorini, G, Leccese, P, Piga, M, Conti, F, Fraticelli, P, Quartuccio, L, Alberici, F, Salvarani, C, Bettio, S, Negrini, S, Selmi, C, Sciascia, S, Moroni, G, Colla, L, Manno, C, Urban, M, Vannacci, A, Pozzi, M, Fabbrini, P, Polti, S, Felicetti, M, Marchi, M, Padoan, R, Delvino, P, Caporali, R, Montecucco, C, Dagna, L, Cariddi, A, Toniati, P, Tamanini, S, Furini, F, Bortoluzzi, A, Tinazzi, E, Delfino, L, Badiu, I, Rolla, G, Venerito, V, Iannone, F, Berti, A, Bortolotti, R, Racanelli, V, Jeannin, G, Padula, A, Cauli, A, Priori, R, Gabrielli, A, Bond, M, Tedesco, M, Pazzola, G, Tomietto, P, Pellecchio, M, Marvisi, C, Maritati, F, Palmisano, A, Dejaco, C, Willeit, J, Kiechl, S, Olivotto, I, Willeit, P, Prisco, D, Vaglio, A, Emmi, G, Bettiol, Alessandra, Sinico, Renato Alberto, Schiavon, Franco, Monti, Sara, Bozzolo, Enrica Paola, Franceschini, Franco, Govoni, Marcello, Lunardi, Claudio, Guida, Giuseppe, Lopalco, Giuseppe, Paolazzi, Giuseppe, Vacca, Angelo, Gregorini, Gina, Leccese, Pietro, Piga, Matteo, Conti, Fabrizio, Fraticelli, Paolo, Quartuccio, Luca, Alberici, Federico, Salvarani, Carlo, Bettio, Silvano, Negrini, Simone, Selmi, Carlo, Sciascia, Savino, Moroni, Gabriella, Colla, Loredana, Manno, Carlo, Urban, Maria Letizia, Vannacci, Alfredo, Pozzi, Maria Rosa, Fabbrini, Paolo, Polti, Stefano, Felicetti, Mara, Marchi, Maria Rita, Padoan, Roberto, Delvino, Paolo, Caporali, Roberto, Montecucco, Carlomaurizio, Dagna, Lorenzo, Cariddi, Adriana, Toniati, Paola, Tamanini, Silvia, Furini, Federica, Bortoluzzi, Alessandra, Tinazzi, Elisa, Delfino, Lorenzo, Badiu, Iuliana, Rolla, Giovanni, Venerito, Vincenzo, Iannone, Florenzo, Berti, Alvise, Bortolotti, Roberto, Racanelli, Vito, Jeannin, Guido, Padula, Angela, Cauli, Alberto, Priori, Roberta, Gabrielli, Armando, Bond, Milena, Tedesco, Martina, Pazzola, Giulia, Tomietto, Paola, Pellecchio, Marco, Marvisi, Chiara, Maritati, Federica, Palmisano, Alessandra, Dejaco, Christian, Willeit, Johann, Kiechl, Stefan, Olivotto, Iacopo, Willeit, Peter, Prisco, Domenico, Vaglio, Augusto, and Emmi, Giacomo

Published
2021

26. Early Lupus Project: one-year follow-up of an Italian cohort of patients with systemic lupus erythematosus of recent onset

Author

Sebastiani, G, Prevete, I, Iuliano, A, Piga, M, Iannone, F, Coladonato, L, Govoni, M, Bortoluzzi, A, Mosca, M, Tani, C, Doria, A, Iaccarino, L, Tincani, A, Fredi, M, Conti, F, Spinelli, F, Galeazzi, M, Bellisai, F, Zanetti, A, Carrara, G, Scire, C, Mathieu, A, Sebastiani G. D., Prevete I., Iuliano A., Piga M., Iannone F., Coladonato L., Govoni M., Bortoluzzi A., Mosca M., Tani C., Doria A., Iaccarino L., Tincani A., Fredi M., Conti F., Spinelli F. R., Galeazzi M., Bellisai F., Zanetti A., Carrara G., Scire C. A., Mathieu A., Sebastiani, G, Prevete, I, Iuliano, A, Piga, M, Iannone, F, Coladonato, L, Govoni, M, Bortoluzzi, A, Mosca, M, Tani, C, Doria, A, Iaccarino, L, Tincani, A, Fredi, M, Conti, F, Spinelli, F, Galeazzi, M, Bellisai, F, Zanetti, A, Carrara, G, Scire, C, Mathieu, A, Sebastiani G. D., Prevete I., Iuliano A., Piga M., Iannone F., Coladonato L., Govoni M., Bortoluzzi A., Mosca M., Tani C., Doria A., Iaccarino L., Tincani A., Fredi M., Conti F., Spinelli F. R., Galeazzi M., Bellisai F., Zanetti A., Carrara G., Scire C. A., and Mathieu A.

Abstract

Objective: To describe the clinical and serological features of a prospectively followed cohort of early diagnosed systemic lupus erythematosus (SLE) patients during a one-year follow-up period. Methods: SLE patients with disease duration less than 12 months were consecutively enrolled in a multicentre, prospective study. At study entry and then every 6 months, a large panel of data was recorded. Results: Of 260 patients enrolled, 185 had at least 12 months of follow-up; of these, 84.3% were female, 92.4% were Caucasians. Mean diagnostic delay was about 20 months; higher values of European Consensus Lupus Activity Measurement (ECLAM) and of organs/systems involved were both associated with shorter diagnostic delay. Clinical and serological parameters improved after study entry. However, patients' quality of life deteriorated and cardiovascular risk factors significantly increased. About one-third of patients with active disease at study entry went into remission (ECLAM = 0). Negative predictors for remission were: oral ulcers, arthritis, low C4, anti-SSB (Ro) antibodies and therapy with mycophenolate. There was a widespread use of glucocorticoids both at baseline and during follow-up. Conclusion: Clinical symptoms and serological parameters improve during the first period after diagnosis. However, patients’ quality of life deteriorates. The widespread use of glucocorticoids is probably the reason for the early significant increase of some cardiovascular risk factors.

Published
2018

27. Clinical and ultrasonographic predictors for achieving minimal disease activity in patients with psoriatic arthritis: The UPSTREAM (Ultrasound in PSoriatic arthritis TREAtMent) prospective observational study protocol

Author

Canzoni, M, Piga, M, Zabotti, A, Scire, C, Carrara, G, Olivieri, I, Iagnocco, A, Canzoni M., Piga M., Zabotti A., Scire C. A., Carrara G., Olivieri I., Iagnocco A., Canzoni, M, Piga, M, Zabotti, A, Scire, C, Carrara, G, Olivieri, I, Iagnocco, A, Canzoni M., Piga M., Zabotti A., Scire C. A., Carrara G., Olivieri I., and Iagnocco A.

Abstract

Introduction Psoriatic arthritis (PsA) occurs in 10%-15% of people with psoriasis and accounts for 10%-20% of early arthritis clinics referral. Only a few prognostic factors of therapeutic response in patients with PsA have been identified. In the last years, the role of imaging has grown up and the European League Against Rheumatism recognised that ultrasound (US) has higher sensitivity than clinical examination to detect inflammatory disease activity. The aims of the Ultrasound in PSoriatic arthritis TREAtMent (UPSTREAM) study are to integrate clinic and US in order to inform whether US has provide an added prognostic value in PsA. Methods and analysis UPSTREAM is an observational prospective cohort study enrolling patients with PsA having clinically active joint disease and starting a new course of therapy. The primary objective is to evaluate the additional value of US over clinical examination in detecting patients achieving minimal disease activity after 6 months. Data will be obtained at baseline and at standard clinical follow-up visits. Patient's clinical assessment will be performed according to the core set proposed by the Group for Research and Assessment of Psoriasis and Psoriatic Arthritis-Outcome Measures in Rheumatology. Sonographic evaluations will be performed by expert sonographers in 42 joints, 36 tendons, 12 entheses and 2 bursae, according to a score that will be purposely developed for PsA by the US Study Group of the Italian Society for Rheumatology. The UPSTREAM study will identify clinical and US predictors of response to treatment in patients with PsA and active peripheral arthritis starting a new course of therapy. Ethics and dissemination Ethic approval for this study has been obtained from the institutional review board (IRB)/independent ethics committee (IEC) Comitato Etico Lazio 1 (Prot. N 198 02-02-2017) and then locally from the IRB/IEC of each participating centre. Results will be published in relevant scientific journals and be di

