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1. The Updated Role of Ultrasound in Assessing Dermatological Manifestations in Systemic Sclerosis

Author: Ruaro B, Santiago T, Hughes M, Lepri G, Poillucci G, Baratella E, Salton F, and Confalonieri M
Subjects: systemic sclerosis, ultrasound, elastosonography, skin, skin thickness, digital ulcers, Diseases of the musculoskeletal system, RC925-935
Abstract: Barbara Ruaro,1 Tania Santiago,2,3 Michael Hughes,4 Gemma Lepri,5 Gabriele Poillucci,6 Elisa Baratella,6 Francesco Salton,1 Marco Confalonieri1 1Unit of Pulmonology, University Hospital of Trieste, Trieste, Italy; 2Department of Rheumatology, Centro Hospitalare Universitário de Coimbra, Coimbra, Portugal; 3Medicine Faculty, University of Coimbra, Coimbra, Portugal; 4Department of Rheumatology, Royal Hallamshire Hospital, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK; 5Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Firenze, Florence, Italy; 6Department of Radiology, Department of Medicine, Surgery and Health Science, University of Trieste, Trieste, ItalyCorrespondence: Barbara RuaroUnit of Pulmonology, University Hospital of Trieste, Trieste, ItalyTel +39 3470502394Email barbara.ruaro@yahoo.itAbstract: Systemic sclerosis (SSc), an autoimmune connective tissue disease, characterized by skin fibrosis, increased dermal thickness and microvascular involvement. Fibroblasts and myofibroblasts deposit excessive amounts of collagenous and non-collagenous extracellular matrix components in the skin. This leads to microvascular abnormalities and Raynaud’s phenomenon, with painful digital ulcers (DU) at the fingertips adding to patient discomfort. The skin involvement and severity in SSc was evaluated by the Modified Rodnan skin score (mRSS). Although high-frequency ultrasound (HUS) has been widely researched in the study of skin thickness and DU in SSc, its adoption into clinical practice is not yet common. However, novel insights into the still relatively unknown disease pathogenesis in SSc and its evaluation may be provided by HUS, including early (pre-clinical) skin involvement. It may also be useful in both the evaluation and follow-up of DU. Indeed, it is a non-invasive, safe, inexpensive and reproducible method able to assess not only SSc patients’ cutaneous structural changes, but also their vascular system changes. Moreover, several recent studies have reported that elastosonography (ES) is of use when investigating skin involvement in systemic sclerosis. This review aims at providing information as to role HUS and ES play in research advancements and the clinical perspectives in the evaluation of skin thickness and DU in SSc patients.Keywords: systemic sclerosis, ultrasound, elastosonography, skin, skin thickness, digital ulcers
Published: 2021

2. The role of lung biopsy for diagnosis and prognosis of interstitial lung disease in systemic sclerosis: a systematic literature review

Author: Damiani, A, Orlandi, M, Bruni, C, Bandini, G, Lepri, G, Scaletti, C, Ravaglia, C, Frassanito, F, Guiducci, S, Moggi-Pignone, A, Matucci-Cerinic, M, Poletti, V, Tofani, L, Colby, TV, Randone, S Bellando, and Tomassetti, Sara
Published: 2024
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3. POS0044 DECIPHERING THE GUT MICROBIOTA OF VERY EARLY SYSTEMIC SCLEROSIS (VEDOSS) PATIENTS: A TAXONOMIC AND FUNCTIONAL CHARACTERIZATION

Author: Russo, E., primary, Lepri, G., additional, Baldi, S., additional, Fioretto, B. S., additional, Romano, E., additional, El Aoufy, K., additional, Ramazzotti, M., additional, Rosa, I., additional, Ghezzi, G., additional, Gloria, L. DI, additional, Orlandi, M., additional, Bertorello, S., additional, Bruni, C., additional, Guiducci, S., additional, Manetti, M., additional, Matucci-Cerinic, M., additional, Amedei, A., additional, and Bellando Randone, S., additional
Published: 2024
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4. Systemic sclerosis sine scleroderma: clinical and serological features and relationship with other cutaneous subsets in a large series of patients from the national registry 'SPRING' of the Italian Society for Rheumatology

Author: De Angelis, R, Ferri, C, Giuggioli, D, Bajocchi, G, Dagna, L, Bellando-Randone, S, Zanframundo, G, Foti, R, Cacciapaglia, F, Cuomo, G, Ariani, A, Rosato, E, Lepri, G, Girelli, F, Riccieri, V, Zanatta, E, Bosello, S, Cavazzana, I, Ingegnoli, F, De Santis, M, Murdaca, G, Abignano, G, Romeo, N, Della Rossa, A, Caminiti, M, Iuliano, A, Ciano, G, Beretta, L, Bagnato, G, Lubrano, E, De Andres, I, Giollo, A, Saracco, M, Agnes, C, Cipolletta, E, Lumetti, F, Spinella, A, Magnani, L, Campochiaro, C, De Luca, G, Codullo, V, Visalli, E, Di Vico, C, Gigante, A, Pellagrino, G, Pigatto, E, Lazzaroni, M, Franceschini, F, Generali, E, Mennillo, G, Barsotti, S, Mariano, G, Furini, F, Vultaggio, L, Parisi, S, Peroni, C, Rozza, D, Zanetti, A, Carrara, G, Landolfi, G, Scire, C, Bianchi, G, Fusaro, E, Sebastiani, G, Govoni, M, D'Angelo, S, Cozzi, F, Guiducci, S, Doria, A, Salvarani, C, Iannone, F, Matucci-Cerinic, M, Giorgio, A, Alessia, B, Francesca, C, Renato, C, Dall'Ara, F, Angelo, D, Marica, D, Gianluca, S, Rossella, T, De Angelis R., Ferri C., Giuggioli D., Bajocchi G., Dagna L., Bellando-Randone S., Zanframundo G., Foti R., Cacciapaglia F., Cuomo G., Ariani A., Rosato E., Lepri G., Girelli F., Riccieri V., Zanatta E., Bosello S. L., Cavazzana I., Ingegnoli F., De Santis M., Murdaca G., Abignano G., Romeo N., Della Rossa A., Caminiti M., Iuliano A. M., Ciano G., Beretta L., Bagnato G., Lubrano E., De Andres I., Giollo A., Saracco M., Agnes C., Cipolletta E., Lumetti F., Spinella A., Magnani L., Campochiaro C., De Luca G., Codullo V., Visalli E., Di Vico C., Gigante A., Pellagrino G., Pigatto E., Lazzaroni M. -G., Franceschini F., Generali E., Mennillo G., Barsotti S., Mariano G. P., Furini F., Vultaggio L., Parisi S., Peroni C. L., Rozza D., Zanetti A., Carrara G., Landolfi G., Scire C. A., Bianchi G., Fusaro E., Sebastiani G. D., Govoni M., D'Angelo S., Cozzi F., Guiducci S., Doria A., Salvarani C., Iannone F., Matucci-Cerinic M., Giorgio A., Alessia B., Francesca C., Renato C., Dall'Ara F., Angelo D. C., Marica D., Gianluca S., Rossella T., De Angelis, R, Ferri, C, Giuggioli, D, Bajocchi, G, Dagna, L, Bellando-Randone, S, Zanframundo, G, Foti, R, Cacciapaglia, F, Cuomo, G, Ariani, A, Rosato, E, Lepri, G, Girelli, F, Riccieri, V, Zanatta, E, Bosello, S, Cavazzana, I, Ingegnoli, F, De Santis, M, Murdaca, G, Abignano, G, Romeo, N, Della Rossa, A, Caminiti, M, Iuliano, A, Ciano, G, Beretta, L, Bagnato, G, Lubrano, E, De Andres, I, Giollo, A, Saracco, M, Agnes, C, Cipolletta, E, Lumetti, F, Spinella, A, Magnani, L, Campochiaro, C, De Luca, G, Codullo, V, Visalli, E, Di Vico, C, Gigante, A, Pellagrino, G, Pigatto, E, Lazzaroni, M, Franceschini, F, Generali, E, Mennillo, G, Barsotti, S, Mariano, G, Furini, F, Vultaggio, L, Parisi, S, Peroni, C, Rozza, D, Zanetti, A, Carrara, G, Landolfi, G, Scire, C, Bianchi, G, Fusaro, E, Sebastiani, G, Govoni, M, D'Angelo, S, Cozzi, F, Guiducci, S, Doria, A, Salvarani, C, Iannone, F, Matucci-Cerinic, M, Giorgio, A, Alessia, B, Francesca, C, Renato, C, Dall'Ara, F, Angelo, D, Marica, D, Gianluca, S, Rossella, T, De Angelis R., Ferri C., Giuggioli D., Bajocchi G., Dagna L., Bellando-Randone S., Zanframundo G., Foti R., Cacciapaglia F., Cuomo G., Ariani A., Rosato E., Lepri G., Girelli F., Riccieri V., Zanatta E., Bosello S. L., Cavazzana I., Ingegnoli F., De Santis M., Murdaca G., Abignano G., Romeo N., Della Rossa A., Caminiti M., Iuliano A. M., Ciano G., Beretta L., Bagnato G., Lubrano E., De Andres I., Giollo A., Saracco M., Agnes C., Cipolletta E., Lumetti F., Spinella A., Magnani L., Campochiaro C., De Luca G., Codullo V., Visalli E., Di Vico C., Gigante A., Pellagrino G., Pigatto E., Lazzaroni M. -G., Franceschini F., Generali E., Mennillo G., Barsotti S., Mariano G. P., Furini F., Vultaggio L., Parisi S., Peroni C. L., Rozza D., Zanetti A., Carrara G., Landolfi G., Scire C. A., Bianchi G., Fusaro E., Sebastiani G. D., Govoni M., D'Angelo S., Cozzi F., Guiducci S., Doria A., Salvarani C., Iannone F., Matucci-Cerinic M., Giorgio A., Alessia B., Francesca C., Renato C., Dall'Ara F., Angelo D. C., Marica D., Gianluca S., and Rossella T.
Abstract: Objective To describe demographic, clinical and laboratory features of systemic sclerosis sine scleroderma (ssSSc) in a large multicentre systemic sclerosis (SSc) cohort. Methods Data involving 1808 SSc patients from Italian Systemic sclerosis PRogression INvestiGation registry were collected. The ssSSc was defined by the absence of any cutaneous sclerosis and/or puffy fingers. Clinical and serological features of ssSSc were compared with limited cutaneous (lcSSc) and diffuse cutaneous (dcSSc) subsets. Results Among patients with SSc, only 61 (3.4%) were classified as having ssSSc (F/M=19/1). Time from Raynaud's phenomenon (RP) onset to diagnosis was longer in ssSSc (3 years, IQR 1-16.5) than lcSSc (2 years, IQR 0-7), and dcSSc (1 year, IQR 0-3) (p<0.001). Clinical ssSSc phenotype was comparable to lcSSc, except for digital pitting scars (DPS) (19.7% vs 42%, p=0.01), but significantly milder than dcSSc, particularly for digital ulcers (DU) (6.6% vs 35.7%, p<0.001), oesophagus (46.2% vs 63.5%, p=0.009), lung (mean diffusion capacity for carbon monoxide 72.2±19.6 vs 62.4±22.8, p=0.009; mean forced vital capacity 105.6±21.7 vs 89.2±20.9, p<0.001) and major videocapillaroscopic alterations (late pattern 8.6% vs 47.6%, p<0.001). Moreover, in ssSSc the percentages of anticentromere and antitopoisomerase were comparable to lcSSc (40% and 18.3% vs 36.7% and 26.6%), but divergent respect to dcSSc (8.6% and 67.4%, p<0.001). Conclusion The ssSSc is a quite rare disease variant characterised by clinico-serological features comparable to lcSSc, but significantly different from dcSSc. Overall, longer RP duration, low percentages of DPS and peripheral microvascular abnormalities, and increased anti-centromere seropositivity distinguish ssSSc. Further investigations based on national registries might provide useful insights on the actual relevance of the ssSSc within the scleroderma spectrum.
Published: 2023

5. Systemic sclerosis sine scleroderma: clinical and serological features and relationship with other cutaneous subsets in a large series of patients from the national registry 'SPRING' of the Italian Society for Rheumatology

