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

Author

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

Subjects
medicine.medical_specialty, antisynthetase antibodies, antisynthetase syndrome, arthritis, interstitial lung disease, myositis, Medizin, Arthritis, lcsh:Medicine, Antisynthetase syndrome, Interstitial lung disease, Article, NO, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, medicine, antisynthetase antibodies, antisynthetase syndrome, arthritis, interstitial lung disease, myositis, ddc:610, Myositis, 030203 arthritis & rheumatology, Antisynthetase antibodies, biology, business.industry, lcsh:R, Autoantibody, General Medicine, medicine.disease, arthriti, 030228 respiratory system, Time course, Cohort, biology.protein, Antibody, business, antisynthetase antibodie
Abstract

Antisynthetase syndrome (ASSD) is a rare clinical condition that is characterized by the occurrence of a classic clinical triad, encompassing myositis, arthritis, and interstitial lung disease (ILD), along with specific autoantibodies that are addressed to different aminoacyl tRNA synthetases (ARS). Until now, it has been unknown whether the presence of a different ARS might affect the clinical presentation, evolution, and outcome of ASSD. In this study, we retrospectively recorded the time of onset, characteristics, clustering of triad findings, and survival of 828 ASSD patients (593 anti-Jo1, 95 anti-PL7, 84 anti-PL12, 38 anti-EJ, and 18 anti-OJ), referring to AENEAS (American and European NEtwork of Antisynthetase Syndrome) collaborative group's cohort. Comparisons were performed first between all ARS cases and then, in the case of significance, while using anti-Jo1 positive patients as the reference group. The characteristics of triad findings were similar and the onset mainly began with a single triad finding in all groups despite some differences in overall prevalence. The "ex-novo" occurrence of triad findings was only reduced in the anti-PL12-positive cohort, however, it occurred in a clinically relevant percentage of patients (30%). Moreover, survival was not influenced by the underlying anti-aminoacyl tRNA synthetase antibodies' positivity, which confirmed that antisynthetase syndrome is a heterogeneous condition and that antibody specificity only partially influences the clinical presentation and evolution of this condition. ispartof: JOURNAL OF CLINICAL MEDICINE vol:8 issue:11 ispartof: location:Switzerland status: published

Published
2019

2. Anti-RNA polymerase III antibodies: A marker of systemic sclerosis with rapid onset and skin thickening progression

Author

Ilaria, Cavazzana, Angela, Ceribelli, Ceribelli, Angela, Paolo, Airo', Airo', Paolo, Stefania, Zingarelli, Zingarelli, Stefania, Angela, Tincani, Tincani, Angela, Franco, Franceschini, and Franceschini, Franco

Subjects
Male, Pathology, medicine.medical_specialty, Anti-nuclear antibody, Immunology, Rapid systemic sclerosis onset, Systemic Sclerosis, Disease, Scleroderma, RNA polymerase III, Antibody Specificity, medicine, Humans, Immunology and Allergy, skin and connective tissue diseases, Autoantibodies, Skin, Scleroderma, Systemic, integumentary system, biology, Anti-RNA polymerase III, Skin thickening, business.industry, Systemic, Autoantibody, RNA Polymerase III, Middle Aged, Anti-nuclear antibodies, medicine.disease, Raynaud's phenomenon, Skin thickening progression, Rapid onset, Disease Progression, biology.protein, Female, Biomarkers, Antibody, business
Abstract

Anti-RNA polymerase III antibodies (ARA) are a specific marker for Systemic Sclerosis (SSc), associated to severe disease with major organ and diffuse cutaneous involvement. In our series, ARA were found in 19 of 216 sera, in 15 cases as isolated antibodies' specificity, with a statistically negative association with other SSc-specific autoantibodies (p: 0.00003). The prevalence of ARA among 73 anticentromere and anti-topoisomerase I (topo I) negative sera, was 20.5%. Patients with isolated ARA had more rapid disease onset, defined as the interval from the appearance of Raynaud's phenomenon to the first symptom other than Raynaud's, than patients with isolated anti-topo I antibodies (median: 2 months vs 13 months; p: 0.0013). A rapid onset of SSc (within 6 months from Raynaud's phenomenon onset) was found in all patients with isolated ARA and only in 34% of those with anti-topo I (p

Published
2009

3. International cohort study of 73 anti-Ku-positive patients: association of p70/p80 anti-Ku antibodies with joint/bone features and differentiation of disease populations by using principal-components analysis