Published
2018

28. Ultrasonography in psoriatic arthritis: Which sites should we scan?

Author

Zabotti, A, Piga, M, Canzoni, M, Sakellariou, G, Iagnocco, A, Scire, C, Adinolfi, A, Azzolin, I, Bandinelli, F, Batticciotto, A, Boffini, N, Bortoluzzi, A, Carrara, G, Cavatorta, F, Cagnotto, G, Caprioli, M, Colaci, M, De Lucia, O, Delle Sedie, A, Denaro, V, Di Matteo, A, Di Sabatino, V, Epis, O, Hoxha, A, Farina, I, Germano, G, Filippou, G, Filippucci, E, Focherini, M, Gabba, A, Idolazzi, L, La Paglia, G, Luccioli, F, Macchioni, P, Magnani, M, Massarotti, M, Mastaglio, C, Navarini, L, Parisi, S, Diamanti, A, Picerno, V, Piras, M, Porta, F, Possemato, N, Prevete, I, Raffeiner, B, Ramonda, R, Ravagnani, V, Rossi, D, Rossi, S, Scolieri, P, Santoboni, G, Scioscia, C, Terenzi, R, Tinazzi, I, Toscano, C, Venditti, C, Volpe, A, Vukatana, G, Zanframundo, G, Zabotti A., Piga M., Canzoni M., Sakellariou G., Iagnocco A., Scire C. A., Adinolfi A., Azzolin I., Bandinelli F., Batticciotto A., Boffini N., Bortoluzzi A., Carrara G., Cavatorta F. P., Cagnotto G., Caprioli M., Colaci M., De Lucia O., Delle Sedie A., Denaro V., Di Matteo A., Di Sabatino V., Epis O. M., Hoxha A., Farina I., Germano G., Filippou G., Filippucci E., Focherini M. C., Gabba A., Idolazzi L., La Paglia G. M. C., Luccioli F., Macchioni P., Magnani M., Massarotti M., Mastaglio C., Navarini L., Parisi S., Diamanti A. P., Picerno V., Piras M., Porta F., Possemato N., Prevete I., Raffeiner B., Ramonda R., Ravagnani V., Rossi D., Rossi S., Scolieri P., Santoboni G., Scioscia C., Terenzi R., Tinazzi I., Toscano C., Venditti C., Volpe A., Vukatana G., Zanframundo G., Zabotti, A, Piga, M, Canzoni, M, Sakellariou, G, Iagnocco, A, Scire, C, Adinolfi, A, Azzolin, I, Bandinelli, F, Batticciotto, A, Boffini, N, Bortoluzzi, A, Carrara, G, Cavatorta, F, Cagnotto, G, Caprioli, M, Colaci, M, De Lucia, O, Delle Sedie, A, Denaro, V, Di Matteo, A, Di Sabatino, V, Epis, O, Hoxha, A, Farina, I, Germano, G, Filippou, G, Filippucci, E, Focherini, M, Gabba, A, Idolazzi, L, La Paglia, G, Luccioli, F, Macchioni, P, Magnani, M, Massarotti, M, Mastaglio, C, Navarini, L, Parisi, S, Diamanti, A, Picerno, V, Piras, M, Porta, F, Possemato, N, Prevete, I, Raffeiner, B, Ramonda, R, Ravagnani, V, Rossi, D, Rossi, S, Scolieri, P, Santoboni, G, Scioscia, C, Terenzi, R, Tinazzi, I, Toscano, C, Venditti, C, Volpe, A, Vukatana, G, Zanframundo, G, Zabotti A., Piga M., Canzoni M., Sakellariou G., Iagnocco A., Scire C. A., Adinolfi A., Azzolin I., Bandinelli F., Batticciotto A., Boffini N., Bortoluzzi A., Carrara G., Cavatorta F. P., Cagnotto G., Caprioli M., Colaci M., De Lucia O., Delle Sedie A., Denaro V., Di Matteo A., Di Sabatino V., Epis O. M., Hoxha A., Farina I., Germano G., Filippou G., Filippucci E., Focherini M. C., Gabba A., Idolazzi L., La Paglia G. M. C., Luccioli F., Macchioni P., Magnani M., Massarotti M., Mastaglio C., Navarini L., Parisi S., Diamanti A. P., Picerno V., Piras M., Porta F., Possemato N., Prevete I., Raffeiner B., Ramonda R., Ravagnani V., Rossi D., Rossi S., Scolieri P., Santoboni G., Scioscia C., Terenzi R., Tinazzi I., Toscano C., Venditti C., Volpe A., Vukatana G., and Zanframundo G.

Published
2018

29. The predictive role of ultrasound-detected tenosynovitis and joint synovitis for flare in patients with rheumatoid arthritis in stable remission. Results of an Italian multicentre study of the Italian Society for Rheumatology Group for Ultrasound: The STARTER study

Author

Filippou, G, Sakellariou, G, Scire, C, Carrara, G, Rumi, F, Bellis, E, Adinolfi, A, Batticciotto, A, Bortoluzzi, A, Cagnotto, G, Caprioli, M, Canzoni, M, Cavatorta, F, De Lucia, O, Di Sabatino, V, Draghessi, A, Farina, I, Focherini, M, Gabba, A, Gutierrez, M, Idolazzi, L, Luccioli, F, Macchioni, P, Massarotti, M, Mastaglio, C, Menza, L, Muratore, M, Parisi, S, Picerno, V, Piga, M, Ramonda, R, Raffeiner, B, Rossi, D, Rossi, S, Rossini, P, Scioscia, C, Venditti, C, Volpe, A, Iagnocco, A, Filippou G., Sakellariou G., Scire C. A., Carrara G., Rumi F., Bellis E., Adinolfi A., Batticciotto A., Bortoluzzi A., Cagnotto G., Caprioli M., Canzoni M., Cavatorta F. P., De Lucia O., Di Sabatino V., Draghessi A., Farina I., Focherini M. C., Gabba A., Gutierrez M., Idolazzi L., Luccioli F., MacChioni P., Massarotti M. S., Mastaglio C., Menza L., Muratore M., Parisi S., Picerno V., Piga M., Ramonda R., Raffeiner B., Rossi D., Rossi S., Rossini P., Scioscia C., Venditti C., Volpe A., Iagnocco A., Filippou, G, Sakellariou, G, Scire, C, Carrara, G, Rumi, F, Bellis, E, Adinolfi, A, Batticciotto, A, Bortoluzzi, A, Cagnotto, G, Caprioli, M, Canzoni, M, Cavatorta, F, De Lucia, O, Di Sabatino, V, Draghessi, A, Farina, I, Focherini, M, Gabba, A, Gutierrez, M, Idolazzi, L, Luccioli, F, Macchioni, P, Massarotti, M, Mastaglio, C, Menza, L, Muratore, M, Parisi, S, Picerno, V, Piga, M, Ramonda, R, Raffeiner, B, Rossi, D, Rossi, S, Rossini, P, Scioscia, C, Venditti, C, Volpe, A, Iagnocco, A, Filippou G., Sakellariou G., Scire C. A., Carrara G., Rumi F., Bellis E., Adinolfi A., Batticciotto A., Bortoluzzi A., Cagnotto G., Caprioli M., Canzoni M., Cavatorta F. P., De Lucia O., Di Sabatino V., Draghessi A., Farina I., Focherini M. C., Gabba A., Gutierrez M., Idolazzi L., Luccioli F., MacChioni P., Massarotti M. S., Mastaglio C., Menza L., Muratore M., Parisi S., Picerno V., Piga M., Ramonda R., Raffeiner B., Rossi D., Rossi S., Rossini P., Scioscia C., Venditti C., Volpe A., and Iagnocco A.

Abstract

Objective To define the role of ultrasound (US) for the assessment of patients with rheumatoid arthritis (RA) in clinical remission, including joint and tendon evaluation. Methods A multicentre longitudinal study has been promoted by the US Study Group of the Italian Society for Rheumatology. 25 Italian centres participated, enrolling consecutive patients with RA in clinical remission. All patients underwent complete clinical assessment (demographic data, disease characteristics, laboratory exams, clinical assessment of 28 joints and patient/physician-reported outcomes) and Power Doppler (PD) US evaluation of wrist, metacarpalphalangeal joints, proximal interphalangeal joints and synovial tendons of the hands and wrists at enrolment, 6 and 12 months. The association between clinical and US variables with flare, disability and radiographic progression was evaluated by univariable and adjusted logistic regression models. Results 361 patients were enrolled, the mean age was 56.20 (±13.31) years and 261 were women, with a mean disease duration of 9.75 (±8.07) years. In the 12 months follow-up, 98/326 (30.1%) patients presented a disease flare. The concurrent presence of PD positive tenosynovitis and joint synovitis predicted disease flare, with an OR (95% CI) of 2.75 (1.45 to 5.20) in crude analyses and 2.09 (1.06 to 4.13) in adjusted analyses. US variables did not predict the worsening of function or radiographic progression. US was able to predict flare at 12 months but not at 6 months. Conclusions PD positivity in tendons and joints is an independent risk factor of flare in patients with RA in clinical remission. Musculoskeletal ultrasound evaluation is a valuable tool to monitor and help decision making in patients with RA in clinical remission.