Author: De Angelis R., Ferri C., Giuggioli D., Bajocchi G., Dagna L., Bellando-Randone S., Zanframundo G., Foti R., Cacciapaglia F., Cuomo G., Ariani A., Rosato E., Lepri G., Girelli F., Riccieri V., Zanatta E., Bosello S. L., Cavazzana I., Ingegnoli F., De Santis M., Murdaca G., Abignano G., Romeo N., Della Rossa A., Caminiti M., Iuliano A. M., Ciano G., Beretta L., Bagnato G., Lubrano E., De Andres I., Giollo A., Saracco M., Agnes C., Cipolletta E., Lumetti F., Spinella A., Magnani L., Campochiaro C., De Luca G., Codullo V., Visalli E., Di Vico C., Gigante A., Pellagrino G., Pigatto E., Lazzaroni M. -G., Franceschini F., Generali E., Mennillo G., Barsotti S., Mariano G. P., Furini F., Vultaggio L., Parisi S., Peroni C. L., Rozza D., Zanetti A., Carrara G., Landolfi G., Scire C. A., Bianchi G., Fusaro E., Sebastiani G. D., Govoni M., D'Angelo S., Cozzi F., Guiducci S., Doria A., Salvarani C., Iannone F., Matucci-Cerinic M., Giorgio A., Alessia B., Francesca C., Renato C., Dall'Ara F., Angelo D. C., Marica D., Gianluca S., Rossella T., De Angelis, R, Ferri, C, Giuggioli, D, Bajocchi, G, Dagna, L, Bellando-Randone, S, Zanframundo, G, Foti, R, Cacciapaglia, F, Cuomo, G, Ariani, A, Rosato, E, Lepri, G, Girelli, F, Riccieri, V, Zanatta, E, Bosello, S, Cavazzana, I, Ingegnoli, F, De Santis, M, Murdaca, G, Abignano, G, Romeo, N, Della Rossa, A, Caminiti, M, Iuliano, A, Ciano, G, Beretta, L, Bagnato, G, Lubrano, E, De Andres, I, Giollo, A, Saracco, M, Agnes, C, Cipolletta, E, Lumetti, F, Spinella, A, Magnani, L, Campochiaro, C, De Luca, G, Codullo, V, Visalli, E, Di Vico, C, Gigante, A, Pellagrino, G, Pigatto, E, Lazzaroni, M, Franceschini, F, Generali, E, Mennillo, G, Barsotti, S, Mariano, G, Furini, F, Vultaggio, L, Parisi, S, Peroni, C, Rozza, D, Zanetti, A, Carrara, G, Landolfi, G, Scire, C, Bianchi, G, Fusaro, E, Sebastiani, G, Govoni, M, D'Angelo, S, Cozzi, F, Guiducci, S, Doria, A, Salvarani, C, Iannone, F, Matucci-Cerinic, M, Giorgio, A, Alessia, B, Francesca, C, Renato, C, Dall'Ara, F, Angelo, D, Marica, D, Gianluca, S, Rossella, T, De Angelis, R., Ferri, C., Giuggioli, D., Bajocchi, G., Dagna, L., Bellando-Randone, S., Zanframundo, G., Foti, R., Cacciapaglia, F., Cuomo, G., Ariani, A., Rosato, E., Lepri, G., Girelli, F., Riccieri, V., Zanatta, E., Bosello, S. L., Cavazzana, I., Ingegnoli, F., De Santis, M., Murdaca, G., Abignano, G., Romeo, N., Della Rossa, A., Caminiti, M., Iuliano, A. M., Ciano, G., Beretta, L., Bagnato, G., Lubrano, E., De Andres, I., Giollo, A., Saracco, M., Agnes, C., Cipolletta, E., Lumetti, F., Spinella, A., Magnani, L., Campochiaro, C., De Luca, G., Codullo, V., Visalli, E., Di Vico, C., Gigante, A., Pellagrino, G., Pigatto, E., Lazzaroni, M. -G., Franceschini, F., Generali, E., Mennillo, G., Barsotti, S., Mariano, G. P., Furini, F., Vultaggio, L., Parisi, S., Peroni, C. L., Rozza, D., Zanetti, A., Carrara, G., Landolfi, G., Scire, C. A., Bianchi, G., Fusaro, E., Sebastiani, G. D., Govoni, M., D'Angelo, S., Cozzi, F., Guiducci, S., Doria, A., Salvarani, C., Iannone, F., Matucci-Cerinic, M., Giorgio, A., Alessia, B., Francesca, C., Renato, C., Dall'Ara, F., Angelo, D. C., Marica, D., Gianluca, S., and Rossella, T.
Subjects: Scleroderma, Systemic, Settore MED/16 - REUMATOLOGIA, Epidemiology, Immunology, Systemic, Autoimmunity, Autoimmune Disease, Autoimmune Diseases, Scleroderma, Rheumatology, Immunology and Allergy, Seasons, Human
Abstract: ObjectiveTo describe demographic, clinical and laboratory features of systemic sclerosis sine scleroderma (ssSSc) in a large multicentre systemic sclerosis (SSc) cohort.MethodsData involving 1808 SSc patients from Italian Systemic sclerosis PRogression INvestiGation registry were collected. The ssSSc was defined by the absence of any cutaneous sclerosis and/or puffy fingers. Clinical and serological features of ssSSc were compared with limited cutaneous (lcSSc) and diffuse cutaneous (dcSSc) subsets.ResultsAmong patients with SSc, only 61 (3.4%) were classified as having ssSSc (F/M=19/1). Time from Raynaud’s phenomenon (RP) onset to diagnosis was longer in ssSSc (3 years, IQR 1–16.5) than lcSSc (2 years, IQR 0–7), and dcSSc (1 year, IQR 0–3) (pConclusionThe ssSSc is a quite rare disease variant characterised by clinico-serological features comparable to lcSSc, but significantly different from dcSSc. Overall, longer RP duration, low percentages of DPS and peripheral microvascular abnormalities, and increased anti-centromere seropositivity distinguish ssSSc. Further investigations based on national registries might provide useful insights on the actual relevance of the ssSSc within the scleroderma spectrum.
Published: 2023

6. The safety of iloprost in systemic sclerosis in a real-life experience

Author: Bellando-Randone, S., Bruni, C., Lepri, G., Fiori, G., Bartoli, F., Conforti, ML, Moggi-Pignone, A., Guiducci, S., Giuggioli, D., Colaci, M., Spinella, A., Ferri, C., and Matucci-Cerinic, M.
Published: 2018
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7. POS0613 THE ROLE OF MICROBIOTA IN DIFFERENT DISEASE STAGES OF SYSTEMIC SCLEROSIS: A POSSIBLE IMPACT IN GASTROINTESTINAL TRACT DISEASE ACTIVITY?

Author: Russo, E., primary, Ghezzi, G., additional, Baldi, S., additional, Fioretto, B. S., additional, Romano, E., additional, El Aoufy, K., additional, Ramazzotti, M., additional, Rosa, I., additional, Lepri, G., additional, DI Gloria, L., additional, Bruni, C., additional, Melchiorre, D., additional, Guiducci, S., additional, Manetti, M., additional, Matucci-Cerinic, M., additional, Amedei, A., additional, and Bellando Randone, S., additional
Published: 2023
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8. AB0867 LOW STARTING DOSE AND TITRATION OF NINTEDANIB FOR SSC-ILD: ANALYSIS OF TOLERABILITY IN A SINGLE-CENTER COHORT

Author: Fiorentini, E., primary, Bonomi, F., additional, Cometi, L., additional, Lepri, G., additional, Bellando-Randone, S., additional, and Guiducci, S., additional
Published: 2023
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9. POS1313 EFFICACY OF IMMUNOGLOBULIN TREATMENT ON GASTROINTESTINAL, CUTANEOUS AND VASCULAR INVOLVEMENT IN SYSTEMIC SCLEROSIS: DATA FROM AN ITALIAN COHORT OF 65 PATIENTS

Author: Bonomi, F., primary, Damiani, A., additional, Coccia, C., additional, Levani, J., additional, Fiorentini, E., additional, Lepri, G., additional, Orlandi, M., additional, Bartoli, F., additional, Matucci-Cerinic, M., additional, Bellando-Randone, S., additional, and Guiducci, S., additional
Published: 2023
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10. Systemic sclerosis sine scleroderma: clinical and serological features and relationship with other cutaneous subsets in a large series of patients from the national registry 'SPRING' of the Italian Society for Rheumatology

Author: De Angelis, R., Ferri, C., Giuggioli, D., Bajocchi, G., Dagna, L., Bellando-Randone, S., Zanframundo, G., Foti, Roberta, Cacciapaglia, F., Cuomo, G., Ariani, A., Rosato, E., Lepri, G., Girelli, F., Riccieri, V., Zanatta, E., Bosello, Silvia Laura, Cavazzana, I., Ingegnoli, F., De Santis, M., Murdaca, G., Abignano, G., Romeo, N., Della Rossa, A., Caminiti, M., Iuliano, A. M., Ciano, G., Beretta, Carlo Luigi, Bagnato, G., Lubrano, E., De Andres, I., Giollo, A., Saracco, M., Agnes, C., Cipolletta, Eleonora, Lumetti, F., Spinella, A., Magnani, L., Campochiaro, C., De Luca, G., Codullo, V., Visalli, E., Di Vico, C., Gigante, A., Pellagrino, G., Pigatto, E., Lazzaroni, M. -G., Franceschini, F., Generali, E., Mennillo, G., Barsotti, S., Mariano, G. P., Furini, F., Vultaggio, L., Parisi, S., Peroni, C. L., Rozza, D., Zanetti, Maria Assunta, Carrara, Giancarlo, Landolfi, G., Scire, C. A., Bianchi, G., Fusaro, Enrica Maria, Sebastiani, Gian Domenico, Govoni, M., D'Angelo, S., Cozzi, F., Guiducci, S., Doria, A., Salvarani, C., Iannone, F., Matucci-Cerinic, M., Giorgio, A., Alessia, B., Francesca, C., Renato, C., Dall'Ara, F., Angelo, D. C., Marica, D., Gianluca, S., Rossella, T., Foti R., Bosello S. L. (ORCID:0000-0002-4837-447X), Beretta L. (ORCID:0000-0001-9924-2066), Cipolletta E., Zanetti A., Carrara G., Fusaro E., Sebastiani G. D., De Angelis, R., Ferri, C., Giuggioli, D., Bajocchi, G., Dagna, L., Bellando-Randone, S., Zanframundo, G., Foti, Roberta, Cacciapaglia, F., Cuomo, G., Ariani, A., Rosato, E., Lepri, G., Girelli, F., Riccieri, V., Zanatta, E., Bosello, Silvia Laura, Cavazzana, I., Ingegnoli, F., De Santis, M., Murdaca, G., Abignano, G., Romeo, N., Della Rossa, A., Caminiti, M., Iuliano, A. M., Ciano, G., Beretta, Carlo Luigi, Bagnato, G., Lubrano, E., De Andres, I., Giollo, A., Saracco, M., Agnes, C., Cipolletta, Eleonora, Lumetti, F., Spinella, A., Magnani, L., Campochiaro, C., De Luca, G., Codullo, V., Visalli, E., Di Vico, C., Gigante, A., Pellagrino, G., Pigatto, E., Lazzaroni, M. -G., Franceschini, F., Generali, E., Mennillo, G., Barsotti, S., Mariano, G. P., Furini, F., Vultaggio, L., Parisi, S., Peroni, C. L., Rozza, D., Zanetti, Maria Assunta, Carrara, Giancarlo, Landolfi, G., Scire, C. A., Bianchi, G., Fusaro, Enrica Maria, Sebastiani, Gian Domenico, Govoni, M., D'Angelo, S., Cozzi, F., Guiducci, S., Doria, A., Salvarani, C., Iannone, F., Matucci-Cerinic, M., Giorgio, A., Alessia, B., Francesca, C., Renato, C., Dall'Ara, F., Angelo, D. C., Marica, D., Gianluca, S., Rossella, T., Foti R., Bosello S. L. (ORCID:0000-0002-4837-447X), Beretta L. (ORCID:0000-0001-9924-2066), Cipolletta E., Zanetti A., Carrara G., Fusaro E., and Sebastiani G. D.
Abstract: Objective To describe demographic, clinical and laboratory features of systemic sclerosis sine scleroderma (ssSSc) in a large multicentre systemic sclerosis (SSc) cohort. Methods Data involving 1808 SSc patients from Italian Systemic sclerosis PRogression INvestiGation registry were collected. The ssSSc was defined by the absence of any cutaneous sclerosis and/or puffy fingers. Clinical and serological features of ssSSc were compared with limited cutaneous (lcSSc) and diffuse cutaneous (dcSSc) subsets. Results Among patients with SSc, only 61 (3.4%) were classified as having ssSSc (F/M=19/1). Time from Raynaud's phenomenon (RP) onset to diagnosis was longer in ssSSc (3 years, IQR 1-16.5) than lcSSc (2 years, IQR 0-7), and dcSSc (1 year, IQR 0-3) (p<0.001). Clinical ssSSc phenotype was comparable to lcSSc, except for digital pitting scars (DPS) (19.7% vs 42%, p=0.01), but significantly milder than dcSSc, particularly for digital ulcers (DU) (6.6% vs 35.7%, p<0.001), oesophagus (46.2% vs 63.5%, p=0.009), lung (mean diffusion capacity for carbon monoxide 72.2±19.6 vs 62.4±22.8, p=0.009; mean forced vital capacity 105.6±21.7 vs 89.2±20.9, p<0.001) and major videocapillaroscopic alterations (late pattern 8.6% vs 47.6%, p<0.001). Moreover, in ssSSc the percentages of anticentromere and antitopoisomerase were comparable to lcSSc (40% and 18.3% vs 36.7% and 26.6%), but divergent respect to dcSSc (8.6% and 67.4%, p<0.001). Conclusion The ssSSc is a quite rare disease variant characterised by clinico-serological features comparable to lcSSc, but significantly different from dcSSc. Overall, longer RP duration, low percentages of DPS and peripheral microvascular abnormalities, and increased anti-centromere seropositivity distinguish ssSSc. Further investigations based on national registries might provide useful insights on the actual relevance of the ssSSc within the scleroderma spectrum.
Published: 2023

11. Lung vascular changes as biomarkers of severity in systemic sclerosis-associated interstitial lung disease

Author: Bruni, C., Occhipinti, M., Pienn, M., Camiciottoli, G., Bartolucci, M., Bosello, Silvia Laura, Payer, C., Balint, Z., Larici, Anna Rita, Tottoli, A., Tofani, L., De Lorenzis, E., Lepri, G., Bellando-Randone, S., Spinella, A., Giuggioli, D., Masini, F., Cuomo, G., Lavorini, F., Colagrande, S., Olschewski, H., Matucci-Cerinic, M., Bosello S. L. (ORCID:0000-0002-4837-447X), Larici A. R. (ORCID:0000-0002-1882-6244), Bruni, C., Occhipinti, M., Pienn, M., Camiciottoli, G., Bartolucci, M., Bosello, Silvia Laura, Payer, C., Balint, Z., Larici, Anna Rita, Tottoli, A., Tofani, L., De Lorenzis, E., Lepri, G., Bellando-Randone, S., Spinella, A., Giuggioli, D., Masini, F., Cuomo, G., Lavorini, F., Colagrande, S., Olschewski, H., Matucci-Cerinic, M., Bosello S. L. (ORCID:0000-0002-4837-447X), and Larici A. R. (ORCID:0000-0002-1882-6244)
Abstract: OBJECTIVES: It has recently become possible to assess lung vascular and parenchymal changes quantitatively in thoracic CT images using automated software tools. We investigated the vessel parameters of patients with SSc, quantified by CT imaging, and correlated them with interstitial lung disease (ILD) features. METHODS: SSc patients undergoing standard of care pulmonary function testing and CT evaluation were retrospectively evaluated. CT images were analysed for ILD patterns and total pulmonary vascular volume (PVV) extents with Imbio lung texture analysis. Vascular analysis (volumes, numbers and densities of vessels, separating arteries and veins) was performed with an in-house developed software. A threshold of 5% ILD extent was chosen to define the presence of ILD, and commonly used cut-offs of lung function were adopted. RESULTS: A total of 79 patients [52 women, 40 ILD, mean age 56.2 (s.d. 14.2) years, total ILD extent 9.5 (10.7)%, PVV/lung volume % 2.8%] were enrolled. Vascular parameters for total and separated PVV significantly correlated with functional parameters and ILD pattern extents. SSc-associated ILD (SSc-ILD) patients presented with an increased number and volume of arterial vessels, in particular those between 2 and 4 mm of diameter, and with a higher density of arteries and veins of <6 mm in diameter. Considering radiological and functional criteria concomitantly, as well as the descriptive trends from the longitudinal evaluations, the normalized PVVs, vessel numbers and densities increased progressively with the increase/worsening of ILD extent and functional impairment. CONCLUSION: In SSc patients CT vessel parameters increase in parallel with ILD extent and functional impairment, and may represent a biomarker of SSc-ILD severity.
Published: 2023

12. Combination therapy with Bosentan and Sildenafil improves Raynaud’s phenomenon and fosters the recovery of microvascular involvement in systemic sclerosis