Author

Lakota, Katja, Thallinger, Gerhard, Sodin-Semrl, Snezna, Rozman, Blaz, Ambrozic, Ales, Tomsic, Matija, Praprotnik, Sonja, Cucnik, Sasa, Mrak-Poljsak, Katjusa, Ceribelli, Angela, Cavazzana, Ilaria, Franceschini, Franco, Vencovsky, Jiri, Czirjak, Laszlo, Varju, Cecilia, Steiner, Gunther, Aringer, Martin, Stamenkovic, Bojana, Distler, Oliver, and Matucci-Cerinic, Marco

Published
2012

8. Early and Late Response and Glucocorticoid-Sparing Effect of Belimumab in Patients with Systemic Lupus Erythematosus with Joint and Skin Manifestations: Results from the Belimumab in Real Life Setting Study—Joint and Skin (BeRLiSS-JS)

Author

Margherita Zen, Mariele Gatto, Roberto Depascale, Francesca Regola, Micaela Fredi, Laura Andreoli, Franco Franceschini, Maria Letizia Urban, Giacomo Emmi, Fulvia Ceccarelli, Fabrizio Conti, Alessandra Bortoluzzi, Marcello Govoni, Chiara Tani, Marta Mosca, Tania Ubiali, Maria Gerosa, Enrica P. Bozzolo, Valentina Canti, Paolo Cardinaletti, Armando Gabrielli, Giacomo Tanti, Elisa Gremese, Ginevra De Marchi, Salvatore De Vita, Serena Fasano, Francesco Ciccia, Giulia Pazzola, Carlo Salvarani, Simone Negrini, Andrea Di Matteo, Rossella De Angelis, Giovanni Orsolini, Maurizio Rossini, Paola Faggioli, Antonella Laria, Matteo Piga, Alberto Cauli, Salvatore Scarpato, Francesca Wanda Rossi, Amato De Paulis, Enrico Brunetta, Angela Ceribelli, Carlo Selmi, Marcella Prete, Vito Racanelli, Angelo Vacca, Elena Bartoloni, Roberto Gerli, Elisabetta Zanatta, Maddalena Larosa, Francesca Saccon, Andrea Doria, Luca Iaccarino, Zen, Margherita, Gatto, Mariele, Depascale, Roberto, Regola, Francesca, Fredi, Micaela, Andreoli, Laura, Franceschini, Franco, Letizia Urban, Maria, Emmi, Giacomo, Ceccarelli, Fulvia, Conti, Fabrizio, Bortoluzzi, Alessandra, Govoni, Marcello, Tani, Chiara, Mosca, Marta, Ubiali, Tania, Gerosa, Maria, Bozzolo, Enrica P., Canti, Valentina, Cardinaletti, Paolo, Gabrielli, Armando, Tanti, Giacomo, Gremese, Elisa, De Marchi, Ginevra, De Vita, Salvatore, Fasano, Serena, Ciccia, Francesco, Pazzola, Giulia, Salvarani, Carlo, Negrini, Simone, Di Matteo, Andrea, DE ANGELIS, Rossella, Orsolini, Giovanni, Rossini, Maurizio, Faggioli, Paola, Laria, Antonella, Piga, Matteo, Cauli, Alberto, Scarpato, Salvatore, Wanda Rossi, Francesca, De Paulis, Amato, Brunetta, Enrico, Ceribelli, Angela, Selmi, Carlo, Prete, Marcella, Racanelli, Vito, Vacca, Angelo, Bartoloni, Elena, Gerli, Roberto, Zanatta, Elisabetta, Larosa, Maddalena, Saccon, Francesca, Doria, Andrea, and Iaccarino, Luca

Subjects
disease activity score (DAS)-28, low disease activity, remission, systemic lupus erythematosus, belimumab, Cutaneous LE Area and Severity Index (CLASI), Medicine (miscellaneous)
Abstract

Aim. To assess the efficacy of belimumab in joint and skin manifestations in a nationwide cohort of patients with SLE. Methods. All patients with skin and joint involvement enrolled in the BeRLiSS cohort were considered. Belimumab (intravenous, 10 mg/kg) effectiveness in joint and skin manifestations was assessed by DAS28 and CLASI, respectively. Attainment and predictors of DAS28 remission (