Published
2018

30. Discordance between patient and physician global assessment of disease activity in Behçet's syndrome: a multicenter study cohort

Author

Floris, A., Espinosa, G., Serpa Pinto, L., Kougkas, N., Lo Monaco, A., Lopalco, G., Orlando, I., Bertsias, G., Cantarini, L., Cervera, R., Correia, J., Govoni, M., Iannone, F., Mathieu, A., Neri, P., Martins Silva, A., Vasconcelos, C., Muntoni, M., Cauli, A., Piga, M., Avgoustidis, N., Cangemi, I., Chessa, E., Congia, M., D'Amico, M. E., Faria, R., Lledo, G. M., Pirani, V., Rios-Garces, R., Santos, E., Venerito, V., and Vitale, A.

Subjects
Adult, medicine.medical_specialty, Malaltia de Behçet, Visual analogue scale, Severity of Illness Index, NO, Disease activity, Outcome measure, Internal medicine, Physicians, Assistència sanitària, medicine, Humans, Patients reported outcomes, S syndrome, Medical care, Greece, Behçet's disease, business.industry, Behçet’s syndrome, Behcet Syndrome, Odds ratio, Middle Aged, Rheumatology, Multicenter study, Italy, Spain, Cohort, business, Inactive disease, Research Article
Abstract

Background To compare the patients’ and physician’s global assessment of disease activity in Behçet’s syndrome (BS) and investigate the frequency, magnitude, and determinants of potential discordance. Methods A total of 226 adult BS patients with a median (IQR) age of 46.9 (35.6–55.2) years were enrolled across Italy, Greece, Portugal, and Spain. Demographic, clinical, and therapeutic variables, as well as the patient reported outcomes, were collected at the recruitment visit. The physical (PCS) and mental (MCS) component summary scores of the Short Form Questionnaire 36 (SF-36) and the Behçet’s syndrome Overall Damage Index (BODI) were calculated. Disease activity was assessed by the patients’ (PtGA) and physician’s global assessment (PGA) in a 10-cm visual analog scale, as well as the Behçet Disease Current Activity Form (BDCAF). Discordance (∆) was calculated by subtracting the PGA from the PtGA and defined as positive (PtGA>PGA) and negative (PtGA Results Median PtGA and PGA scores were 2.0 (0.3–5.0) and 1.0 (0.0–3.0) cm, respectively. The discordance prevalence varied (from 29.6 to 55.3%) according to the cutoff applied, and the majority (> 80%) of disagreements were due to patients rating higher their disease activity. Higher values of BDCAF were associated to increased rate of positive discordance. When BDCAF = 0, the median (IQR) values of PtGA and PGA were 0.2 (0–2) and 0 (0–1), respectively. PCS (adjusted odds ratio (adjOR) 0.96 per unit, 95% CI 0.93–0.98, p = 0.006) and MCS (adjOR 0.96 per unit, 95% CI 0.93–0.99, p = 0.003) were independently associated with positive discordance using both cutoffs. Active ocular involvement emerged as a potential determinant of negative discordance (adjOR 5.88, 95% CI 1.48–23.30, p = 0.012). Conclusions PtGA and PGA should be considered as complementary measures in BS, as patients and physicians may be influenced by different factors when assessing active disease manifestations. Particularly, PtGA may be a useful tool in the assessment of BS disease activity, as it carries a low risk to misclassify an inactive disease, and may allow to capture aspects of the patient’s health that negatively affect his well-being and the treatment.

Published
2020

31. Erratum. management of adult-onset still's disease with interleukin-1 inhibitors. evidence- and consensus-based statements by a panel of Italian experts

Author

Colafrancesco, S., Manara, M., Bortoluzzi, A., Serban, T., Bianchi, G., Cantarini, L., Ciccia, F., Dagna, L., Govoni, M., Montecucco, C., Priori, R., Ravelli, A., Sfriso, P., Sinigaglia, L., Alivernini, S., Baldissera, E., Bartoloni, E., Berti, A., Bugatti, S., Camellino, D., Cammelli, D., Caporali, R., Caso, F., Cavallaro, E., Cavalli, G., Colaci, M., Costa, L., Di Scala, G., Emmi, G., Frassi, M., Gerli, R., Giacomelli, R., Gremese, E., Iannone, F., Lapadula, G., Leveghi, L., Lopalco, G., Manna, R., Marotto, D., Mathieu, A., Neri, R., Patisso, I., Piga, M., Punzi, L., Romano, M., Ruscitti, P., Salvarani, C., Scarpa, R., Scrivo, R., Talarico, R., Verrecchia, E., Viapiana, O., Vitale, A., and Vitiello, G.

Subjects
030203 arthritis & rheumatology, 0301 basic medicine, medicine.medical_specialty, Pediatrics, Adult-onset Still's disease, lcsh:Diseases of the musculoskeletal system, business.industry, Published Erratum, MEDLINE, Interleukin, Rheumatology, 03 medical and health sciences, 030104 developmental biology, 0302 clinical medicine, Internal medicine, medicine, lcsh:RC925-935, business
Abstract

Following publication of the original article [1], it was brought to our attention that the AOSD Consensus Group was incorrectly tagged and therefore not searchable. The publishers apologize for this error.

Published
2020

32. Linee di indirizzo operative per la conciliazione delle attività sanitarie e giudiziarie nell'ambito dei trapianti di organi e tessuti a scopo terapeutico: 'PROCUREMENT e PROCURE', Centro Nazionale Trapianti

Author

Aurelio, MT, Aurelio, M, Balestra, L, Bettoni, C, Cardillo, M, Cerri, N, Cossolini, M, De Feo, T, Dettori, G, Ferri, F, La Torraca, R, Marchesi, M, Milanesi, F, Pea, E, Persutti, R, Piccolo, G, Piga, M, Scagliusi, M, Siciliano, T, Terenghi, P, Morgutti, M, Vescovi, S, Aurelio, MT, De Feo, TM, Milanesi, FC, Piga, MA, Aurelio, MT, Aurelio, M, Balestra, L, Bettoni, C, Cardillo, M, Cerri, N, Cossolini, M, De Feo, T, Dettori, G, Ferri, F, La Torraca, R, Marchesi, M, Milanesi, F, Pea, E, Persutti, R, Piccolo, G, Piga, M, Scagliusi, M, Siciliano, T, Terenghi, P, Morgutti, M, Vescovi, S, Aurelio, MT, De Feo, TM, Milanesi, FC, and Piga, MA

Published
2020

33. Comparative study between two European inception cohorts of patients with early systemic lupus erythematosus

Author

Prevete, I, Espinosa, G, Bellisai, F, Bortoluzzi, A, Conti, F, Fredi, M, Fonseca-Aizpuru, E, de Viedma, V, Gonzalez-Garcia, A, Gonzalez-Leon, R, Iaccarino, L, Iannone, F, Marin-Ballve, A, Mitjavila, F, Pallares, L, Piga, M, Rios-Garces, R, Suarez, S, Tani, C, Zanetti, A, Ruiz-Irastorza, G, Sebastiani, G, Fonseca-Aizpuru, EM, de Viedma, VG, Sebastiani, GD, Prevete, I, Espinosa, G, Bellisai, F, Bortoluzzi, A, Conti, F, Fredi, M, Fonseca-Aizpuru, E, de Viedma, V, Gonzalez-Garcia, A, Gonzalez-Leon, R, Iaccarino, L, Iannone, F, Marin-Ballve, A, Mitjavila, F, Pallares, L, Piga, M, Rios-Garces, R, Suarez, S, Tani, C, Zanetti, A, Ruiz-Irastorza, G, Sebastiani, G, Fonseca-Aizpuru, EM, de Viedma, VG, and Sebastiani, GD

Abstract

Objective To compare the main characteristics of two inception cohorts (Italian [ITC] and Spanish [SPC]) cohorts of patients with systemic lupus erythematosus (SLE) at the time of diagnosis and at one year of follow-up. Methods Demographic, clinical and immunological characteristics, and treatments at SLE diagnosis and at 12 months of follow-up of ITC and SPC were compared. Results One hundred and sixty-four patients in the ITC and 231 patients in the SPC were compared. the patients from ITC were younger at SLE diagnosis (41.1±15.0 years vs. 46.4±15.6 years; p<0.001) and had a higher prevalence of arthritis (62.8% vs. 45.5%; p=0.001), serositis (25.6% vs. 16.0%; p=0.026), neurological involvement (7.9% vs. 1.7%; p=0.006), and immunological abnormalities (anti-dsDNA, anti-Sm, antiphospholipid antibodies) (93.9% vs. 77.8%; p<0.001). Conversely, photosensitivity (29.5% in ITC vs. 45.9% in SPC; p=0.001) and oral ulcers (12.4% vs. 30.3%; p<0.001) were more frequent at onset of SLE in the Spanish patients. At the first 12 months of follow-up, these differences were maintained. At SLE onset, more Italian patients received glucocorticoids (85.4% vs. 50.2%; p<0.001) and immunosuppressive agents. At 12 months of follow-up, more Spanish patients were treated with antimalarials (75.6% in ITC vs. 90.0% in SPC; p<0.001). Conversely, the use of glucocorticoids was lower in SPC (89.0% in ITC vs. 57.1% in SPC; p<0.001). Conclusion These cohorts presented different profiles in terms of pattern of organ/system involvement and disease treatment, possibly as a consequence of patient selection or different disease management approaches between Italy and Spain.