Author: Bellando-Randone, S., Lepri, G., Bruni, C., Blagojevic, J., Radicati, A., Cometi, L., De Paulis, A., Matucci-Cerinic, M., and Guiducci, S.
Published: 2016
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13. Symposia

Author: Correll, Christoph, Kutcher, Stanley P., McClellan, John, Buitelaar, Jan, Pappadopulos, Elizabeth, Rothenberger, Aribert, Mattejat, Fritz, Scott, Stephen, Weisz, John, Schulz, Eberhard, Felder, Wilhelm, Fleischhaker, Christian, Böhme, R., Sixt, B., Jan van der Gaag, Rutger, Katz, Laurence Y., Cox, Brian J., Gunasekara, Shiny, Miller, Alec L., Laor, Nathaniel, Riedesser, Peter, Caffo, Ernesto, Leckman, James, Ammaniti, Massimo, Nicolais, Giampaolo, Speranza, Mario, Steiner, Hans, Delizonna, Laura, Schallauer, Astrid, Thienemann, Margo, McFarlane, Alexander C., van Hooff, Miranda, Sawyer, Michael, Cianchetti, Carlo, Gaddour, Naoufel, Sana, Mokni, Anouar, Mechri, Mondher, Letaief, Lotfi, Gaha, Härtling, Fabian, Bittner, Robert, Haenschel, Corinna, Cap, Marcus, Goncharova, Tanja, Linden, David E. J., Dittmann, Ralf, Maestele, Anneliese, Mehler, Claudia, Meyer, Eberhard, Jenner, Jack A., Boeing, Leonie, Murray, Val, Pelosi, Anthony, McCabe, Robert, Blackwood, Douglas, Wrate, Robert, Pellerano, S., Pintor, M., Mellis, G. L., Piroddi, T., Flisher, Alan, Nesa, Monique, Rooney, Rosanna, Roberts, Clare, Kane, Robert, Silburn, Sven, Pike, Lisbeth, Deaton, Helge Staby, Lustig, Stuart, Funk, Michelle, Rickards, Anne, Reddihough, Dinah, Wright-Rossi, Roslyn, Simpson, Jacqui, Seuthe, Dieter David, Vielhaber, H., Orden, Kinderklinik Dritter, Backmund, H., Gerlinghoff, M., Schwab-Stone, Mary, Jespers, Ine, Vermeiren, Robert, Ruchkin, Vladislav, Blatny, Marek, Hrdlicka, Michal, Urbanek, Tomas, Jelinek, Martin, Balastikova, Veronika, Jeammet, Philippe, Frottin, Alain, Filipovic, Andjelka, Albert, Eric, Schelotto, Dora Musetti, Knezevic, Mladen, Jovancevic, Milivoj, Hill, Jonathan, Lawlor, Maria, Kienbacher, Christian, Prause, Carolin, Stöckl, Margit, Bogyi, Gertrude, Friedrich, Max H., Klein, Michael, Kürschner, Katrin, Murray, Lynne, Leidecker, Victoria, Sharp, Helen, Luoma, Ilona, Kaukonen, Pälvi, Tamminen, Tuula, Nurcombe, Barry, Martin, Graham, McDermott, Brett, Resch, Franz, Schimmelman, Benno Graf, Edwards, Jane, McGorry, Patrick D., Lambert, Martin, Conus, Philippe, Preuss, Ulrich, Bürgin, Dieter, Strauss, Monika, Parzer, Peter, Spiel, Georg, von Korff, C., Ballin, H.-A., Gößler, R., Günter, M., Sange, G., Meng, Heiner, Koch, Eginhard, Minde, Klaus, True, Mary, Pisani, L., Oumar, F., Padilla, J., Bouville, Jean-François, Vogel, Wendy, Schmeck, Klaus, Goth, Kirstin, Purper-Ouakil, Diane, Dessons, Véronique, Doyen, Catherine, Perez-Diaz, Fernando, Mouren-Simeoni, Marie-Christine, Karwautz, Andreas, Wagner, Gudrun, Schwienbacher, Klaus, Haidvogl, Maria, Nobis, Gerald, Treasure, Janet Linda, Collier, David Andrew, Brunner, Romuald, Hueg, A., Haffner, Johann, Schmid, Marc, Goldbeck, Lutz, Nützel, Jakob, Höfling, Volkmar, Schermelleh-Engel, Karin, Moosbrugger, Helfried, Tomàs, Josep, Cornellà, Josep, Llusent, Alex, Bielsa, Anna, Belfer, Myron, Robertson, Brian, Mandlhate, Custodia, Seck, Birama, Zwirs, Barbara, Burger, Huib, Schulpen, Tom, Salman Al-Obedy, A. Karem, Romanchuk, Oleh, Namyslowska, Irena, Reigstad, Björn S., Jorgensen, Kirsti Margrethe, Matthys, Walter, Lochman, John, Zonnevylle-Bender, Marjo, van de Wiel, Nicolle, Wagner, Angela, Jennen-Steinmetz, Christine, Goepel, Christopher, van Bokhoven, Irene, van Goozen, Stephanie, Franciosi, L. Patt, Acquoy, Leode Graaf, Tischlinger, Anne, Pharm, B., Bronder, Knut Halyard, Schleimer, Kari, Walter, Joachim, Ephraime, Boia, Dmitrieva, Tatjana, Silva, Alvaro Seligman, Becker, Katja, Steinhausen, Hans-Christoph, Metzke, C. Winkler, Furtado, Erikson F., Laucht, Manfred, Bilke, Oliver, Zimmermann, Petra, Wittchen, Hans-Ulrich, Lieb, Roselind, Hannesdottir, Helga, Tyrfingsson, Thorarinn, Döpfner, Manfred, Hahlweg, Kurt, Kuschel, Annett, Bertram, Heike, Heinrichs, Nina, Freund-Braier, Inez, Brix, Gabriele, Hautmann, Christopher, Pluck, Julia, Crijnen, Alfons, van Lier, Pol, Vuijk, Patricia, Frank, Reiner, Vandvik, Inger Helene, Schäfert, Rainer, van Weel, Jeanne, Schieveld, Jan, Fegert, Jörg M., Friedrich, William, Celestin-Westreich, Smadar, Celestin, Leon Patrice, Ponjaert-Kristoffersen, Ingrid, Nagao, Keizo, Kisida, M., Shindo, E., Larsen, Helmer Baying, Helweg-Larsen, Karin, Lindauer, Ramón, Booij, Jan, Olff, Miranda, den Heeten, Gerard, Gersons, Berthold, Boer, Frits, Schoentjes, Eric, Bal, Sarah, Schulte-Markwort, Michael, Solantaus, Tytti, Toikka, Sini, Alasuutari, Maarit, Steck, Barbara, Grether, Andrea, Ehrensperger, M., Amsler, Felix, Kappos, L., Saha, Rina, Paschen, Bela, Baldus, Christiane, Haagen, Miriam, Pott, Martina, Romer, Georg, Ono, Yoshiro, Homma, H., Ishida, Y., Ide, H., Okamoto, M., Kameoka, S., Nakayama, Hiroshi, Yamamoto, A., Mukaddes, Nahit Motavalli, Tyano, Sam, Mozes, Tamar, Caplan, R., Malhotra, Savita, Ledda, Maria Giuseppina, Fratta, Al, Mannino, S., Corona, Simona, Zuddas, A., Olalla, Macarena Marin, Garcia, Ruth, Ramirez, Bernardo Perez, Campion, Ross, Hindley, Peter, Gupta, Nitin, Bhattacharaya, Anish, Kapoor, Mehak, van de Willge, G., Klemm, Silke, Smesny, S., Stockebrand, M., Grunwald, S., Juffer, Femmie, van Ijzendoorn, Marinus H., Bakermans-Kranenburg, Marian J., Ziegenhain, Ute, Derksen, B., Dreisörner, R., Gutschner, Daniel, Maldonado-Duran, Martin, Ferndndez-Criado, Manuel, Heidenreich, Felicia, Moro, Marie Rose, Millhuff, Charles, Pope, Kirby, Theisen, Frank, Himmerich, Hubertus, Kraus, T., Schuld, A., Pollmächter, T., Apter, Alan, Gothelf, D., Brand-Gothelf, A., Ratzoni, Gidi, Kikinzon, L., Weizman, A., Bloch, Yuval, Haberhausen, Michael, Müller, Daniel, Fayyad, John, Filho, Altino Bessa Marques, de Menezes, Adolfo Bezerra, Campo, John, Shafer, Sheree, Strohm, Jennifer, Lucas, Amanda, Shaeffer, David, Altman, Harold, Gelachek, Christine, Motomura, Naoyasu, Takino, Yozo, Iwakiri, Masahiro, Pössel, Patrick, Seemann, Simone, Hautzinger, Martin, Mutale, Theodore, Haase, Christian, Abidi, Majid Ali, Raheem, Shehla, Faw, Leyla, Hogue, Aaron, Liddle, Howard, Catthoor, Kirsten, Hutsebaut, Joost, Jasinski, Donald, Faries, Douglas, Moore, Rodney, Streeck-Fischer, Annette, Sannwald, Renate, Barth, Gottfried Maria, Schwarz, Christoph, Staigle, Monika, Pham, Manh-Hiep, Balanzin, Dario, Materi, Joelle, Eresund, Pia, Mokhovikov, Alexander, Stankovic, Sandra, Munir, Kerim, Erol, Nese, Çetin, Füsun Çuhadarodlu, Hassiotis, Angela, Flament, Martine, Scholz, Michael, Rix, Maud, Nestler, Franziska, Selisko, Annegret, Godart, Nathalie, Perdereau, Fabienne, Rein, Zoé, Curt, Florence, Akister, Jane, Lee, Pei-Chin Peggy, Tsai, Sho-Man Susan, Ho, Lai-Shiun, Wu, Su-Chun, Miermont, Jacques, Swenson, Joel, Teherani, Mardjane, Falissard, Bruno, Cottraux, Jean, Plück, Julia, Oades, Robert, Simons-Sprong, Mirjam, Schothorst, P. F., Swaab-Barneveld, J. T., Juran, Stephanie, Weisbrod, Matthias, Chen, Eric, Röpcke, Bernd, Popovic-Deusic, Smiljka, Poustka, Luise, Wild-Wall, Nele, Papousek, Mechthilde, Keren, Mirelle, Feldman, Ruth, Maestro, S., Chilosi, A., Pecini, C., Pfanner, L., Greenhill, Laurence, Jahnsen, K., den Berg, L. T. W. Jong-van, Zito, J. M., Posner, Kelly, Skrobala, Anne, Goldberg, Pablo, Kotler, Lisa, Findling, Robert, Bussing, Regina, Sayal, Kapil, Mitchell, Geoffrey, Huss, Michael, Högl, Barbara, Grimmlinger, Renate, Käppler, Karl Christoph, Teodoro, Maycoln M. L., Oswald, Sylvia Hiromi, Dagnoni, Janine M., Pinheiro, M. I., Heleno, C. T., Rothe-Neves, R., Haase, V. G., Prette, A. Del, Lambertucci, Marimilia Rodrigues, Rodrigues, J. L., Freitas, P. M., Lourenco, C. A. P., Carvalho, H. C. W., Baumeister, J., Weisenhorn, M., Stadelmann, S., Oswald, S. H., Ruder, H., Ruggerini, Ciro, Vicini, Stefania, Pupulin, Enrico, Guidi, Antonio, Puura, Kaija, Mäntymaa, Mirjami, von Klitzing, Kai, Rosvald, Orna, Kröber, Hans-Ludwig, Stöver, A., Proske, R., Semmelbeck, Rainer, Walther, Marc, Schmelzle, Matthias, Egli-Alge, Monika, Beckett, Richard, Gerhold, Constanze, Turkmen-Barta, Lieselotte, Chiland, Colette, Meyer-Bahlburg, Heino, Ceglie, Domenico Di, Lehmkuhl, Ulrike, Uccellini, Orlando, Bertolini, Mario, Neri, Francesca, Albanese, Delia, Bertola, Raffaella, Snoek, Maartje, Kas, Martien, Schulze, Ulrike Margarete Elisabeth, Calame, Silke, Keller, Ferdinand, Santel, Stephanie, Krauel, Kerstin, Rotte, Michael, Münte, Thomas F., van Elburg, Annemarie, Poustka, Fritz, Bölte, Sven, Feineis-Matthews, Sabine, Boite, Sven, Hubl, D., Prvulovic, D., Dierks, T., Klauck, Sabine, Moilanen, Irma, Mattila, M., Laurila, J., Jussila, K., Pyper, A., Linna, S. L., Ebeling, Hanna, Pauls, D., Korpilahti, Pirjo, Loukusa, Soile, Jansson-Verkasalo, Eira, Hebebrand, Johannes, Herpertz-Dahlmann, Beate, Hinney, Anke, Wermter, Anne-Kathrin, Friedel, Susann, Geller, Frank, Schafer, Helmut, Fernandez-Aranda, Fernando, Holliday, Joanna, Holtkamp, Kristian, Mika, C., Heer, M., Uher, Rudolf, Barbarich, Nicole, Henry, Shannan E., Bailer, Ursula, Frank, Guido, Kaye, Walter H., Wentz, Elisabet, Lacey, J. Hubert, Waller, Glenn, Rastam, Maria, Turk, Jeremy, Gillberg, Christopher, Verhulst, Frank C., Zwaanswijk, Marieke, Verhaak, Peter, Bensing, Jozien, van der Ende, Jan, Verhulst, Frank, Sourander, Andre, Santalahti, Paivi, Ford, Tamsin, Goodman, Robert, Meltzer, Howard, Seiffge-Krenke, Inge, Fritsch, Richard, Cutler, Marika, Anthony, E. James, Rydelius, Per-Anders, Castell, Rolf, Pejovic-Milovancevic, Milica, Pavlovic, Miroslav, Kalman, Noa, Linder, Muli, Luria, Ido, Levkovitz, Yechiel, Yamazaki, Kosuke, da Silva, Pedro Caldeira, Santos, Grata, Martins, Filipa, Chiu, Yen-Nan, Tsai, Wen-Jer, Gau, Shur-Fen, Tseng, Chang-Chang, Su, Shu-Chen, Croonenberghs, Jan, Brouw, Lucas, Wauters, Annick, Bruning, Nicole, Manjaly, Zina, Fink, Gereon, Aleksic, Olivera, Rudic, Nenad, Jansen, Lucres, Wied, Christine C. Gispen-De, Lahuis, Bertine, Swaab, Hanna, Pietersen, Jolijn, Gevers, Carolien, Kamp-Becker, Inge, Germerott, Isabell, Howlin, Patricia, Gaudière, Forresi, Barbara, Lepri, G., Laval, Soumaila, Wiefel, Andreas, Biringen, Z., Titze, Karl, Lenz, K., Seither, C., Witte, B., Dunitz-Scheer, Marguerite, Wilken, Markus, Krasnovsky, Alexandra, Scheer, Peter, Cordeiro, Maria, Muratori, Filippo, Felloni, B., Cesari, A., Helmig, Linda, Fonagy, Peter, Moody, Chris, Fultz, Jim, Glanzmann, René, Lutz-Latil, Nathalie, van Wyl, Agnes, Puras, Dainius, Hervds, Amaia, Tsiantis, John, Dragonas, T., Davis, H., Ispanovic, V., Paradisiotou, A., Shanini, Mimoza, Jones, Lynne, Uka, A., Rrustemi, A., von Knorring, Anne-Liis, Deboutte, Dirk, Dorhmi, Souraya, Agoub, Mohamed, Moussaoui, Driss, Battas, Omar, Halvorsen, Inger, Andersen, Anne, Heyerdahl, Sonja, Baillot, Denise, La Roche, Michele, Furino, Claudia, Buchholtz, Annick, Goldfield, Gary, Henderson, Katherine, Hagenah, Ulrich, Blume, Varinja, Flacke-Redanz, Marlene, Dahlmann, Beate Herpertz, Sallas, Angelique A., Adam, Hubertus, Ephraime, Boia, Jr., Mozambique, Goci-Uka, Aferdita, Schlüter-Müller, Susanne, Bawa, Umesh, Khalik, Fakhri, Forouher, Nima, Sadamatsu, Miyuki, Nanba, Kato, Nobumasa, Kasai, Kiyoto, Nanba, Eiji, Schmidt, Martin H., Esser, Günter, van Engeland, Herman, Willemsen-Swinkels, Sophie, Dietz, Claudine, Beernink, Anne Claire, Vidojevic, Oliver, Milacic, Ivona, Strous, Rael, Hegesh, Roni, Kertzman, Simion, Ben-Nahum, Z., Kotler, Moshe, van Daalen, Emma, Zeegers, Mijke, Pol, Hilleke Hulshoff, Williams, Charles, Sank, Jessica, Paulk, Martha, Schwarz, George, Wharton, Paul, Raleva, Marija, Paketchieva, Kamka, Filipovska, Angelina, Nix, Carole Müller, Guex, Margarita Forcada, Daigham, Abdel-Mohsen, Indredavik, Marit S., Vik, Torstein, Kulseng, Siri, Brubakk, Ann-Mari, Cuddy, Marion, Riley, Katharine, Vollmer, Brigitte, Wyatt, John, Murray, Robin, Soininen, Mika, Paavonen, E. Juulia, Fjällberg, Mika, Salmi, Juha, Fredrik, Almgvist, Aronen, Eeva T., Reis, Olaf, Bohne, Stephanie P., Kraenz, Susanne, Ahn, Dong-Hyun, Kim, Tae-Ho, Choi, Jun-Ho, Kim, Yun-Young, Begovac, Ivan, Skocic, Milena, Rudan, Vlasta, Filipovic, Oleg, Wolanczyk, Tomasz, Brynska, Anita, Wojtowicz, Stanislaw, Celia, Salvador, Aerts, Cisca, Cleve, Elisabeth, Hartmann, Hellmut, Kühle, Hans-Jürgen, Heidorn, Fridjof, Zeyer, Solveigh, Fuentes, Joaquin, Martin, Andrés, Sukhodolsky, Denis G., Kaltiala-Heino, Riittakerttu, Rimpelä, Matti, Andershed, Henrik, O’Donnell, Deborah, Pearce, Michelle, Burgin, Dieter, Becker, Andreas, Hagenberg, Nicola, Berking, Matthias, Roessner, Veit, Hanssen-Bauer, Ketil, Aalen, Odd, Junglas, Jürgen, Huh, Yoon-Seok, Kim, Yun Young, Oh, Kyung-Ja, Wang, Kai, Tarren-Sweeney, Michael, Leiblum, D. M., Kühl, Renate, Nötzel, Cornelia, Pfeiffer, Ernst, Lenz, Klaus, Rosling, Agneta, Poller, Marianne, Cross, Donna, Klabin, Simone, Kaplan, Diana, Mickel, Lars, Lehmkuhl, Gerd, Möckel, Regina, Leor, Shani, Frisch, Leor, Frisch, Amos, Weizman, Abraham, Zanozin, Andrey, Jamart, Sylvie, Hayez, Jean-Yves, Leor, Agnes, Ahle, Maria Elisabeth, Amitay, Galit Ben, Kosov, Irene, Reiss, Ahuva, Tamar, Moses, Smedje, Hans, Allik, Hiie, Steyaert, Jean, Castermans, Dries, Creemers, John, Kaczynska-Haladyj, Koenraad Devriendt Marta, Ballabriga, Maria Claustre Jané, Judez, Joaquima, Pelaez, Empar, Sole, Pilar, Rodriguez, Lidia, Palmen, Saskia, Kemner, Chantal, Schnack, Hugo, Kahn, Rene, Fabrizi, Anna, Gabriel, Levi, Mercadante, Marcos, Ramos, Sergiode Paula, Rosario-Campos, Maria Concecao, Rutter, Michael, Collishaw, Stephan, Maughan, Barbara, Pickles, Andrew, Messer, Julie, Caspi, Avshalom, Moffitt, Terrie, Kreppner, Jana, Borge, Anne Inger H., Luthar, Suniya, Hamarman, Stephanie, Ulger, C., Fossella, J., Brimacombe, M., Dermody, J., Stein, Mark, Waldman, L. D., Sarampote, C., Robb, A., Cook, E. H., Kirley, Aiveen, Lowe, N., Hawi, Z., Mullins, C., Daly, G., Waldman, I., McCarro, M., O’Donell, van der Meulen, Emma, Bakker, S. C., Pauls, D. L., Sinke, R. J., Polanczyk, Guilherme, Zeni, C., Genro, J. P., Roman, Tatiana, Hutz, Mara, Schaff, Christa, Haemmerle, Patrick, Sontag, Harald, Vetro, Agnes, Gadoros, Julia, Roosen-Runge, Gotthard, Hattab, Jocelyn, Hummel, Peter, Braun-Lewensohn, Orna, Schechter, Daniel, Zeanah, Charles, Myers, Michael, Liebowitz, Michael, Davies, Mark, Soong, Wei-Tsuen, James, Deborah, Sofroniou, Nick, Gegelashvili, Marine, Parikh, Umesh, Kane, John M., Malhotra, Anil K., Shah, Manoj, Pleak, Richard R., Hizami, Ronen, Michelson, David, Danckaerts, Marina, Zuddas, Alessandro, Zhang, Shuyu, Hazell, Philip, Zeiner, P., Johnson, M., Häßler, Frank, Suyash, Prasad, Sonuga-Barke, Edmund, Poole, Lynne, Mares, Sarah, Jureidini, Jon, Steel, Zachary, Newman, Louise, Lucas, Torsten, Paulus, Stephanie, Aßhauer, Martin, Miller, Birgit, Björn, Gunilla Jarkman, Bodén, Christina, Gustafsson, Per, Ivkic, Viola Povse, Tenjovic, Lazar, Jelena, Radosavljev, Deusic, Smiljka Popovic, Graham, Philip, Klasen, Henrikje, Tan, Jacinta, Hope, Tony, Stewart, Anne, Fitzpatrick, Raymond, Kölch, Michael, Diaz-Caneja, Angeles, Johnson, Sonia, Dippold, Ines, Keller, Katja Wiethoffi Ferdinand, Bailey, Sue, Whittle, Nathan, Hennighausen, Klaus, Kohls, Gregor, Maas, Verena, Rinker, Tanja, Zachau, Swantje, Christmann, Gabriele, Jaremkiewicz, Anna, Schecker, Michael, von Suchodoletz, Waldemar, Uwer, Ruth, Albrecht, Ronald, Glass, Lisa, Csépe, Valéria, Honbology, Ferenc, Rago, Anett, Mészdros, Eva, Schwartz, Richard G., Shafer, Valerie L., Green, Jonathan, Jacobs, C., Kroll, L., Briskman, J., Dunn, G., Beecham, J., Tobias, B., Baird, L., Ogden, Terje, Fitzgerald, Michael, Bellgrove, Mark, Gill, Michael, Robertson, Ian H., McArdle, Paul, Burke, Amanda, Hong, K. Michael, Hoven, Christina, Wasserman, Danuta, Braun, Katharina, Bock, Jórg, Helmecke, Carina, Gruß, Michael, Poeggel, Gerd, Marsden, Charles, Muchimapura, S., Pardon, M.-C., Bianchi, M., Feldon, Joram, Rüedi-Bettschen, Daniela, Dettling, Andrea C., Pryce, Christopher R., Clement, Hans-Willi, Sommer, O., Pschibul, A., Rombach, C., Gerlach, M., Mehler-Wex, Claudia, Zeiske, S., Grünblatt, E., Gille, G., Rausch, D., Gerlach, Manfred, El-Din, Amira Seif, Kadri, Nadia, Andaloussi, Houda Hjiej, Chihabeddine, Khadija, Almaqrami, Mohammed, von Gontard, Alexander, Okuno, M., Quaschner, Kurt, Bilenberg, Niels, Obel, Carsten, Henriksen, Tine Brink, Hedegaard, Morten, Secher, Niels Jurgen, Olsen, Jorn, Fonseca, Antonio, Koch, Isabelle Nathalie, Bite, Ieva, Cohen, Phyllis, Russell, Katrin, Broyden, Nichaela, Lancaster, Gillian, Eichhorn, Christina, Tiedtke, Karola, Feldman, Ronald, Warnke, Andreas, Scheuerpflug, Peter, Vetter, V., Bartling, Jürgen, Konrad, Kerstin, Neufang, Susanne, Hanisch, Charlotte, Fink, Gereon R., Durston, Sarah, Davidson, Matthew C., Tottenham, Nim, Spicer, Julie, Galvan, Adriana, Horvitz, John, Fossella, John A., Watts, Richard, Casey, B. J., Brandeis, Daniel, Fallgatter, Andreas J., Ehlis, Ann-Christine, Seifert, Jürgen, Strik, W. K., Zillessen, K. E., Herrmann, Martin J., Schulte-Körne, Gerd, Lyytinen, Heikki, Guttorm, Tomi, Poikkeus, Anna-Maija, Eklund, Kenneth M., Lyytinen, Paula, Torppa, Minna, Laakso, M.-L., Leskinen, E., Tolvanen, A., Paracchini, Silvia, Schumacher, J., König, I. R., Libertus, Claudia, Griesemann, Heide, Kleensang, A., Ziegler, A., Propping, P., Näthen, M., Wolmer, Leo, Zagout, Iyad, Galili-Weisstub, Esti, Fisch, Gene, Swillen, Ann, Vogels, Annick, Freitag, Christine, Bouville, Jean-Francois, Atlanti-Duault, Laetitia, Baubet, Thierry, Osrow, Robyn, Leplomb, Marie-Madeleine, Marchandy, Yves, Bennabi, Malika, Halpern, Ricardo, Monteiro, Odon, Durkin, Abbey-Robin, Haapanen, Rudy, Bauer, Susanne, Friedrich, Max, Stadler, Christina, Sterzer, Philipp, Kleinschmidt, Andreas, Nowraty, Irene, Müller, W. E., Knölker, Ulrich, Schmid, Gabriele, Berndt, Swantje, Behn, B., Puls, Jan Hendrik, Stevens, Luc, Jungmann, Joachim, Juretic, Zoran, Ercegovic, Nela, Schepker, Renate, Çuhadaroglu-Çetin, Füsun, Herhaus, G., Melfsen, S., Cheng, Daomeng, Harder, Donald, Laws, Harry, Nakane, Yoshibumi, Takeshita, Kenzo, Naruse, Hiroshi, Zhu, Yan, Liu, Jun, Du, Yasong, Sikorski, John, Hamerlynck, Sannie, Hart, Lisettet, Nauta-Janssen, Lucres, Chitsabesan, Prathiba, Nguyen, Hien, Simeon, Jovan, Cuzner, Cathy, Schachter, Howard, Martins, Ana Soledade, Kieling, Chirstian, Comassetto, Julia, Goncalves, Renata, Oswald, Silvia, Buchmann, Johannes, Kirschner, J., Garvey, M., Moll, Gunther, Heinrich, Hartmut, Malhotra, Sameer, Poulakis, Zeffie, Menahem, Sam, Sauer, Karin, Samia, Tilouch, Rimeh, Hannachi, Sonia, Missaoui, Allodi, Mara Westling, Biscaldi, Monica, Wagner, Bettina, Uchida, Chiyoko, Jozefiak, Thomas, Penge, Roberta, Biaggini, Valentina Ivancich, Fischbein, Siv, Joukamaa, Matti, Taanila, Anja, Veijola, Juha, Karvonen, Juha T., Miettunen, Jouko, Llaberia, Edelmira Domenech, Domenech, Teresa Corbella, Ballabriga, Maria Claustre Jane, Sanz, Josepa Canals, Esparo, Griselda, Sola, Sergi Ballespi, Liu, Xuejun, Kano, Yukiko, Ohta, Masataka, Nagai, Yoko, Arai, Takashi, Linyan, Su, Bridge, Jeff, Birmaher, Boris, Di Lorenzo, Carlo, Iyengar, Satish, Brent, David, Blanz, Bernhard, Weninger, Laura, Libal, Gerhard, Skrabal, Anna, Bowden, Michael, Cooper, Howard, Simonsen, Inger, Bechstrom, Carl, Medby, Mette, Erkolahti, Ritva, Klosinski, Gunther, Oba, Mihoko, Murase, Satomi, Murakami, Takashi, Takai, Jiro, Kaneko, Hitoshi, Honjo, Shuji, Rickards, Katrina, Weber, Annhild, Karle, Michael, Lazartigues, Alain, Planche, Pascale, Lemonnier, Eric, Pavuluri, Mani, Schenkel, Lindsay, Shaw, Ryan, Sweeny, John, Rigon, Giancarlo, Costa, Stefano, Mancaruso, Alessandra, Mansi, Roberta, Poggioli, Daniele Giovanni, Chiodo, Simona, Radobuljac, Maja, Groleger, Urban, Ovsenik, Nada, Tomori, Martina, Haas, Barbara, Denoix, Susanne, Kimmig, Franz, Weinhardt, Marc, Schmitz, Günter, Filschke, Berit, Fliegauf, Conny, Kim, Ji-Hae, Krischer, Maya, Stone, Michael H., Sevecke, Kathrin, Doepfner, Manfred, König, Cornelia, Grasmann, Dörte, Schlander, Michael, Ralston, Stephen, Pereira, R. Rodrigues, Brussel, W., Vlasveld, L., Tuynman-Qua, H. G., Lorenzo, M. J., Tauscher-Wisniewski, R., Palazzi, Stefano, Guaia, Ettore, Kolakowski, Artur, Pisula, Agnieszka, Wilens, Timothy, Banaschewski, Tobias, Uebel, Henrik, Albrecht, Björn, Robatzek, Monika, Migliaccio-Walle, Kristen, Caro, Jaime, Allen, Albert J., Sangal, R. Bart, Owens, Judith, Kelsey, Douglas, Sutton, Virginia, Schuh, Kory, Bahadir, Aliye Tugba, Yaman, Zeynep, Arman, Ayse Rodopman, Kuscu, Kemal, Yazgan, Yanki, Berkem, Meral, Feldman, Peter, Denai, Milton, Simpson, Alexander, Kratochvil, Christopher, Newcorn, Jeffrey, Biederman, Joseph, Gelowitz, Douglas, Thomason, Christine, Gao, Haitao, Bijttebier, Patricia, Decoene, Steff, Niklaus, Pia, Duits, Nils, Auer, Ulrich, Schnoor, Kathleen, Schläfke, Detlef, Çetin, Füsun Çuhadaroälu, Harper, Gordon, Hamdan, Sami, El-Haib, Muhammed, Canat, Saynur, Halfon, Olivier, Bolognini, Monique, Plancherel, B., Phan, Olivier, Corcos, Maurice, Cardinaux, Jean-René, Magistretti, Pierre J., Pierrehumbert, Blaise, Koskinen, Minna, Engqvist, Ulf, Allin, Matthew, Rifkin, Larry, Lancaster, Sandra, Borghini, Ayala, Jaugey, Laure, Forcada-Guex, Margarita, Jaunin, Lyne, Müller-Nix, Carole, Ansermet, François, Simoes, Mariada Conceicao Taborda, Lima, Luiza Nobre, Dias, Mariada Luz Vale, Siefen, Rainer Georg, Como, Ariel, Alikaj, Valbona, Tomori, Sonila, Capozzi, Flavia, Romano, Angela, Roello, Mara, Piperno, Francesca, Mann, Mali A., Stösser, Dieter, Barth, Gottfried, Pimenov, Alina, Schwab, Jenny, Bingöl, Hülya, Barbe, Rémy, Elkshishy, Heba, Jovanovic, Ana, Lakic, Aneta, Milovanovic, Vesna, Vukasinovic, Milorad, Bridge, Jeffrey, Kolko, David, Brent, David A., Gilson, Kathryn, Montague, Roslyn, Shochet, Ian, Marques, Cristina Maria Ribeiro, Cepeda, Teresa, Ligges, Carolin, Ligges, Marc, Huonker, Ralph, Leppänen, Paavo, Guttorm, Torni K., Hämäläinen, Jarmo, Puolakanaho, Anne, Plume, Ellen, König, Inke R., Deimel, Wolfgang, Nöthen, Markus M., Propping, Peter, Kleensang, André, Müller-Myhsok, Bertram, Ziegler, Andreas, Hong, Sung-Do David, Gao, Xueping, Li, Xuerong, Lee, Soyoung Irene, Kim, Eui-Jung, Cho, In-Hee, Kim, Ji-Hoon, Park, Se-Hyun, Choi, J-Wook, Heger, Steffen, Schreiner, Andreas, Rettig, Klaus, Medori, Rossella, Gustafsson, Peik, Hansson, Kjell, Eidevall, Lena, Thernlund, Gunilla, Heiser, Philip, Dempfle, A., Smidt, Judith, Grabarkiewicz, Justyna, Kiefl, Hans, Hemminger, U., Saar, K., Swanson, James, Volkov, Nora D., Gupta, S., Williams, L., Agler, D., Wasdell, M., Wigal, S., Martins, Silvia, Tramontina, Silza, Eizirik, Mariana, Vitiello, Benedetto, Clevenger, Walter, Faraone, Stephen, McGough, James, McCracken, James, Rohde, Luis Augusto, Greenhill, Larry, Leary, Michael, Larsson, Bo, Gunning, W. Boudewijn, Villat, Jean-Marie, O’Connell, Redmond, Bellgrove, Mark A., Dockree, Paul, Traube, Raymond, Braunschweig, Mary, Chabanier, Jacques, De Leo, Germana, Ibanez, Margerita, Mikolajaks, Olivette, Ropstad, Ida, Young, J. Gerald, Aiello, Rachele, Porcari, Viviana, Salatiello, Maria Patrizia, Lo Bue, Anna, Dell’Oglio, Valentina, Cardella, Rosaria, Chifari, Sabrina, Undheim, Anne Mari, Su, Linyan, Luo, Xuerong, Barton, Joanne, Baying, Lioba, Rellum, Thomas, Duezel, Emrah, Hinrichs, Hermann, Bartel, Christoph, Linde, Iris, Friederichs, E., Bangs, Mark, Remschmidt, Helmut, Doreleijers, Theodore, Rebernig, Elisabeth, Camerini, Giovanni Battista, Otero, Soraya, Rivas, Ana, Pombo, Guadalupe, Yeghiyan, Maruke, Kachatur, Gasparyan, Danileyan, Arman, Ivarsson, Tord, Valderhaug, Robert, Walitza, Susanne, Wewetzer, Christoph, Barth, Nikolaus, Hahn, F., Asbahr, Fernando, Castillo, Ana Regina, Ito, Ligia, Latorre, Mariado Rosario, Moreira, Michelle, Lotufo-Neto, Francisco, Symann, Sophie, Charlier, Dominique, Plattner, Belinda, Schallauer, Astrid Elisabeth, Mohler, Beat, Staub, P., Müller, Carsten, Oelkers-Ax, Rieke, Fischer, Jochen, Hermanns, Uta, Nickel, Anne, Bolay, Hans Volker, Cherro-Aguerre, Miguel, Sorensen, Merete Juul, Nissen, Judith Becker, Mors, Ole, Thomsen, Per Hove, Sund, Anne Mari, Drugli, May Britt, Wichstrom, Lars, Schwannauer, Matthias, Taylor, Emily, Wrate, Rob, Martin, Matthias, Larsson, Jan-Olov, Larsson, Henrik, Lichtenstein, Paul, Ludolph, Andrea G., Mottaghy, Felix, Kraemer, Susanne, Claus, Dieter, Krause, Bernhard, Fegert, Jbrg M., Hurtig, Tuula, Malakhova, Anna, Maniadaki, Katerina, Kakouros, Efthymios, Jensen, Peter, Garcia, Joe Albert, Glied, Sherry, Crowe, Maura, Foster, E. Michael, Golse, Bernard, Junghanß, Jenny, Salin, Aino-Maija, Rytölä, Päivi, Hiltunen, Pauliina, Remschmidt, Helmut, editor, and Belfer, Myron L., editor
Published: 2004
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14. POS0487 THE CROSSTALK OF THE SKIN-ORAL-GUT MICROBIOME AXIS IN LIMITED AND DIFFUSE CUTANEOUS SYSTEMIC SCLEROSIS