Published
2020

34. Comparison of early vs. delayed anakinra treatment in patients with adult onset Still's disease and effect on clinical and laboratory outcomes

Author

Vitale, A, Cavalli, G, Ruscitti, P, Sota, J, Colafrancesco, S, Priori, R, Valesini, G, Argolini, Lm, Baldissera, E, Bartoloni, E, Cammelli, D, Canestrari, G, Cavallaro, E, Massaro, Mg, Cipriani, P, De Marchi, G, De Vita, S, Emmi, G, Frassi, M, Gerli, R, Gremese, Elisa, Iannone, F, Fornaro, M, Paladini, A, Lopalco, G, Manna, Raffaele, Mathieu, A, Montecucco, C, Mosca, M, Piazza, I, Piga, M, Pontikaki, I, Romano, M, Rossi, S, Rossini, M, Silvestri, E, Stagnaro, C, Talarico, R, Frediani, B, Tincani, A, Viapiana, O, Vitiello, G, Galozzi, P, Sfriso, P, Gaggiano, C, Grosso, S, Rigante, Donato, Dagna, L, Giacomelli, R, Cantarini, L., Gremese E (ORCID:0000-0002-2248-1058), Manna R (ORCID:0000-0003-1560-3907), Rigante D (ORCID:0000-0001-7032-7779), Vitale, A, Cavalli, G, Ruscitti, P, Sota, J, Colafrancesco, S, Priori, R, Valesini, G, Argolini, Lm, Baldissera, E, Bartoloni, E, Cammelli, D, Canestrari, G, Cavallaro, E, Massaro, Mg, Cipriani, P, De Marchi, G, De Vita, S, Emmi, G, Frassi, M, Gerli, R, Gremese, Elisa, Iannone, F, Fornaro, M, Paladini, A, Lopalco, G, Manna, Raffaele, Mathieu, A, Montecucco, C, Mosca, M, Piazza, I, Piga, M, Pontikaki, I, Romano, M, Rossi, S, Rossini, M, Silvestri, E, Stagnaro, C, Talarico, R, Frediani, B, Tincani, A, Viapiana, O, Vitiello, G, Galozzi, P, Sfriso, P, Gaggiano, C, Grosso, S, Rigante, Donato, Dagna, L, Giacomelli, R, Cantarini, L., Gremese E (ORCID:0000-0002-2248-1058), Manna R (ORCID:0000-0003-1560-3907), and Rigante D (ORCID:0000-0001-7032-7779)

Abstract

Background: Aim of this study was to search for any difference in the outcome of patients with adult onset Still's disease (AOSD) treated with anakinra (ANK) in relation with the interval between disease onset and the start of anti-interleukin(IL)-1 treatment and according with the different lines of ANK treatment. Patients and Methods: One hundred and forty-one AOSD patients treated with ANK have been retrospectively assessed. Statistically significant differences (p < 0.05) were analyzed in the frequency of ANK effectiveness, primary or secondary inefficacy to ANK and rate of resolution of clinical and laboratory AOSD manifestations after 3, 6, and 12 months since ANK treatment according with different lines of treatment and different times between AOSD onset and start of ANK. Results: No significant differences were identified in the ANK effectiveness and frequency of primary or secondary inefficacy for patients starting ANK within 6 months (p = 0.19, p = 0.14, and p = 0.81, respectively) or 12 months (p = 0.37, p = 0.23, and p = 0.81, respectively) since AOSD onset compared with patients starting ANK thereafter; no significant differences were identified in ANK effectiveness and primary or secondary inefficacy according with different lines of ANK treatment (p = 0.06, p = 0.19, and p = 0.13, respectively). Patients starting ANK within 6 and 12 months since AOSD onset showed a significantly quicker decrease of erythrocyte sedimentation rate and C-reactive protein than observed among patients undergoing ANK treatment after 6 and 12 months. The number of swollen joints at the 3 month follow-up visit was significantly lower among patients undergoing ANK within 6 months since AOSD onset (p = 0.01), while no significance was identified at the 6 and 12 month assessments (p = 0.23 and p = 0.45, respectively). At the 3 and 6 month visits, the number of swollen joints was significantly higher among patients previously treated with conventional and biological disease mo

Published
2020

35. Effect of carotid image-based phenotypes on cardiovascular risk calculator: AECRS1.0

Author

Khanna, N.N. Jamthikar, A.D. Gupta, D. Araki, T. Piga, M. Saba, L. Carcassi, C. Nicolaides, A. Laird, J.R. Suri, H.S. Gupta, A. Mavrogeni, S. Protogerou, A. Sfikakis, P. Kitas, G.D. Suri, J.S.

Abstract

Today, the 10-year cardiovascular risk largely relies on conventional cardiovascular risk factors (CCVRFs) and suffers from the effect of atherosclerotic wall changes. In this study, we present a novel risk calculator AtheroEdge Composite Risk Score (AECRS1.0), designed by fusing CCVRF with ultrasound image-based phenotypes. Ten-year risk was computed using the Framingham Risk Score (FRS), United Kingdom Prospective Diabetes Study 56 (UKPDS56), UKPDS60, Reynolds Risk Score (RRS), and pooled composite risk (PCR) score. AECRS1.0 was computed by measuring the 10-year five carotid phenotypes such as IMT (ave., max., min.), IMT variability, and total plaque area (TPA) by fusing eight CCVRFs and then compositing them. AECRS1.0 was then benchmarked against the five conventional cardiovascular risk calculators by computing the receiver operating characteristics (ROC) and area under curve (AUC) values with a 95% CI. Two hundred four IRB-approved Japanese patients’ left/right common carotid arteries (407 ultrasound scans) were collected with a mean age of 69 ± 11 years. The calculators gave the following AUC: FRS, 0.615; UKPDS56, 0.576; UKPDS60, 0.580; RRS, 0.590; PCRS, 0.613; and AECRS1.0, 0.990. When fusing CCVRF, TPA reported the highest AUC of 0.81. The patients were risk-stratified into low, moderate, and high risk using the standardized thresholds. The AECRS1.0 demonstrated the best performance on a Japanese diabetes cohort when compared with five conventional calculators. [Figure not available: see fulltext.]. © 2019, International Federation for Medical and Biological Engineering.

Published
2019

36. Rheumatoid Arthritis: Atherosclerosis Imaging and Cardiovascular Risk Assessment Using Machine and Deep Learning–Based Tissue Characterization

Author

Khanna, N.N. Jamthikar, A.D. Gupta, D. Piga, M. Saba, L. Carcassi, C. Giannopoulos, A.A. Nicolaides, A. Laird, J.R. Suri, H.S. Mavrogeni, S. Protogerou, A.D. Sfikakis, P. Kitas, G.D. Suri, J.S.

Abstract

Purpose of the Review: Rheumatoid arthritis (RA) is a chronic, autoimmune disease which may result in a higher risk of cardiovascular (CV) events and stroke. Tissue characterization and risk stratification of patients with rheumatoid arthritis are a challenging problem. Risk stratification of RA patients using traditional risk factor–based calculators either underestimates or overestimates the CV risk. Advancements in medical imaging have facilitated early and accurate CV risk stratification compared to conventional cardiovascular risk calculators. Recent Finding: In recent years, a link between carotid atherosclerosis and rheumatoid arthritis has been widely discussed by multiple studies. Imaging the carotid artery using 2-D ultrasound is a noninvasive, economic, and efficient imaging approach that provides an atherosclerotic plaque tissue–specific image. Such images can help to morphologically characterize the plaque type and accurately measure vital phenotypes such as media wall thickness and wall variability. Intelligence-based paradigms such as machine learning– and deep learning–based techniques not only automate the risk characterization process but also provide an accurate CV risk stratification for better management of RA patients. Summary: This review provides a brief understanding of the pathogenesis of RA and its association with carotid atherosclerosis imaged using the B-mode ultrasound technique. Lacunas in traditional risk scores and the role of machine learning–based tissue characterization algorithms are discussed and could facilitate cardiovascular risk assessment in RA patients. The key takeaway points from this review are the following: (i) inflammation is a common link between RA and atherosclerotic plaque buildup, (ii) carotid ultrasound is a better choice to characterize the atherosclerotic plaque tissues in RA patients, and (iii) intelligence-based paradigms are useful for accurate tissue characterization and risk stratification of RA patients. © 2019, Springer Science+Business Media, LLC, part of Springer Nature.