Author: Russo, E., primary, Carboni, D., additional, Baldi, S., additional, Fioretto, B., additional, Romano, E., additional, El Aoufy, K., additional, Ramazzotti, M., additional, Rosa, I., additional, Lepri, G., additional, DI Gloria, L., additional, Bruni, C., additional, Melchiorre, D., additional, Guiducci, S., additional, Manetti, M., additional, Matucci-Cerinic, M., additional, Amedei, A., additional, and Bellando Randone, S., additional
Published: 2022
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15. AB0747 Oral microbiome in rheumatic diseases.What involvement?

Author: Ceccherini, M. T., primary, Bellando-Randone, S., additional, Guiducci, S., additional, Romano, E., additional, Carboni, D., additional, El Aoufy, K., additional, Lepri, G., additional, Cometi, L., additional, Tofani, L., additional, Matucci-Cerinic, M., additional, and Melchiorre, D., additional
Published: 2022
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16. Prognostic Value of Lung Ultrasound B-Lines in Systemic Sclerosis

Author: Gargani, L., Bruni, C., Romei, C., Frumento, P., Moreo, A., Agoston, G., Guiducci, S., Bellando-Randone, S., Lepri, G., Belloli, L., Della Rossa, A., Delle Sedie, A., Stagnaro, C., De Nes, M., Salvadori, S., Mosca, M., Falaschi, F., Epis, O., Picano, E., Matucci-Cerinic, M., and Gargani, Luna
Subjects: Pulmonary and Respiratory Medicine, medicine.medical_specialty, systemic sclerosis, Population, Critical Care and Intensive Care Medicine, Pulmonary function testing, B-lines, 03 medical and health sciences, 0302 clinical medicine, DLCO, Internal medicine, medicine, In patient, 030212 general & internal medicine, education, interstitial lung disease, education.field_of_study, ultrasound, business.industry, Ultrasound, Interstitial lung disease, prognosis, medicine.disease, Rheumatology, Lung ultrasound, 030228 respiratory system, Radiology, Cardiology and Cardiovascular Medicine, business
Abstract: Background A high percentage of systemic sclerosis (SSc) patients experience interstitial lung disease (ILD) during the disease course. Recent data have shown that lung ultrasound (LUS) can assess ILD by the evaluation of B-lines, the sonographic sign of pulmonary interstitial involvement. Research Question To establish the prognostic value of B-lines in a large number of patients with SSc. Study Design and Methods A total of 396 consecutive patients with SSc, who were enrolled at three Rheumatology Departments, underwent a comprehensive LUS examination on the anterolateral and posterior chest for a total of 58 scanning sites. All available clinical, imaging, and functional data were recorded. Patients were followed after enrolment to establish the prognostic role of LUS. Results The median number of B-lines was higher in patients with the diffuse cutaneous subset (44 vs 17 B-lines; P Interpretation Lung ultrasound B-lines are associated with worsening or development of pulmonary deterioration. In the near future, LUS might become part of the diagnostic and prognostic armamentarium in patients with SSc, which would allow a more sustainable and user-friendly approach to this very fragile population.
Published: 2020
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17. Undifferentiated connective tissue disease: State of the art on clinical practice guidelines

Author: Antunes, M, Scire, C, Talarico, R, Alexander, T, Avcin, T, Belocchi, C, Doria, A, Franceschini, F, Galetti, I, Govoni, M, Hachulla, E, Launay, D, Lepri, G, Macieira, C, Matucci-Cerinic, M, Montecucco, C, Moraes-Fontes, M, Mouthon, L, Paolino, S, Ramoni, V, Tani, C, Tas, S, Tincani, A, Van Vollenhoven, R, Zen, M, Fonseca, J, Bombardieri, S, Schneider, M, Smith, V, Cutolo, M, Mosca, M, Beretta, L, Antunes M., Scire C. A., Talarico R., Alexander T., Avcin T., Belocchi C., Doria A., Franceschini F., Galetti I., Govoni M., Hachulla E., Launay D., Lepri G., Macieira C., Matucci-Cerinic M., Montecucco C. M., Moraes-Fontes M. F., Mouthon L., Paolino S., Ramoni V., Tani C., Tas S. W., Tincani A., Van Vollenhoven R., Zen M., Fonseca J., Bombardieri S., Fonseca J. E., Schneider M., Smith V., Cutolo M., Mosca M., Beretta L., Antunes, M, Scire, C, Talarico, R, Alexander, T, Avcin, T, Belocchi, C, Doria, A, Franceschini, F, Galetti, I, Govoni, M, Hachulla, E, Launay, D, Lepri, G, Macieira, C, Matucci-Cerinic, M, Montecucco, C, Moraes-Fontes, M, Mouthon, L, Paolino, S, Ramoni, V, Tani, C, Tas, S, Tincani, A, Van Vollenhoven, R, Zen, M, Fonseca, J, Bombardieri, S, Schneider, M, Smith, V, Cutolo, M, Mosca, M, Beretta, L, Antunes M., Scire C. A., Talarico R., Alexander T., Avcin T., Belocchi C., Doria A., Franceschini F., Galetti I., Govoni M., Hachulla E., Launay D., Lepri G., Macieira C., Matucci-Cerinic M., Montecucco C. M., Moraes-Fontes M. F., Mouthon L., Paolino S., Ramoni V., Tani C., Tas S. W., Tincani A., Van Vollenhoven R., Zen M., Fonseca J., Bombardieri S., Fonseca J. E., Schneider M., Smith V., Cutolo M., Mosca M., and Beretta L.
Abstract: The term a € undifferentiated connective tissue disease' (UCTD) is generally used to describe clinical entities characterised by clinical and serological manifestations of systemic autoimmune diseases but not fulfilling the criteria for defined connective tissue diseases (CTDs). In this narrative review, we summarise the results of a systematic literature research, which was performed as part of the ERN ReCONNET project, aimed at evaluating existing clinical practice guidelines (CPGs) or recommendations. No specific CPG on UCTD were found, potential areas of intervention are absence of a consensus definition of UCTD, need for specific monitoring and therapeutic protocols, stratification of UCTD based on the risk of developing a defined CTD and preventive measure for the future development of a more severe condition. Patients feel uncertainty regarding the name of the disease and feel the need of a better education and understanding of these conditions and its possible changes over time.
Published: 2019

18. A6.28 The role of Plexin-D1/Semaphorin 3E pathway in the dysregulation of vascular tone control in systemic sclerosis (SSc)

Author: Mazzotta, C, Romano, E, Bruni, C, Manetti, M, Lepri, G, Bellando-Randone, S, Blagojevic, J, Ibba-Manneschi, L, Matucci-Cerinic, M, and Guiducci, S
Published: 2015
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19. POS0842 LUNG ULTRASOUND TO ASSESS THE SEVERITY OF INTERSTITIAL LUNG DISEASE IN SYSTEMIC SCLEROSIS

Author: Bruni, C., primary, Mattolini, L., additional, Tofani, L., additional, Gargani, L., additional, Landini, N., additional, Lepri, G., additional, Orlandi, M., additional, Guiducci, S., additional, Bellando Randone, S., additional, and Matucci-Cerinic, M., additional
Published: 2021
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20. AB0334 THE FIRST 1000 DAYS OF LIFE AND REPRODUCTIVE DISORDERS IN WOMAN WITH RHEUMATIC DISEASE (RDS)