Published
2019

37. Peripheral nervous system involvement in systemic lupus erythematosus: a review of the evidence

Author

Bortoluzzi, A., Silvagni, E., Federica Furini, Piga, M., and Govoni, M.

Subjects
Systemic lupus erythematosus, peripheral neuropathy, Humans, Lupus Erythematosus, Systemic, Peripheral Nervous System Diseases, Peripheral nervous system, Peripheral nervous system, Systemic lupus erythematosus, peripheral neuropathy, NO
Abstract

In the past years the peripheral nervous system (PNS) involvement in systemic lupus erythematosus (SLE) has received little attention despite its potential significant impact. The true prevalence of PNS in SLE reported in studies is variable and strongly influenced by American College of Rheumatology (ACR) case definition that includes seven PNS manifestations (acute inflammatory demyelinating polyradiculoneuropathy, autonomic disorder, mononeuropathy, myasthenia gravis, cranial neuropathy, plexopathy and polyneuropathy). Other peripheral manifestations, such as chronic inflammatory demyelinating polyradiculoneuropathy and small fibre neuropathy, not included in the ACR nomenclature, have not been well characterised in SLE. The aim of this review is to focus on epidemiology, pathogenesis, diagnosis and clinical features of all possible different expressions of PNS involvement in SLE, with the final objective to profile the patient's clinical characteristics.

Published
2019

38. Influence of Antisynthetase Antibodies Specificities on Antisynthetase Syndrome Clinical Spectrum Time Course

Author

Cavagna, L, Trallero-Araguás, E, Meloni, F, Cavazzana, I, Rojas-Serrano, J, Feist, E, Zanframundo, G, Morandi, V, Meyer, A, Pereira da Silva, J, Matos Costa, C, Molberg, O, Andersson, H, Codullo, V, Mosca, M, Barsotti, S, Neri, R, Scirè, C, Govoni, M, Furini, F, Lopez-Longo, F, Martinez-Barrio, J, Schneider, U, Lorenz, H, Doria, A, Ghirardello, A, Ortego-Centeno, N, Confalonieri, M, Tomietto, P, Pipitone, N, Rodriguez Cambron, A, Blázquez Cañamero, M, Voll, R, Wendel, S, Scarpato, S, Maurier, F, Limonta, M, Colombelli, P, Giannini, M, Geny, B, Arrigoni, E, Bravi, E, Migliorini, P, Mathieu, A, Piga, M, Drott, U, Delbrueck, C, Bauhammer, J, Cagnotto, G, Vancheri, C, Sambataro, G, De Langhe, E, Sainaghi, P, Monti, C, Gigli Berzolari, F, Romano, M, Bonella, F, Specker, C, Schwarting, A, Villa Blanco, I, Selmi, C, Ceribelli, A, Nuno, L, Mera-Varela, A, Perez Gomez, N, Fusaro, E, Parisi, S, Sinigaglia, L, Del Papa, N, Benucci, M, Cimmino, M, Riccieri, V, Conti, F, Sebastiani, G, Iuliano, A, Emmi, G, Cammelli, D, Sebastiani, M, Manfredi, A, Bachiller-Corral, J, Sifuentes Giraldo, W, Paolazzi, G, Saketkoo, L, Giorgi, R, Salaffi, F, Cifrian, J, Caporali, R, Locatelli, F, Marchioni, E, Pesci, A, Dei, G, Pozzi, M, Claudia, L, Distler, J, Knitza, J, Schett, G, Iannone, F, Fornaro, M, Franceschini, F, Quartuccio, L, Gerli, R, Bartoloni, E, Bellando Randone, S, Zampogna, G, Gonzalez Perez, M, Mejia, M, Vicente, E, Triantafyllias, K, Lopez-Mejias, R, Matucci-Cerinic, M, Selva-O'Callaghan, A, Castañeda, S, Montecucco, C, Gonzalez-Gay, M, Cavagna, Lorenzo, Trallero-Araguás, Ernesto, Meloni, Federica, Cavazzana, Ilaria, Rojas-Serrano, Jorge, Feist, Eugen, Zanframundo, Giovanni, Morandi, Valentina, Meyer, Alain, Pereira da Silva, Jose Antonio, Matos Costa, Carlo Jorge, Molberg, Oyvind, Andersson, Helena, Codullo, Veronica, Mosca, Marta, Barsotti, Simone, Neri, Rossella, Scirè, Carlo, Govoni, Marcello, Furini, Federica, Lopez-Longo, Francisco Javier, Martinez-Barrio, Julia, Schneider, Udo, Lorenz, Hanns-Martin, Doria, Andrea, Ghirardello, Anna, Ortego-Centeno, Norberto, Confalonieri, Marco, Tomietto, Paola, Pipitone, Nicolò, Rodriguez Cambron, Ana Belen, Blázquez Cañamero, María Ángeles, Voll, Reinhard Edmund, Wendel, Sarah, Scarpato, Salvatore, Maurier, Francois, Limonta, Massimiliano, Colombelli, Paolo, Giannini, Margherita, Geny, Bernard, Arrigoni, Eugenio, Bravi, Elena, Migliorini, Paola, Mathieu, Alessandro, Piga, Matteo, Drott, Ulrich, Delbrueck, Christiane, Bauhammer, Jutta, Cagnotto, Giovanni, Vancheri, Carlo, Sambataro, Gianluca, De Langhe, Ellen, Sainaghi, Pier Paolo, Monti, Cristina, Gigli Berzolari, Francesca, Romano, Mariaeva, Bonella, Francesco, Specker, Christof, Schwarting, Andreas, Villa Blanco, Ignacio, Selmi, Carlo, Ceribelli, Angela, Nuno, Laura, Mera-Varela, Antonio, Perez Gomez, Nair, Fusaro, Enrico, Parisi, Simone, Sinigaglia, Luigi, Del Papa, Nicoletta, Benucci, Maurizio, Cimmino, Marco Amedeo, Riccieri, Valeria, Conti, Fabrizio, Sebastiani, Gian Domenico, Iuliano, Annamaria, Emmi, Giacomo, Cammelli, Daniele, Sebastiani, Marco, Manfredi, Andreina, Bachiller-Corral, Javier, Sifuentes Giraldo, Walter Alberto, Paolazzi, Giuseppe, Saketkoo, Lesley Ann, Giorgi, Roberto, Salaffi, Fausto, Cifrian, Jose, Caporali, Roberto, Locatelli, Francesco, Marchioni, Enrico, Pesci, Alberto, Dei, Giulia, Pozzi, Maria Rosa, Claudia, Lomater, Distler, Jorg, Knitza, Johannes, Schett, George, Iannone, Florenzo, Fornaro, Marco, Franceschini, Franco, Quartuccio, Luca, Gerli, Roberto, Bartoloni, Elena, Bellando Randone, Silvia, Zampogna, Giuseppe, Gonzalez Perez, Montserrat I, Mejia, Mayra, Vicente, Esther, Triantafyllias, Konstantinos, Lopez-Mejias, Raquel, Matucci-Cerinic, Marco, Selva-O'Callaghan, Albert, Castañeda, Santos, Montecucco, Carlomaurizio, Gonzalez-Gay, Miguel Angel, Cavagna, L, Trallero-Araguás, E, Meloni, F, Cavazzana, I, Rojas-Serrano, J, Feist, E, Zanframundo, G, Morandi, V, Meyer, A, Pereira da Silva, J, Matos Costa, C, Molberg, O, Andersson, H, Codullo, V, Mosca, M, Barsotti, S, Neri, R, Scirè, C, Govoni, M, Furini, F, Lopez-Longo, F, Martinez-Barrio, J, Schneider, U, Lorenz, H, Doria, A, Ghirardello, A, Ortego-Centeno, N, Confalonieri, M, Tomietto, P, Pipitone, N, Rodriguez Cambron, A, Blázquez Cañamero, M, Voll, R, Wendel, S, Scarpato, S, Maurier, F, Limonta, M, Colombelli, P, Giannini, M, Geny, B, Arrigoni, E, Bravi, E, Migliorini, P, Mathieu, A, Piga, M, Drott, U, Delbrueck, C, Bauhammer, J, Cagnotto, G, Vancheri, C, Sambataro, G, De Langhe, E, Sainaghi, P, Monti, C, Gigli Berzolari, F, Romano, M, Bonella, F, Specker, C, Schwarting, A, Villa Blanco, I, Selmi, C, Ceribelli, A, Nuno, L, Mera-Varela, A, Perez Gomez, N, Fusaro, E, Parisi, S, Sinigaglia, L, Del Papa, N, Benucci, M, Cimmino, M, Riccieri, V, Conti, F, Sebastiani, G, Iuliano, A, Emmi, G, Cammelli, D, Sebastiani, M, Manfredi, A, Bachiller-Corral, J, Sifuentes Giraldo, W, Paolazzi, G, Saketkoo, L, Giorgi, R, Salaffi, F, Cifrian, J, Caporali, R, Locatelli, F, Marchioni, E, Pesci, A, Dei, G, Pozzi, M, Claudia, L, Distler, J, Knitza, J, Schett, G, Iannone, F, Fornaro, M, Franceschini, F, Quartuccio, L, Gerli, R, Bartoloni, E, Bellando Randone, S, Zampogna, G, Gonzalez Perez, M, Mejia, M, Vicente, E, Triantafyllias, K, Lopez-Mejias, R, Matucci-Cerinic, M, Selva-O'Callaghan, A, Castañeda, S, Montecucco, C, Gonzalez-Gay, M, Cavagna, Lorenzo, Trallero-Araguás, Ernesto, Meloni, Federica, Cavazzana, Ilaria, Rojas-Serrano, Jorge, Feist, Eugen, Zanframundo, Giovanni, Morandi, Valentina, Meyer, Alain, Pereira da Silva, Jose Antonio, Matos Costa, Carlo Jorge, Molberg, Oyvind, Andersson, Helena, Codullo, Veronica, Mosca, Marta, Barsotti, Simone, Neri, Rossella, Scirè, Carlo, Govoni, Marcello, Furini, Federica, Lopez-Longo, Francisco Javier, Martinez-Barrio, Julia, Schneider, Udo, Lorenz, Hanns-Martin, Doria, Andrea, Ghirardello, Anna, Ortego-Centeno, Norberto, Confalonieri, Marco, Tomietto, Paola, Pipitone, Nicolò, Rodriguez Cambron, Ana Belen, Blázquez Cañamero, María Ángeles, Voll, Reinhard Edmund, Wendel, Sarah, Scarpato, Salvatore, Maurier, Francois, Limonta, Massimiliano, Colombelli, Paolo, Giannini, Margherita, Geny, Bernard, Arrigoni, Eugenio, Bravi, Elena, Migliorini, Paola, Mathieu, Alessandro, Piga, Matteo, Drott, Ulrich, Delbrueck, Christiane, Bauhammer, Jutta, Cagnotto, Giovanni, Vancheri, Carlo, Sambataro, Gianluca, De Langhe, Ellen, Sainaghi, Pier Paolo, Monti, Cristina, Gigli Berzolari, Francesca, Romano, Mariaeva, Bonella, Francesco, Specker, Christof, Schwarting, Andreas, Villa Blanco, Ignacio, Selmi, Carlo, Ceribelli, Angela, Nuno, Laura, Mera-Varela, Antonio, Perez Gomez, Nair, Fusaro, Enrico, Parisi, Simone, Sinigaglia, Luigi, Del Papa, Nicoletta, Benucci, Maurizio, Cimmino, Marco Amedeo, Riccieri, Valeria, Conti, Fabrizio, Sebastiani, Gian Domenico, Iuliano, Annamaria, Emmi, Giacomo, Cammelli, Daniele, Sebastiani, Marco, Manfredi, Andreina, Bachiller-Corral, Javier, Sifuentes Giraldo, Walter Alberto, Paolazzi, Giuseppe, Saketkoo, Lesley Ann, Giorgi, Roberto, Salaffi, Fausto, Cifrian, Jose, Caporali, Roberto, Locatelli, Francesco, Marchioni, Enrico, Pesci, Alberto, Dei, Giulia, Pozzi, Maria Rosa, Claudia, Lomater, Distler, Jorg, Knitza, Johannes, Schett, George, Iannone, Florenzo, Fornaro, Marco, Franceschini, Franco, Quartuccio, Luca, Gerli, Roberto, Bartoloni, Elena, Bellando Randone, Silvia, Zampogna, Giuseppe, Gonzalez Perez, Montserrat I, Mejia, Mayra, Vicente, Esther, Triantafyllias, Konstantinos, Lopez-Mejias, Raquel, Matucci-Cerinic, Marco, Selva-O'Callaghan, Albert, Castañeda, Santos, Montecucco, Carlomaurizio, and Gonzalez-Gay, Miguel Angel

Abstract

Antisynthetase syndrome (ASSD) is a rare clinical condition that is characterized by the occurrence of a classic clinical triad, encompassing myositis, arthritis, and interstitial lung disease (ILD), along with specific autoantibodies that are addressed to different aminoacyl tRNA synthetases (ARS). Until now, it has been unknown whether the presence of a different ARS might affect the clinical presentation, evolution, and outcome of ASSD. In this study, we retrospectively recorded the time of onset, characteristics, clustering of triad findings, and survival of 828 ASSD patients (593 anti-Jo1, 95 anti-PL7, 84 anti-PL12, 38 anti-EJ, and 18 anti-OJ), referring to AENEAS (American and European NEtwork of Antisynthetase Syndrome) collaborative group's cohort. Comparisons were performed first between all ARS cases and then, in the case of significance, while using anti-Jo1 positive patients as the reference group. The characteristics of triad findings were similar and the onset mainly began with a single triad finding in all groups despite some differences in overall prevalence. The "ex-novo" occurrence of triad findings was only reduced in the anti-PL12-positive cohort, however, it occurred in a clinically relevant percentage of patients (30%). Moreover, survival was not influenced by the underlying anti-aminoacyl tRNA synthetase antibodies' positivity, which confirmed that antisynthetase syndrome is a heterogeneous condition and that antibody specificity only partially influences the clinical presentation and evolution of this condition.

Published
2019

39. Long-term retention rate of anakinra in adult onset Still’s disease and predictive factors for treatment response.

Author

Vitale, A, Cavalli, G, Colafrancesco, S, Priori, R, Valesini, G, Argolini, Lm, Baldissera, E, Bartoloni, E, Cammelli, D, Canestrari, G, Sota, J, Cavallaro, E, Massaro, Maria Grazia, Ruscitti, P, Cipriani, P, De Marchi, G, De Vita, S, Emmi, G, Ferraccioli, Gianfranco, Frassi, M, Gerli, R, Gremese, Elisa, Iannone, F, Lapadula, G, Lopalco, G, Manna, Raffaele, Mathieu, A, Montecucco, C, Mosca, M, Piazza, I, Piga, M, Pontikaki, I, Romano, M, Rossi, S, Rossini, M, Silvestri, E, Stagnaro, C, Talarico, R, Tincani, A, Viapiana, O, Vitiello, G, Galozzi, P, Sfriso, P, Gaggiano, C, Rigante, Donato, Dagna, L, Giacomelli, R, Cantarini, L, Ferraccioli G (ORCID:0000-0001-6246-2428), Gremese E (ORCID:0000-0002-2248-1058), Manna R (ORCID:0000-0003-1560-3907), Rigante D (ORCID:0000-0001-7032-7779), Vitale, A, Cavalli, G, Colafrancesco, S, Priori, R, Valesini, G, Argolini, Lm, Baldissera, E, Bartoloni, E, Cammelli, D, Canestrari, G, Sota, J, Cavallaro, E, Massaro, Maria Grazia, Ruscitti, P, Cipriani, P, De Marchi, G, De Vita, S, Emmi, G, Ferraccioli, Gianfranco, Frassi, M, Gerli, R, Gremese, Elisa, Iannone, F, Lapadula, G, Lopalco, G, Manna, Raffaele, Mathieu, A, Montecucco, C, Mosca, M, Piazza, I, Piga, M, Pontikaki, I, Romano, M, Rossi, S, Rossini, M, Silvestri, E, Stagnaro, C, Talarico, R, Tincani, A, Viapiana, O, Vitiello, G, Galozzi, P, Sfriso, P, Gaggiano, C, Rigante, Donato, Dagna, L, Giacomelli, R, Cantarini, L, Ferraccioli G (ORCID:0000-0001-6246-2428), Gremese E (ORCID:0000-0002-2248-1058), Manna R (ORCID:0000-0003-1560-3907), and Rigante D (ORCID:0000-0001-7032-7779)

Abstract

We have evaluated long-term retention rate of anakinra in adult onset Still disease and predictive factors for treatment response. The risk of losing anakinra efficacy increases along with the number of swollen joints at the start of therapy, while the typical skin rash is a negative predictor of anakinra withdrawal.