Author: Orlandi, M., primary, Vannuccini, S., additional, El Aoufy, K., additional, Melis, M. R., additional, Lepri, G., additional, Bellando-Randone, S., additional, Guiducci, S., additional, Matucci-Cerinic, M., additional, and Petraglia, F., additional
Published: 2021
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21. POS0317 THE PERFORMANCE OF DIFFUSING CAPACITY FOR MONOXIDE CARBON (DLCO) AND FORCED VITAL CAPACITY (FVC) IN PREDICTING THE ONSET OF SYSTEMIC SCLEROSIS (SSc)-INTERSTITIAL LUNG DISEASE (ILD) IN THE EUROPEAN SCLERODERMA TRIALS AND RESEARCH (EUSTAR) DATABASE

Author: Lepri, G., primary, Bruni, C., additional, Tofani, L., additional, Moggi Pignone, A., additional, Orlandi, M., additional, Sara, T., additional, Hughes, M., additional, Del Galdo, F., additional, Irace, R., additional, Distler, O., additional, Riccieri, V., additional, Allanore, Y., additional, Gheorghiu, A. M., additional, Siegert, E., additional, De Vries-Bouwstra, J., additional, Hachulla, E., additional, Tikly, M., additional, Damjanov, N., additional, Spertini, F., additional, Mouthon, L., additional, Hoffmann-Vold, A. M., additional, Gabrielli, A., additional, Guiducci, S., additional, Matucci-Cerinic, M., additional, Furst, D., additional, and Bellando Randone, S., additional
Published: 2021
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22. Systemic sclerosis: State of the art on clinical practice guidelines

Author: Smith, V, Scire, C, Talarico, R, Airo, P, Alexander, T, Allanore, Y, Bruni, C, Codullo, V, Dalm, V, De Vries-Bouwstra, J, Della Rossa, A, Distler, O, Galetti, I, Launay, D, Lepri, G, Mathian, A, Mouthon, L, Ruaro, B, Sulli, A, Tincani, A, Vandecasteele, E, Vanhaecke, A, Vanthuyne, M, Van Den Hoogen, F, Van Vollenhoven, R, Voskuyl, A, Zanatta, E, Bombardieri, S, Burmester, G, Eurico, F, Frank, C, Hachulla, E, Houssiau, F, Mueller-Ladner, U, Schneider, M, Van Laar, J, Vieira, A, Cutolo, M, Mosca, M, Matucci-Cerinic, M, Smith V., Scire C. A., Talarico R., Airo P., Alexander T., Allanore Y., Bruni C., Codullo V., Dalm V., De Vries-Bouwstra J., Della Rossa A., Distler O., Galetti I., Launay D., Lepri G., Mathian A., Mouthon L., Ruaro B., Sulli A., Tincani A., Vandecasteele E., Vanhaecke A., Vanthuyne M., Van Den Hoogen F., Van Vollenhoven R., Voskuyl A. E., Zanatta E., Bombardieri S., Burmester G., Eurico F. J., Frank C., Hachulla E., Houssiau F., Mueller-Ladner U., Schneider M., Van Laar J. M., Vieira A., Cutolo M., Mosca M., Matucci-Cerinic M., Smith, V, Scire, C, Talarico, R, Airo, P, Alexander, T, Allanore, Y, Bruni, C, Codullo, V, Dalm, V, De Vries-Bouwstra, J, Della Rossa, A, Distler, O, Galetti, I, Launay, D, Lepri, G, Mathian, A, Mouthon, L, Ruaro, B, Sulli, A, Tincani, A, Vandecasteele, E, Vanhaecke, A, Vanthuyne, M, Van Den Hoogen, F, Van Vollenhoven, R, Voskuyl, A, Zanatta, E, Bombardieri, S, Burmester, G, Eurico, F, Frank, C, Hachulla, E, Houssiau, F, Mueller-Ladner, U, Schneider, M, Van Laar, J, Vieira, A, Cutolo, M, Mosca, M, Matucci-Cerinic, M, Smith V., Scire C. A., Talarico R., Airo P., Alexander T., Allanore Y., Bruni C., Codullo V., Dalm V., De Vries-Bouwstra J., Della Rossa A., Distler O., Galetti I., Launay D., Lepri G., Mathian A., Mouthon L., Ruaro B., Sulli A., Tincani A., Vandecasteele E., Vanhaecke A., Vanthuyne M., Van Den Hoogen F., Van Vollenhoven R., Voskuyl A. E., Zanatta E., Bombardieri S., Burmester G., Eurico F. J., Frank C., Hachulla E., Houssiau F., Mueller-Ladner U., Schneider M., Van Laar J. M., Vieira A., Cutolo M., Mosca M., and Matucci-Cerinic M.
Abstract: Systemic sclerosis (SSc) is an orphan disease characterised by autoimmunity, fibrosis of the skin and internal organs, and vasculopathy. SSc may be associated with high morbidity and mortality. In this narrative review we summarise the results of a systematic literature research, which was performed as part of the European Reference Network on Rare and Complex Connective Tissue and Musculoskeletal Diseases project, aimed at evaluating existing clinical practice guidelines or recommendations. Only in the domains 'Vascular & Ulcers' (ie, non-pharmacological approach to digital ulcer), 'PAH' (ie, screening and treatment), 'Treatment' and 'Juveniles' (ie, evaluation of juveniles with Raynaud's phenomenon) evidence-based and consensus-based guidelines could be included. Hence there is a preponderance of unmet needs in SSc referring to the diagnosis and (non-)pharmacological treatment of several SSc-specific complications. Patients with SSc experience significant uncertainty concerning SSc-related taxonomy, management (both pharmacological and nonpharmacological) and education. Day-to-day impact of the disease (loss of self-esteem, fatigue, sexual dysfunction, and occupational, nutritional and relational problems) is underestimated and needs evaluation.
Published: 2018

23. Further evidence that chilblains are a cutaneous manifestation of COVID ‐19 infection

Author: Hughes, M., primary, Rogers, S., additional, Lepri, G., additional, Bruni, C., additional, and Matucci‐Cerinic, M., additional
Published: 2020
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24. OP0181 FUNCTIONAL CUT-OFFS TO DISTINGUISH PULMONARY VASCULAR AND PARENCHYMAL INVOLVEMENT IN SYSTEMIC SCLEROSIS (SSC): A QUANTITATIVE ANALYSIS OF IMAGING FEATURES AT CHEST COMPUTED TOMOGRAPHY (CT)

Author: Bruni, C., primary, Occhipinti, M., additional, Camiciottoli, G., additional, Bartolucci, M., additional, Lepri, G., additional, Fabbrizzi, A., additional, Tottoli, A., additional, Bassetto, A., additional, Ciardi, G., additional, Giuggioli, D., additional, Cuomo, G., additional, Masini, F., additional, Lavorini, F., additional, Calistri, L., additional, and Matucci-Cerinic, M., additional
Published: 2020
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25. SAT0308 SCREENING TOOLS FOR PULMONARY ARTERIAL HYPERTENSION (PAH) IN SYSTEMIC SCLEROSIS (SSC): A SYSTEMATIC LITERATURE REVIEW (SLR).

Author: Bruni, C., primary, De Luca, G., additional, Lazzaroni, M. G., additional, Zanatta, E., additional, Lepri, G., additional, and Matucci-Cerinic, M., additional
Published: 2020
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26. THU0360 EFFICACY OF A SELF-TREATMENT PROTOCOL FOR FACE AND TEMPOROMANDIBULAR JOINTS REHABILITATION IN SYSTEMIC SCLEROSIS (SSC)

Author: Passalacqua, M., primary, Foggi, C., additional, Mauro, N., additional, Tofani, L., additional, Guiducci, S., additional, Bruni, C., additional, Lepri, G., additional, Blagojevic, J., additional, El Aoufy, K., additional, Fiori, G., additional, Bartoli, F., additional, Maddali Bongi, S., additional, Mitola, M., additional, Gizduloch, M., additional, Matucci-Cerinic, M., additional, and Bellando Randone, S., additional
Published: 2020
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27. AB0550 DIFFUSING CAPACITY OF THE LUNG FOR CARBON MONOXIDE (DLCO) VS FORCED VITAL CAPACITY (FVC): SYSTEMATIC LITERATURE REVIEW AND META-ANALYSIS TO EXAMINE THEIR ABILITY TO MEASURE CHANGE IN CLINICAL TRIALS IN SYSTEMIC SCLEROSIS (SSC)

Author: Bellando Randone, S., primary, Cappellini, E., additional, Nidiaci, L., additional, Lepri, G., additional, Lazzaroni, M. G., additional, Campochiaro, C., additional, Bagnato, G., additional, Sambataro, D., additional, Sambataro, G., additional, Matucci-Cerinic, M., additional, and Furst, D., additional
Published: 2020
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28. SAT0553 QUANTITATIVE ANALYSIS OF IMAGING FEATURES AT CHEST CT OF PULMONARY ARTERIAL AND VENOUS COMPONENTS IN SYSTEMIC SCLEROSIS-INTERSTITIAL LUNG DISEASE (SSc-ILD).

Author: Bruni, C., primary, Occhipinti, M., additional, Camiciottoli, G., additional, Bartolucci, M., additional, Pienn, M., additional, Lepri, G., additional, Fabbrizzi, A., additional, Tottoli, A., additional, Ciardi, G., additional, Giuggioli, D., additional, Cuomo, G., additional, Masini, F., additional, Olschewski, H., additional, Lavorini, F., additional, Calistri, L., additional, and Matucci-Cerinic, M., additional
Published: 2020
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29. Incidence and risk factors for gangrene in patients with systemic sclerosis from the EUSTAR cohort

Author: Mihai, Carina, Distler, Oliver, Gheorghiu, Ana Maria, Constantin, Paul I, Dobrota, Rucsandra, Jordan, Suzana, Smith, Vanessa, Hachulla, Eric, Henes, Jörg, Siegert, Elise, Vettori, Serena, Müller-Ladner, Ulf, Matucci Cerinic, Marco, Allanore, Yannick, Lepri, G, Jaeger, Vk, Walker, Ua, Iannone, F, Cacciapaglia, F, Tomčík, M, Becvar, R, Rednic, S, Petcu, A, Szabo, I, Codullo, V, Caporali, R, Montecucco, C, Carreira, P, Ioven, B, Minier, T, Czirják, L, Chizzolini, C, Allali, D, Zanatta, E, Doria, A, Gabrielli, A, Airò, P, Lazzaroni, Mg, Radić, M, Martinovic, D, Braun-Moscovici, Y, Balbir-Gurman, A, Hunzelmann, N, Caramaschi, P, Morovic-Vergles, J, Denton, C, Santamaria, V, Heitmann, S, Krasowska, D, Michalska-Jakubus, M, Seidel, M, Foeldvari, I, Helmus, N, Salvador, M, Stamenkovic, B, Stankovic, A, Ananieva, L, Herrick, A, Engelhart, M, De La Puente, C, Hoffmann-Vold, Am, Midtvedt, Ø, Launay, D, Sobanski, V, Riccieri, V, Opris-Belinski, D, Groseanu, L, Ionescu, R, Bojinca, M, Sunderkötter, C, Distler, J, Ingegnoli, F, van der Haecke, A, Ullman, S, Pozzi, Mr, Eyerich, K, Vanthuyne, M, Erler, A, Aringer, M, De Langhe, E, Baresic, M, Mayer, M, Anic, B, Yavuz, S, Granel, B, Popa, S, Agachi, S, Zenone, T, Mathieu, A, Vacca, A, Solanki, K, Veale, D, Loyo, E, Tineo, C, Gigante, A, Rosato, E, Oksel, F, Yagurcu, F, Tănăseanu, Cm, Visalli, E, Benenati, A, Foti, R, Ancuta, C, Dan, D, Adler, S, Villiger, P, Fathi, N, de la Peña Lefebvre PG, González Martín, J, Chatelus, E, Sibilia, J, Litinsky, I, Del Galdo, F, Ann Sakettkoo, L, Kerzberg, E, Bianchi, Wa, Bianchi, Bv, Castellví, I, Limonta, M, Rimar, D, Couto, M, Ribi, C, Spertini, F, Kahl, S, Hsu, V, Poindron, V, Meghit, K, Martin, T, Kolstad, K, Chung, L, Thiele, A, Schmeiser, T, Zdrojewski, Z, Riemekasten, G, Levy, Y, Cardoneanu, A, Burlui, A, Rezus, E, Pamuk, On, Talotta, R, Bongiovanni, S, Puttini, Ps., Mihai, Carina, Distler, Oliver, Gheorghiu, Ana Maria, Constantin, Paul I, Dobrota, Rucsandra, Jordan, Suzana, Smith, Vanessa, Hachulla, Eric, Henes, Jörg, Siegert, Elise, Vettori, Serena, Müller-Ladner, Ulf, Matucci Cerinic, Marco, Allanore, Yannick, Giovanna, Cuomo, Chizzolini, Carlo, Allali, Danièle, and University of Zurich
Subjects: Adult, Male, medicine.medical_specialty, Databases, Factual, systemic sclerosis, digital ulcer, 610 Medicine & health, Disease, ddc:616.07, Logistic regression, Systemic scleroderma, Cohort Studies, Rheumatology, Risk Factors, Internal medicine, Cox proportional hazards regression, medicine, Humans, Pharmacology (medical), In patient, digital ulcers, gangrene, vasculopathy, Aged, Gangrene, Scleroderma, Systemic, business.industry, Incidence (epidemiology), Incidence, 10051 Rheumatology Clinic and Institute of Physical Medicine, food and beverages, Middle Aged, medicine.disease, Cross-Sectional Studies, Cohort, Female, business, systemic sclerosi
Abstract: Objective In patients with SSc, peripheral vasculopathy can promote critical ischaemia and gangrene. The aim of this study was to investigate the prevalence, incidence and risk factors for gangrene in the EUSTAR cohort. Methods We included patients from the EUSTAR database fulfilling the ACR 1980 or the ACR/EULAR 2013 classification criteria for SSc, with at least one visit recording data on gangrene. Centres were asked for supplementary data on traditional cardiovascular risk factors. We analysed the cross-sectional relationship between gangrene and its potential risk factors by univariable and multivariable logistic regression. Longitudinal data were analysed by Cox proportional hazards regression. Results 1757 patients were analysed (age 55.9 [14.5] years, disease duration 7.9 [10.3] years, male sex 16.7%, 24.6% diffuse cutaneous subset [dcSSc]). At inclusion, 8.9% of patients had current or previous digital gangrene, 16.1% had current digital ulcers (DUs) and 42.7% had ever had DUs (current or previous). Older age, DUs ever and dcSSc were statistically significant risk factors for gangrene in the cross-sectional multivariable model. During a median follow-up of 13.1 months, 16/771 (0.9%) patients developed gangrene. All 16 patients who developed gangrene had previously had DUs and gangrene. Further risk factors for incident gangrene were the dcSSc subset and longer disease duration. Conclusion In unselected SSc patients, gangrene occurs in about 9% of SSc patients. DUs ever and, to a lesser extent, the dcSSc subset are strongly and independently associated with gangrene, while traditional cardiovascular risk factors could not be identified as risk factors.
Published: 2019

30. Clinical-serological characterization and treatment outcome of a large cohort of Italian children with Pediatric Autoimmune Neuropsychiatric Disorder Associated with Streptococcal Infection and Pediatric Acute Neuropsychiatric Syndrome.

Author: Lepri, G, Rigante, Donato, Bellando Randone, S, Meini, A, Ferrari, A, Tarantino, Giusyda, Cunningham, Mw, Falcini, F, Rigante D (ORCID:0000-0001-7032-7779), Tarantino G, Lepri, G, Rigante, Donato, Bellando Randone, S, Meini, A, Ferrari, A, Tarantino, Giusyda, Cunningham, Mw, Falcini, F, Rigante D (ORCID:0000-0001-7032-7779), and Tarantino G
Abstract: Objective: Pediatric autoimmune neuropsychiatric disorder associated with Streptococcus pyogenes infection (PANDAS) and pediatric acute-onset neuropsychiatric syndrome (PANS) are emerging immune-mediated encephalopathies characterized by sudden onset of seemingly inexplicable complex neuropsychiatric symptoms, including obsessions, compulsions, and heterogeneous tics, which occur in children. Main goal of this study was to report our experience in a large cohort of Italian children affected by either PANDAS or PANS and treated long term with an antibiotic regimen similar to that used for acute rheumatic fever. Patients and Methods: The clinical charts of a cohort of 371 consecutive Italian children, 345 with PANDAS (93.0%) and 26 with PANS (7.0%), were retrospectively evaluated. Antistreptococcal, antinuclear antibodies, and serologic evaluation for a group of common autoantibodies and microbial agents were also assessed. A strict differential diagnosis with other autoimmune diseases displaying neuropsychiatric manifestations was performed. Results: Antistreptolysin O and anti-DNase B antibody titers were tested and were positive in all PANDAS subjects, but negative in PANS. Anti-Mycoplasma pneumoniae antibodies and anti-Epstein-Barr virus Nuclear Antigen antibodies were found positive in 11 (42.3%) and 5 (19.2%) patients with PANS, respectively. Among PANDAS cases, a clear streptococcal infection was clinically evident at the onset of neurological symptoms in only 74 patients (21.4%), whereas the relationship with Streptococcus pyogenes was confirmed by serologic tests in the other 271 (78.6%). All patients fulfilling the diagnostic criteria for PANDAS (n = 345) received amoxicillin/clavulanic acid for 10-21 days at diagnosis, while those who were diagnosed with PANS (n = 26) received treatment according to the causative agent. Thereafter, all PANDAS/PANS patients received prophylaxis with benzathine benzylpenicillin for an overall period of at least 5 years to pre
Published: 2019

31. Agricultural cooperative 'Co.r.ag.gio.', an example of urban agriculture in Rome (Italy) as a resilient strategy against urban climate change

Author: MARICCHIOLO, FRIDANNA, PANNO, ANGELO, CARRUS, GIUSEPPE, Lepri, G., Brizi, A., N. Kabisch, J, Stadler, S. Duffield, H. Korn, A. Bonn, Maricchiolo, Fridanna, Lepri, G., Brizi, A., Panno, Angelo, and Carrus, Giuseppe
Abstract: In urban contexts, cultivated agricultural land increases permeability, improves positive atmospheric exchanges, protects the complexity of agricultural ecosystem and helps general resilience to urban climate changes. We present the Italian case study of the EU-FP7 funded project called “GLAMURS - Green Lifestyles, alternative models and upscaling regional sustainability” (www.glamurs.eu). GLAMURS investigates transitions to sustainable lifestyles and green economies, through an interdisciplinary approach in seven different regions of Europe. In the Lazio Region of Italy, the young agricultural cooperative "Co.R.Ag.Gio" (“Courage”), COoperativa Romana AGricoltura GIOvani (Roman Agricultural Cooperative of Youth), encourages citizens and institutions to conserve environmental heritages abandoned in the Roman agricultural outskirts. CoRAgGio was founded in 2011, as a free association of young people (farmers, agronomists, chefs, architects, day workers, industrial worker, anthropologists, educators). It promotes an original work perspective in this economic crisis, enhancing passions and experiences in agricultural and horticultural activities, teaching and training (educational farms, courses in sustainable agriculture), food (spreading good practices, 0-km production), crafts. It strives to make public land available to all citizens, and preserve agricultural soils from the expansion of the building sector. It promotes an agricultural urban model that is healthy, organic, multi-functional, replacing the degraded concrete buildings with a proposal of a new way of living, based on ecological concerns, respecting labour dignity, and social meanings of agriculture. The implications of promoting sustainable urban agriculture will be discussed, in terms of economic values, ecological services and food production, improvement of life quality, soil protection, earth resources and biodiversity conservation.
Published: 2017

32. One year in review 2018: systemic vasculitis

Author: Baldini, C., Quartuccio, L., Talarico, R., Posarelli, C., Calabresi, E., Felicetti, M., Cavallaro, E., Lepri, G., Sara Monti, Bond, M., and Elefante, E.
Subjects: Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis, Antirheumatic Agents, Churg-Strauss Syndrome, Cryoglobulinemia, Giant Cell Arteritis, Granulomatosis with Polyangiitis, Hepatitis C, Chronic, Humans, Immunosuppressive Agents, Microscopic Polyangiitis, Systemic Vasculitis, Takayasu Arteritis, Hepatitis C, Chronic
Abstract: Systemic vasculitis are heterogeneous, complex and disabling disorders. Following the previous annual reviews of this series, this paper gives a brief overview on current knowledge about recent literature on small- and large-vessel systemic vasculitis, with a specific focus on pathogenetic and clinical aspects, novel possible disease-related biomarkers and current and future therapies that are in the pipeline.
Published: 2018

33. Systemic sclerosis: State of the art on clinical practice guidelines

Author: Smith, V. (Vanessa), Scirè, C.A. (Carlo Alberto), Talarico, R. (Rosaria), Airo, P. (Paolo), Alexander, T. (Tobias), Allanore, Y. (Yannick), Bruni, C. (Cosimo), Codullo, V. (Veronica), Dalm, V.A.S.H. (Virgil), Vries-Bouwstra, J.K. (Jeska) de, Della Rossa, A. (Alessandra), Distler, O. (Oliver), Galetti, I. (Ilaria), Launay, D. (David), Lepri, G. (Gemma), Mathian, A. (Alexis), Mouthon, L. (Luc), Ruaro, B. (Barbara), Sulli, A. (Alberto), Tincani, A. (Angela), Vandecasteele, E. (Els), Vanhaecke, A. (Amber), Vanthuyne, M. (Marie), Hoogen, F.H.J. van den, Van Vollenhoven, R.F. (Ronald F.), Voskuyl, R.A. (Robert), Zanatta, E. (Elisabetta), Bombardieri, S. (Stefano), Burmester, G.R. (Gerd), Eurico, F.J. (Fonseca João), Frank, C. (Charissa), Hachulla, E. (Eric), Houssiau, F. (Frederic), Mueller-Ladner, U. (Ulf), Schneider, M. (Matthias), Van Laar, J.M. (Jacob M), Vieira, A. (Ana), Cutolo, M. (Maurizio), Mosca, M. (Marta), Matucci-Cerinic, M. (Marco), Smith, V. (Vanessa), Scirè, C.A. (Carlo Alberto), Talarico, R. (Rosaria), Airo, P. (Paolo), Alexander, T. (Tobias), Allanore, Y. (Yannick), Bruni, C. (Cosimo), Codullo, V. (Veronica), Dalm, V.A.S.H. (Virgil), Vries-Bouwstra, J.K. (Jeska) de, Della Rossa, A. (Alessandra), Distler, O. (Oliver), Galetti, I. (Ilaria), Launay, D. (David), Lepri, G. (Gemma), Mathian, A. (Alexis), Mouthon, L. (Luc), Ruaro, B. (Barbara), Sulli, A. (Alberto), Tincani, A. (Angela), Vandecasteele, E. (Els), Vanhaecke, A. (Amber), Vanthuyne, M. (Marie), Hoogen, F.H.J. van den, Van Vollenhoven, R.F. (Ronald F.), Voskuyl, R.A. (Robert), Zanatta, E. (Elisabetta), Bombardieri, S. (Stefano), Burmester, G.R. (Gerd), Eurico, F.J. (Fonseca João), Frank, C. (Charissa), Hachulla, E. (Eric), Houssiau, F. (Frederic), Mueller-Ladner, U. (Ulf), Schneider, M. (Matthias), Van Laar, J.M. (Jacob M), Vieira, A. (Ana), Cutolo, M. (Maurizio), Mosca, M. (Marta), and Matucci-Cerinic, M. (Marco)
Abstract: Systemic sclerosis (SSc) is an orphan disease characterised by autoimmunity, fibrosis of the skin and internal organs, and vasculopathy. SSc may be associated with high morbidity and mortality. In this narrative review we summarise the results of a systematic literature research, which was performed as part of the European Reference Network on Rare and Complex Connective Tissue and Musculoskeletal Diseases project, aimed at evaluating existing clinical practice guidelines or recommendations. Only in the domains 'Vascular & Ulcers' (ie, non-pharmacological approach to digital ulcer), 'PAH' (ie, screening and treatment), 'Treatment' and 'Juveniles' (ie, evaluation of juveniles with Raynaud's phenomenon) evidence-based and consensus-based guidelines could be included. Hence there is a preponderance of unmet needs in SSc referring to the diagnosis and (non-)pharmacological treatment of several SSc-specific complications. Patients with SSc experience significant uncertainty concerning SSc-related taxonomy, management (both pharmacological and nonpharmacological) and education. Day-to-day impact of the disease (loss of self-esteem, fatigue, sexual dysfunction, and occupational, nutritional and relational problems) is underestimated and needs evaluation.
Published: 2018
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34. Cross-sectional evaluation of plasma vitamin D levels in a large cohort of Italian patients with Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infection

Author: Stagi, S, Lepri, G, Rigante, Donato, Matucci Cerinic, M, Falcini, F., Rigante D (ORCID:0000-0001-7032-7779), Stagi, S, Lepri, G, Rigante, Donato, Matucci Cerinic, M, Falcini, F., and Rigante D (ORCID:0000-0001-7032-7779)
Abstract: BACKGROUND: Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS) are immune-mediated diseases characterized by obsessive-compulsive symptoms and/or tics triggered by group A Streptococcus infections. Despite the well-known action of 25-hydroxyvitamin D [25(OH)D] on different conditions driven by systemic inflammation, there are no data about the 25(OH)D status in patients with PANDAS. AIMS: To evaluate plasma 25(OH)D levels in a large cohort of children and adolescents with PANDAS and comparing the results with healthy controls. METHODS: We have evaluated plasma 25(OH)D levels in 179 Italian patients with PANDAS (49 females, 130 males, mean age at diagnosis: 101.4 ± 30.1 months) and in an age-, gender-, and body mass index-matched control group of 224 healthy subjects. RESULTS: Patients with PANDAS have shown more frequently reduced 25(OH)D levels (<30 ng/mL) in comparison with controls (94.6% vs. 82.5%, p = 0.0007). Patients with PANDAS had also lower levels of 25(OH)D than controls (20.4 ± 6.9 ng/mL vs. 24.8 ± 7.3 ng/mL, p < 0.0001). This difference was observed during both winter (13.7 ± 3.25 ng/mL vs. 21.4 ± 5.9 ng/mL, p < 0.0001) and summer (21.8 ± 6.5 ng/mL vs. 32.5 ± 8.7 ng/mL, p < 0.0001). Notably, serum 25(OH)D levels correlated with both number of streptococcal (strep) infections before diagnosis of PANDAS (p < 0.005) and with infection recurrence (p < 0.005). CONCLUSIONS: PANDAS patients have reduced 25(OH)D levels, which appear related to streptococcal infections and the probability of recurrence. Further long-term studies with higher number of patients are needed to investigate and confirm this relationship.
Published: 2018

35. Da Grande Farò il Pompiere: il ruolo dei genitori nelle scelte di formazione Universitaria

Author: Nota, L, Soresi, S, Camussi, E, Gritti, A, Annovazzi, C, Frasca, C, Patrizi, P, Lepri G., L, Lodi, E, Bussu, A, CAMUSSI, ELISABETTA, GRITTI, ALICE, ANNOVAZZI, CHIARA, Lepri G. , L, Bussu, A., Nota, L, Soresi, S, Camussi, E, Gritti, A, Annovazzi, C, Frasca, C, Patrizi, P, Lepri G., L, Lodi, E, Bussu, A, CAMUSSI, ELISABETTA, GRITTI, ALICE, ANNOVAZZI, CHIARA, Lepri G. , L, and Bussu, A.
Published: 2015

36. Psicologia positiva e servizi di consulenza universitari:Promuovendo il benessere delle/degli studenti

Author: Patrizi, P, Lepri G, L, Lodi, E, Bussu, A, Camussi, E, Gritti, A, Annovazzi, C, Scaringi, S, Nota, L, Soresi, S, Patrizi, P, Lepri G, L, Lodi, E, Bussu, A, Camussi, E, Gritti, A, Annovazzi, C, and Scaringi, S
Subjects: Psicologia dell'Orientamento, Università, Life-Design, Career Counselling, M-PSI/05 - PSICOLOGIA SOCIALE
Published: 2015

37. Da Grande Farò il Pompiere: il ruolo dei genitori nelle scelte di formazione Universitaria

Author: CAMUSSI, ELISABETTA, GRITTI, ALICE, ANNOVAZZI, CHIARA, Frasca, C, Patrizi, P, Lepri G. , L, Lodi, E, Bussu, A., Nota, L, Soresi, Camussi, E, Gritti, A, Annovazzi, C, Frasca, C, Patrizi, P, Lepri G., L, Lodi, E, and Bussu, A
Subjects: Psicologia dell'Orientamento, adolescenti, genitori, università, Career Counselling
Published: 2015

38. THU0425 18f-fluorodeoxyglucose positron emission tomography/computed tomography and lung involvement in systemic sclerosis

Author: Bellando Randone, S., primary, Tartarelli, L., additional, Cavigli, E., additional, Tofani, L., additional, Bruni, C., additional, Lepri, G., additional, Blagojevic, J., additional, Moggi Pignone, A., additional, Mihai, C., additional, Avouac, J., additional, Passeri, A., additional, De Cristofaro, M.T., additional, Distler, O., additional, Allanore, Y., additional, Guiducci, S., additional, and Matucci Cerinic, M., additional
Published: 2018
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39. PANDAS and PANS: underestimated and unrecognized neuropsychiatric syndromes in children