Published
2019

40. Ultrasonography in psoriatic arthritis: which sites should we scan?

Author

Zabotti A., Piga M., Canzoni M., Sakellariou G., Iagnocco A., Scire C. A., Adinolfi A., Azzolin I., Bandinelli F., Batticciotto A., Boffini N., Bortoluzzi A., Carrara G., Cavatorta F. P., Cagnotto G., Caprioli M., Colaci M., De Lucia O., Delle Sedie A., Denaro V., Di Matteo A., Di Sabatino V., Epis O. M., Hoxha A., Farina I., Germano G., Filippou G., Filippucci E., Focherini M. C., Gabba A., Idolazzi L., La Paglia G. M. C., Luccioli F., Macchioni P., Magnani M., Massarotti M., Mastaglio C., Navarini L., Parisi S., Diamanti A. P., Picerno V., Piras M., Porta F., Possemato N., Prevete I., Raffeiner B., Ramonda R., Ravagnani V., Rossi D., Rossi S., Scolieri P., Santoboni G., Scioscia C., Terenzi R., Tinazzi I., Toscano C., Venditti C., Volpe A., Vukatana G., Zanframundo G., Zabotti, A, Piga, M, Canzoni, M, Sakellariou, G, Iagnocco, A, Scire, C, Adinolfi, A, Azzolin, I, Bandinelli, F, Batticciotto, A, Boffini, N, Bortoluzzi, A, Carrara, G, Cavatorta, F, Cagnotto, G, Caprioli, M, Colaci, M, De Lucia, O, Delle Sedie, A, Denaro, V, Di Matteo, A, Di Sabatino, V, Epis, O, Hoxha, A, Farina, I, Germano, G, Filippou, G, Filippucci, E, Focherini, M, Gabba, A, Idolazzi, L, La Paglia, G, Luccioli, F, Macchioni, P, Magnani, M, Massarotti, M, Mastaglio, C, Navarini, L, Parisi, S, Diamanti, A, Picerno, V, Piras, M, Porta, F, Possemato, N, Prevete, I, Raffeiner, B, Ramonda, R, Ravagnani, V, Rossi, D, Rossi, S, Scolieri, P, Santoboni, G, Scioscia, C, Terenzi, R, Tinazzi, I, Toscano, C, Venditti, C, Volpe, A, Vukatana, G, and Zanframundo, G

Subjects
Genetics and Molecular Biology (all), medicine.medical_specialty, Immunology, Arthritis, Physical examination, outcomes research, psoriatic arthritis, ultrasonography, rheumatology, immunology and allergy, immunology, biochemistry, genetics and molecular biology (all), Disease, Biochemistry, Sensitivity and Specificity, General Biochemistry, Genetics and Molecular Biology, Rheumatology, Immunology and Allergy, Biochemistry, Genetics and Molecular Biology (all), NO, Tendons, 03 medical and health sciences, Psoriatic arthritis, 0302 clinical medicine, Internal medicine, medicine, Humans, 030212 general & internal medicine, Prospective cohort study, 030203 arthritis & rheumatology, medicine.diagnostic_test, business.industry, outcomes research, psoriatic arthritis, ultrasonography, Arthritis, Psoriatic, Bursa, Synovial, medicine.disease, Joints, Observational study, Differential diagnosis, business
Abstract

In psoriatic arthritis (PsA), ultrasonography (US) plays a growing role in the differential diagnosis and in monitoring treatment response.1 PsA is a heterogeneous disease with different domains and peculiar sites involved.2 Therefore, a dedicated US composite score is needed to monitor disease activity and to identify structural damage progression. A recently published Systematic Literature Review (SLR) identified only two US scores specifically developed for PsA (ie, 5TPD and PsA-Son) and, although these had a good sensitivity to detect inflammation and a good feasibility, they have not been validated in other series.1 3 4 Recently, the Study Group for US of the Italian Society of Rheumatology promoted the Ultrasound in PSoriatic Arthritis TREAtMent (UPSTREAM) study (registered at ClinicalTrial.gov, NCT03330769). UPSTREAM is a multicentre observational prospective cohort study and it represents the first …

Published
2018

41. Ultrasound-detected tenosynovitis independently associates with patient-reported flare in patients with rheumatoid arthritis in clinical remission: Results from the observational study STARTER of the Italian Society for Rheumatology

Author

Bellis, E, Scire, C, Carrara, G, Adinolfi, A, Batticciotto, A, Bortoluzzi, A, Cagnotto, G, Caprioli, M, Canzoni, M, Cavatorta, F, De Lucia, O, Di Sabatino, V, Draghessi, A, Filippou, G, Farina, I, Focherini, M, Gabba, A, Gutierrez, M, Idolazzi, L, Luccioli, F, Macchioni, P, Massarotti, M, Mastaglio, C, Menza, L, Muratore, M, Parisi, S, Picerno, V, Piga, M, Ramonda, R, Raffeiner, B, Rossi, D, Rossi, S, Rossini, P, Sakellariou, G, Scioscia, C, Venditti, C, Volpe, A, Matucci-Cerinic, M, Iagnocco, A, Bellis E., Scire C. A., Carrara G., Adinolfi A., Batticciotto A., Bortoluzzi A., Cagnotto G., Caprioli M., Canzoni M., Cavatorta F. P., De Lucia O., Di Sabatino V., Draghessi A., Filippou G., Farina I., Focherini M. C., Gabba A., Gutierrez M., Idolazzi L., Luccioli F., Macchioni P., Massarotti M. S., Mastaglio C., Menza L., Muratore M., Parisi S., Picerno V., Piga M., Ramonda R., Raffeiner B., Rossi D., Rossi S., Rossini P., Sakellariou G., Scioscia C., Venditti C., Volpe A., Matucci-Cerinic M., Iagnocco A., Bellis, E, Scire, C, Carrara, G, Adinolfi, A, Batticciotto, A, Bortoluzzi, A, Cagnotto, G, Caprioli, M, Canzoni, M, Cavatorta, F, De Lucia, O, Di Sabatino, V, Draghessi, A, Filippou, G, Farina, I, Focherini, M, Gabba, A, Gutierrez, M, Idolazzi, L, Luccioli, F, Macchioni, P, Massarotti, M, Mastaglio, C, Menza, L, Muratore, M, Parisi, S, Picerno, V, Piga, M, Ramonda, R, Raffeiner, B, Rossi, D, Rossi, S, Rossini, P, Sakellariou, G, Scioscia, C, Venditti, C, Volpe, A, Matucci-Cerinic, M, Iagnocco, A, Bellis E., Scire C. A., Carrara G., Adinolfi A., Batticciotto A., Bortoluzzi A., Cagnotto G., Caprioli M., Canzoni M., Cavatorta F. P., De Lucia O., Di Sabatino V., Draghessi A., Filippou G., Farina I., Focherini M. C., Gabba A., Gutierrez M., Idolazzi L., Luccioli F., Macchioni P., Massarotti M. S., Mastaglio C., Menza L., Muratore M., Parisi S., Picerno V., Piga M., Ramonda R., Raffeiner B., Rossi D., Rossi S., Rossini P., Sakellariou G., Scioscia C., Venditti C., Volpe A., Matucci-Cerinic M., and Iagnocco A.

Abstract

Objectives. This study aimed to estimate the prevalence of US-detected tenosynovitis in RA patients in clinical remission and to explore its clinical correlates.Methods. A total of 427 RA patients in clinical remission were consecutively enrolled from 25 Italian rheumatology centres. Tenosynovitis and synovitis were scored by US grey scale (GS) and power Doppler (PD) semi-quantitative scoring systems at wrist and hand joints. Complete clinical assessment was performed by rheumatologists blinded to the US results. A flare questionnaire was used to assess unstable remission (primary outcome), HAQ for functional disability and radiographic erosions for damage (secondary outcomes). Cross-sectional relationships between the presence of each US finding and outcome variables are presented as odds ratios (ORs) and 95% CIs, both crude and adjusted for pre-specified confounders.Results. The prevalence of tenosynovitis in clinical remission was 52.5% (95% CI 0.48, 0.57) for GS and 22.7% (95% CI 0.19, 0.27) for PD, while the prevalence of synovitis was 71.6% (95% CI 0.67, 0.76) for GS and 42% (95% CI 0.37, 0.47) for PD. Among clinical correlates, PD tenosynovitis associated with lower remission duration and morning stiffness while PD synovitis did not. Only PD tenosynovitis showed a significant association with the flare questionnaire [OR 1.95 (95% CI 1.17, 3.26)]. No cross-sectional associations were found with the HAQ. The presence of radiographic erosions associated with GS and PD synovitis but not with tenosynovitis.Conclusions. US-detected tenosynovitis is a frequent finding in RA patients in clinical remission and associates with unstable remission.

Published
2016

45. La donazione d'organi 'samaritana': problemi etico-deontologici e giuridici

Author

Badengo, S, Marchesi, M, Zoja, R, Piga, M, Piga, M A, Badengo, S, Marchesi, M, Zoja, R, Piga, M, and Piga, M A

Abstract

The so called “Samaritan donation” is meant as non-directed altruistic donation, where the donor does not choose the recipient. It’s an uncommon practice in Italy, as in many over other countries. An critical hesitation to develop these type of transplantation programs is the fear of incentivize the recruitment of disadvantaged people to provide organs for paying recipients; another are doubts related to motivations (possible psychiatric or psychological illness). All Sa- maritan donors should be always carefully assessed by an independent Authority, to make sure the donor knows and understands what is involved, is not under any pressure to donate and is giving consent freely and voluntarily. Samaritan donation should not be intended as an alterna- tive to after-death-donation, but its exceptionality should contribute toward increasing aware- ness of people and the amount of after-death-donations.

Published
2013

46. Development and validation of a new algorithm for attribution of neuropsychiatric events in systemic lupus erythematosus

Author

Bortoluzzi, A, Scirè, Ca, Bombardieri, S, Caniatti, L, Conti, F, De Vita, S, Doria, Andrea, Ferraccioli, G, Gremese, E, Mansutti, E, Mathieu, A, Mosca, M, Padovan, M, Piga, M, Tincani, A, Tola, Mr, Tomietto, P, Valesini, G, Zen, Margherita, Govoni, M, on behalf of the Study Group on Neuropsychiatric Systemic Lupus Erythematosus of the Italian Society of Rheumatology, Bortoluzzi, A, Scirè, C, Bombardieri, S, Caniatti, L, Conti, F, De Vita, S, Doria, A, Ferraccioli, G, Gremese, E, Mansutti, E, Mathieu, A, and Mosca, M

Subjects
Male, Central Nervous System, Settore MED/16 - REUMATOLOGIA, Attribution model, Neuropsychiatric, Systemic lupus erythematosus, Adult, Cognition Disorders, Cohort Studies, Diagnosis, Differential, Female, Headache, Humans, Logistic Models, Lupus Erythematosus, Systemic, Lupus Vasculitis, Central Nervous System, Middle Aged, Mood Disorders, Prevalence, ROC Curve, Reference Standards, Retrospective Studies, Algorithms, Models, Statistical, Rheumatology, Pharmacology (medical), systemic lupus erythematosus, Models, Diagnosis, Medicine, Event (probability theory), Confounding, Statistical, neuropsychiatric, Cohort, Algorithm, Cohort study, Lupus Vasculitis, Systemic lupus erythematosu, attribution model, NO, Reference standards, Receiver operating characteristic, Lupus Erythematosus, business.industry, Systemic, Editorials, Retrospective cohort study, Differential, Attribution, business
Abstract

Objective. The aim of this study was to develop and validate an algorithm to assist the attribution of neuropsychiatric (NP) events to underlying disease in SLE patients. Methods. Phase 1 identified and categorized candidate items to be included in the algorithm for the attribution of an NP event to SLE and their relative weights through a literature-informed consensus-driven process. Using a retrospective training cohort of SLE, phase 2 validated items selected in phase 1 and refined weights through a data-driven process, fitting items as independent variables and expert evaluation (clinical judgement) as reference standard in logistic models. Phase 3 consisted of a validation process using an external multicentre retrospective SLE cohort. Results. Phase 1 identified four different items: timing of the NP event, type of event, confounding factors and favouring factors. The training and validating cohorts included 228 and 221 patients, respectively. Each patient experienced at least one NP event characterized using the ACR case definition. In these samples, items selected in phase 1 showed good performance in discriminating patients with NPSLE: the area under the receiver operating characteristic curve using dichotomous outcomes was 0.87 in the training set and 0.82 in the validating set. Relevant cut-offs of the validated score identify events with a positive predictive value of 100% (95% CI 93.2, 100) and 86.3% (95% CI 76.2, 93.2) in the training and validating cohorts, respectively. Conclusion. A new algorithm based on a probability score was developed and validated to determine the relationship between NP events and SLE.

Published
2015

49. La professione del sociologo nel panorama internazionale

Author

Dallago, B, Bruschi, A, Rampazi, M, Negrelli, S, Scaglia, A, Barone, C, Ruggera, L, Di Nicola, P, Gubert, R, Pollini, G, Piga, M, Pretto, A, Artieri Boccia, G, Borrelli, D, Gavrila, M, Galesi, D, Carboni, A, Gianera, A, Perino, A, Manna, E, Savonardo, L, Gherardi, S, Fazzi, L, Fischietti, G, NEGRELLI, SERAFINO, Dallago, B, Bruschi, A, Rampazi, M, Negrelli, S, Scaglia, A, Barone, C, Ruggera, L, Di Nicola, P, Gubert, R, Pollini, G, Piga, M, Pretto, A, Artieri Boccia, G, Borrelli, D, Gavrila, M, Galesi, D, Carboni, A, Gianera, A, Perino, A, Manna, E, Savonardo, L, Gherardi, S, Fazzi, L, Fischietti, G, and NEGRELLI, SERAFINO

Published
2015

50. Factors and comorbidities associated with first neuropsychiatric event in systemic lupus erythematosus: does a risk profile exist? A large multicentre retrospective cross-sectional study on 959 Italian patients

Author

Govoni, M, Bombardieri, S, Bortoluzzi, A, Caniatti, L, Casu, C, Conti, F, De Vita, S, Doria, Andrea, Farina, I, Ferraccioli, G, Gremese, E, Mansutti, E, Mosca, M, Padovan, M, Piga, M, Tincani, A, Tola, Mr, Tomietto, P, Taglietti, M, Trotta, F, Valesini, G, Zen, M, Mathieu, A, and Italian Society of Rheumatology

Subjects
Adult, Male, medicine.medical_specialty, Headache Disorders, Cross-sectional study, anti-phospholipid antibodies, anti-phospholipid syndrome, cerebrovascular disease, neurological involvement, neuropsychiatric lupus, systemic lupus erythematosus, Comorbidity, Disease, Risk profile, Time frame, Rheumatology, immune system diseases, Internal medicine, medicine, Humans, Pharmacology (medical), Lupus vasculitis, skin and connective tissue diseases, Systemic lupus erythematosus, business.industry, Antiphospholipid antibodies, Mental Disorders, Lupus Vasculitis, Central Nervous System, Middle Aged, Antiphospholipid Syndrome, Prognosis, medicine.disease, Stroke, Italy, Mood disorders, Antibodies, Antiphospholipid, Physical therapy, Female, Epidemiologic Methods, business, Neuropsychiatric lupus, Neurological involvement, Anti-phospholipid syndrome, Cerebrovascular disease, Biomarkers
Abstract

Objective. To analyse risk factors and comorbidities potentially associated with CNS involvement in a large cohort of Italian patients affected by SLE. Methods. A number of generic (not strictly SLE related) and specific (disease related) risk factors to which all patients have been exposed in the span of 5 years before the first neuropsychiatric (NP) event or before the last available observation were checked for and their distribution was analysed in 959 SLE patients with and without NP involvement; all the first NP events that occurred in a time frame of 10 years were recorded and categorized as SLE related or SLE unrelated. Results. Three hundred and twenty-six SLE patients with and 633 SLE patients without NP manifestations were included in the study. A total of 469 NP events were recorded. Headache (26.1%), cerebrovascular events (22.7%), mood disorders (8.9%), seizures (14.4%) and cognitive dysfunctions (9.5%) were the most frequent SLE-related NP events. More risk factors [mean 4.52 (2.44) vs 3.73 (2.01); P < 0.0001] were observed in patients with than without NP involvement. Overall, aPLs, LA and APS were factors more strongly associated with NP involvement. Conclusions. In SLE, NP involvement and aPLs were confirmed as closely related. Furthermore, other modifiable generic risk factors, such as hypertension, carotid vasculopathy and dyslipidaemia, appeared to be related to the occurrence of cerebral vascular accident (CVA) and cognitive dysfunctions, suggesting the need for a more intensive preventive strategy to optimize the management of NP lupus.

Published
2012

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