Author: Falcini, F, Lepri, G, Ferrari, A, Rigante, Donato, Matucci Cerinic, M, Meini, A., Rigante, Donato (ORCID:0000-0001-7032-7779), Falcini, F, Lepri, G, Ferrari, A, Rigante, Donato, Matucci Cerinic, M, Meini, A., and Rigante, Donato (ORCID:0000-0001-7032-7779)
Abstract: We describe a large cohort of pediatric patients with PANDAS and PANS, two abrubtly-starting neuropsychiatric syndromes in children with an infection-related pathogenesis.
Published: 2017

40. Vitamin D receptor polymorphisms are not associated with the risk of Kawasaki disease (KD) in a group of italian children

Author: Falcini, F, Marini, F, Stagi, S, Rigante, Donato, Lepri, G, Matucci Cerinic, M, Brandi, Ml, Rigante, Donato (ORCID:0000-0001-7032-7779), Falcini, F, Marini, F, Stagi, S, Rigante, Donato, Lepri, G, Matucci Cerinic, M, Brandi, Ml, and Rigante, Donato (ORCID:0000-0001-7032-7779)
Abstract: We have herein evaluated vitamin D receptor polymorphisms in a cohort of Italian pediatric patients with Kawasaki disease confirming a negative association with the disease risk.
Published: 2017

41. Un servizio di counseling universitario in ottica comunitaria: per contrastare le vulnerabilità sociali e fronteggiare le situazioni di rischio

Author: Patrizi, P., Lepri, G., Lodi, E., Magnano, Paola, and Boerchi, D.
Published: 2016

42. FRI0380 Patient preferences and difficulties concerning the home treatment options in systemic sclerosis (SSC)

Author: Pagnotelli, B, primary, Bruni, C, additional, Randone, S Bellando, additional, Guiducci, S, additional, Lepri, G, additional, Blagojevic, J, additional, Rasero, L, additional, Cerinic, M Matucci, additional, and Furst, DE, additional
Published: 2017
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43. Systemic sclerosis sine scleroderma: clinical and serological features and relationship with other cutaneous subsets in a large series of patients from the national registry ‘SPRING’ of the Italian Society for Rheumatology

Author: Carlo Alberto Scirè, Andrea Doria, Marcello Govoni, Gerolamo Bianchi, Marco Matucci-Cerinic, Florenzo Iannone, Ennio Lubrano, Corrado Campochiaro, Veronica Codullo, Alessandra Della Rossa, Gemma Lepri, Elisabetta Zanatta, Beretta Lorenzo, Doria Andrea, Lepri Gemma, Lorenzo Beretta, Greta Carrara, Alarico Ariani, Simone Parisi, Marta Saracco, Francesco Girelli, Maria De Santis, Federica Lumetti, Dilia Giuggioli, Enrico Fusaro, Simone Barsotti, Ilaria Cavazzana, Federica Furini, Carlo Salvarani, Serena Guiducci, Franco Cozzi, Valeria Riccieri, Francesca Ingegnoli, Edoardo Rosato, Antonietta Gigante, Rosario Foti, Elisa Visalli, Fabio Cacciapaglia, Lorenzo Dagna, Franco Franceschini, Silvia Bellando-Randone, Giovanna Cuomo, Gianluigi Bajocchi, Alessandro Giollo, Giacomo De Luca, Giuseppina Abignano, Carlo Sciré, Anna Zanetti, Giovanni Zanframundo, Edoardo Cipolletta, Silvia Laura Bosello, Clodoveo Ferri, Amelia Spinella, Giuseppa Pagano Mariano, Maurizio Caminiti, Giuseppe Murdaca, Salvatore D'Angelo, Gianpiero Landolfi, Nicoletta Romeo, Gian Domenico Sebastiani, Erika Pigatto, Rossella De Angelis, Davide Rozza, Maria-Grazia Lazzaroni, Anna Maria Iuliano, Giovanni Ciano, Gianluca Bagnato, Ilenia De Andres, Cecilia Agnes, Luca Magnani, Claudio Di Vico, Greta Pellagrino, Elena Generali, Gianna Mennillo, Licia Vultaggio, Clara Lisa Peroni, Abignano Giuseppina, Agnes Cecilia, Amato Giorgio, Ariani Alarico, Bagnato Gianluca, Bajoicchi Gianluigi, Barsotti Simone, Bellando-Randone Silvia, Benenati Alessia, Bianchi Gerolamo, Bosello Silvia, Cacciapaglia Fabio, Calabrese Francesca, Caminiti Maurizio, Campochiaro Corrado, Carignola Renato, Ciano Giovanni, Cipolletta Edoardo, Codullo Veronica, Cozzi Franco, Cuomo Giovanna, D’Angelo Salvatore, Dagna Lorenzo, Dall’Ara Francesca, De Andres Ilenia, De Angelis Rossella, De Cata Angelo, De Luca Giacomo, De Santis Maria, Della Rossa Alessandra, Di Vico Claudio, Doveri Marica, Foti Rosario, Furini Federica, Fusaro Enrico, Generali Elena, Gigante Antonietta, Giollo Alessandro, Girelli Francesco, Giuggioli Dilia, Govoni Marcello, Guiducci Serena, Iannone Florenzo, Ingegnoli Francesca, Iuliano Anna Maria, Lazzaroni Maria Grazia, Lubrano Ennio, Lumetti Federica, Magnani Luca, Mennillo Gianna, Murdaca Giuseppe Ospedale, Pagano Mariano Giuseppa, Parisi Simone, Pellegrino Greta, Peroni Clara Lisa, Pigatto Erika, Riccieri Valeria, Romeo Nicoletta, Rosato Edoardo, Sambataro Gianluca, Saracco Marta, Sebastiani Giandomenico, Spinella Amelia, Talotta Rossella, Visalli Elisa, Vultaggio Licia, Zanatta Elisabetta, and Zanframundo Giovanni
Subjects: Medicine
Abstract: Objective To describe demographic, clinical and laboratory features of systemic sclerosis sine scleroderma (ssSSc) in a large multicentre systemic sclerosis (SSc) cohort.Methods Data involving 1808 SSc patients from Italian Systemic sclerosis PRogression INvestiGation registry were collected. The ssSSc was defined by the absence of any cutaneous sclerosis and/or puffy fingers. Clinical and serological features of ssSSc were compared with limited cutaneous (lcSSc) and diffuse cutaneous (dcSSc) subsets.Results Among patients with SSc, only 61 (3.4%) were classified as having ssSSc (F/M=19/1). Time from Raynaud’s phenomenon (RP) onset to diagnosis was longer in ssSSc (3 years, IQR 1–16.5) than lcSSc (2 years, IQR 0–7), and dcSSc (1 year, IQR 0–3) (p
Published: 2023
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44. The largest cohort of children and adolescents with Pediatric Autoimmune Neuropsychiatric Disorder Associated with Streptococcus Infection (PANDAS): preliminary descriptive analysis

Author: Falcini, F, Meini, A, Lepri, G, Rigante, Donato, Ferrari, A, Casalini, E, and Matucci Cerinic, M.
Subjects: Pediatric Autoimmune Neuropsychiatric Disorder Associated with Streptococcus Infection, Settore MED/38 - PEDIATRIA GENERALE E SPECIALISTICA
Published: 2015

45. Severe vitamin D deficiency in patients with Kawasaki disease: its possible role in the risk to develop coronary artery damage

Author: Falcini, F, Stagi, S, Lepri, G, Casalini, E, Rigante, Donato, and Matucci Cerinic, M.
Subjects: Kawasaki disease, Settore MED/38 - PEDIATRIA GENERALE E SPECIALISTICA
Published: 2015

46. Preliminary analysis of the Very Early Diagnosis of Systemic Sclerosis (VEDOSS) EUSTAR multicentre study: evidence for puffy fingers as a pivotal sign for suspicion of systemic sclerosis

Author: Minier, T., Guiducci, S., Bellando-Randone, S., Bruni, C., Lepri, G., Czirjak, L., Distler, O., Walker, U. A., Fransen, J., Allanore, Y., Denton, C., Cutolo, M., Tyndall, A., Muller-Ladner, U., Matucci-Cerinic, M., Airo, P., Zingarelli, S., Ananieva, L., Desinova, O., Ancuta, C. M., Belibou, C. I., Avouac, J., Becvar, R., Skacelova, S., Beretta, L., Vigone, B., Caramaschi, P., Sabbagh, D., Carpentier, P., Damjanov, N., Simic-Pasalic, K., Distler, J. H. W., Farge-Bancel, D., Hadj-Khelifa, S., Foti, R., Di Gangi, M., De La Pena Lefebvre, P. G., Hachulla, E., Salvador, M. J., Kayser, C., Camargo, C. Z., Kumanovics, G., Li, M., Xu, D., Marasini, B., Belloli, L., Maurer, B., Mayer, M., Mihai, C., Gherghe, A. M., Riccieri, V., Stefanantoni, K., Salsano, F., Rosato, E., Senecal, J. -L., Koenig, M., Senet, P., Frances, C., Sipek, A., Stankovic, A., Stamenkovic, B., Smith, V., Tarner, I. H., Wiland, P., University of Zurich, and Matucci-Cerinic, Marco
Subjects: Male, Anti-nuclear antibody, Pulmonary Fibrosis, 2745 Rheumatology, Scleroderma, Microscopic Angioscopy, Cohort Studies, Antinuclear, Immunology and Allergy, skin and connective tissue diseases, integumentary system, 10051 Rheumatology Clinic and Institute of Physical Medicine, Middle Aged, Connective tissue disease, Autoantibodies, Disease Activity, Outcomes research, Systemic Sclerosis, Adult, Antibodies, Antinuclear, Early Diagnosis, Female, Fingers, Humans, Raynaud Disease, Scleroderma, Systemic, Skin Ulcer, Telangiectasis, Cohort, 2723 Immunology and Allergy, musculoskeletal diseases, medicine.medical_specialty, Immunology, 610 Medicine & health, General Biochemistry, Genetics and Molecular Biology, Antibodies, Rheumatology, 1300 General Biochemistry, Genetics and Molecular Biology, Internal medicine, medicine, 2403 Immunology, business.industry, Systemic, Autoantibody, medicine.disease, Surgery, stomatognathic diseases, Inflammatory diseases Radboud Institute for Health Sciences [Radboudumc 5], Observational study, business, Rheumatism
Abstract: Item does not contain fulltext OBJECTIVES: The EULAR (European League Against Rheumatism) Scleroderma Trials and Research Group (EUSTAR) has identified preliminary criteria for very early diagnosis of systemic sclerosis (SSc). Our aim was to assess the prevalence of each proposed diagnostic item in a large observational patient cohort with Raynaud's phenomenon (RP). METHODS: Baseline data of 469 RP patients enrolled into the Very Early Diagnosis of Systemic Sclerosis (VEDOSS) cohort are presented. RESULTS: 68% of all RP patients were antinuclear antibody (ANA) positive. ANA+ RP patients more frequently had previous or current puffy fingers (PuFi) (38.5% and 23.3%, p
Published: 2014

47. Severe vitamin D deficiency in patients with Kawasaki disease: a potential role in the risk to develop heart vascular abnormalities?

Author: Stagi, S, Rigante, Donato, Lepri, G, Matucci Cerinic, M, Falcini, F., Rigante, Donato (ORCID:0000-0001-7032-7779), Stagi, S, Rigante, Donato, Lepri, G, Matucci Cerinic, M, Falcini, F., and Rigante, Donato (ORCID:0000-0001-7032-7779)
Abstract: Twenty-five-hydroxyvitamin D (25(OH)-vitamin D) is crucial in the regulation of immunologic processes, but-although its deficiency has been reported in patients with different rheumatological disorders-no data are available for Kawasaki disease (KD). The goals of this study were to assess the serum levels of 25(OH)-vitamin D in children with KD and evaluate the relationship with the eventual occurrence of KD-related vascular abnormalities. We evaluated serum 25(OH)-vitamin D levels in 79 children with KD (21 females, 58 males, median age 4.9 years, range 1.4-7.5 years) in comparison with healthy sex-/age-matched controls. A significantly higher percentage of KD patients (98.7 %) were shown to have reduced 25(OH)-vitamin D levels (<30 ng/mL) in comparison with controls (78.6 %, p < 0.0001). Furthermore, KD patients had severely low levels of 25(OH)-vitamin D than controls (9.17 ± 4.94 vs 23.3 ± 10.6 ng/mL, p < 0.0001), especially the subgroup who developed coronary artery abnormalities (4.92 ± 1.36 vs 9.41 ± 4.95 ng/mL, p < 0.0001). In addition, serum 25(OH)-vitamin D levels correlated not only with erythrosedimentation rate (p < 0.0001), C-reactive protein (p < 0.0001), hemoglobin level at KD diagnosis (p < 0.0001) but also with both coronary artery aneurysms (p = 0.005) and non-aneurysmatic cardiovascular lesions (p < 0.05). Low serum concentrations of 25(OH)-vitamin D might have a contributive role in the development of coronary artery complications observed in children with KD.
Published: 2016

48. Vitamin D receptor polymorphisms are not associated with the risk of Kawasaki disease (KD) in a group of Italian children

Author: Falcini, F, Marini, F, Stagi, S, Rigante, Donato, Lepri, G, Matucci Cerinic, M, Brandi, Ml, Rigante, Donato (ORCID:0000-0001-7032-7779), Falcini, F, Marini, F, Stagi, S, Rigante, Donato, Lepri, G, Matucci Cerinic, M, Brandi, Ml, and Rigante, Donato (ORCID:0000-0001-7032-7779)
Abstract: This description deals with the evaluation of a relationship between vitamin D receptor polymorphisms in a group of Italian children and the risk to develop Kawasaki disease, the most important vasculitis in the pediatric age.
Published: 2016

49. Clinical overview of a cohort of 87 Italian patients with Pediatric Autoimmune Neuropsychiatric Disorder Associated with Streptococcus infection (PANDAS)

Author: Falcini, F, Lepri, G, Bertini, F, Tarantino, G, Matucci Cerinic, M, and Rigante, Donato
Subjects: Pediatric autoimmune neuropsychiatric disorders associated with Streptococcal infection, Settore MED/38 - PEDIATRIA GENERALE E SPECIALISTICA
Published: 2014

50. THU0641-HPR The Challenge of Pet Therapy in Rheumatology: Evidence for The Improvement of Patients Compliance in Systemic Sclerosis

Author: Fiori, G., primary, Bartoli, F., additional, Marzi, T., additional, Bruni, C., additional, Lepri, G., additional, Bellando-Randone, S., additional, Guiducci, S., additional, Denaro, V., additional, Tesei, G., additional, and Matucci-Cerinic, M., additional
Published: 2016
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