Published
2023

9. Dose-Dependent Impairment of the Immune Response to the Moderna-1273 mRNA Vaccine by Mycophenolate Mofetil in Patients with Rheumatic and Autoimmune Liver Diseases

Author

Maria De Santis, Francesca Motta, Natasa Isailovic, Massimo Clementi, Elena Criscuolo, Nicola Clementi, Antonio Tonutti, Stefano Rodolfi, Elisa Barone, Francesca Colapietro, Angela Ceribelli, Matteo Vecellio, Nicoletta Luciano, Giacomo Guidelli, Marta Caprioli, Clara Rezk, Lorenzo Canziani, Elena Azzolini, Luca Germagnoli, Nicasio Mancini, Ana Lleo, Carlo Selmi, De Santis, Maria, Motta, Francesca, Isailovic, Natasa, Clementi, Massimo, Criscuolo, Elena, Clementi, Nicola, Tonutti, Antonio, Rodolfi, Stefano, Barone, Elisa, Colapietro, Francesca, Ceribelli, Angela, Vecellio, Matteo, Luciano, Nicoletta, Guidelli, Giacomo, Caprioli, Marta, Rezk, Clara, Canziani, Lorenzo, Azzolini, Elena, Germagnoli, Luca, Mancini, Nicasio, Lleo, Ana, and Selmi, Carlo

Subjects
Pharmacology, Infectious Diseases, antirheumatic agents, Drug Discovery, Immunology, mycophenolate mofetil, COVID-19, Pharmacology (medical), autoimmune diseases, vaccination
Abstract

The purpose of this study was to evaluate the efficacy and safety of the Moderna-1273 mRNA vaccine for SARS-CoV-2 in patients with immune-mediated diseases under different treatments. Anti-trimeric spike protein antibodies were tested in 287 patients with rheumatic or autoimmune diseases (10% receiving mycophenolate mofetil, 15% low-dose glucocorticoids, 21% methotrexate, and 58% biologic/targeted synthetic drugs) at baseline and in 219 (76%) 4 weeks after the second Moderna-1273 mRNA vaccine dose. Family members or caretakers were enrolled as the controls. The neutralizing serum activity against SARS-CoV-2-G614, alpha, and beta variants in vitro and the cytotoxic T cell response to SARS-CoV-2 peptides were determined in a subgroup of patients and controls. Anti-SARS-CoV-2 antibody development, i.e., seroconversion, was observed in 69% of the mycophenolate-treated patients compared to 100% of both the patients taking other treatments and the controls (p < 0.0001). A dose-dependent impairment of the humoral response was observed in the mycophenolate-treated patients. A daily dose of >1 g at vaccination was a significant risk factor for non-seroconversion (ROC AUC 0.89, 95% CI 0.80–98, p < 0.0001). Moreover, in the seroconverted patients, a daily dose of >1 g of mycophenolate was associated with significantly lower anti-SARS-CoV-2 antibody titers, showing slightly reduced neutralizing serum activity but a comparable cytotoxic response compared to other immunosuppressants. In non-seroconverted patients treated with mycophenolate at a daily dose of >1 g, the cytotoxic activity elicited by viral peptides was also impaired. Mycophenolate treatment affects the Moderna-1273 mRNA vaccine immunogenicity in a dose-dependent manner, independent of rheumatological disease.

Published
2022

10. PFAPA syndrome and Behçet's disease: a comparison of two medical entities based on the clinical interviews performed by three different specialists

Author

Francesco Caso, Donato Rigante, Carlo Selmi, Antonio Vitale, Marco Cattalini, Giovanni Lapadula, Luisa Costa, Luca Cantarini, Giuseppe Lopalco, Angela Ceribelli, Mauro Galeazzi, Enrico Brunetta, Florenzo Iannone, Giulia Bersani, Laura Martin Nieves, Cantarini, Luca, Vitale, Antonio, Bersani, Giulia, Nieves, Laura Martin, Cattalini, Marco, Lopalco, Giuseppe, Caso, Francesco, Costa, Luisa, Iannone, Florenzo, Lapadula, Giovanni, Galeazzi, Mauro, Ceribelli, Angela, Brunetta, Enrico, Selmi, Carlo, and Rigante, Donato

Subjects
0301 basic medicine, Pediatrics, Behcet's disease, Disease, Behçet’s disease, 0302 clinical medicine, Stomatitis, Pediatric, Behçet's disease, Behcet Syndrome, Pharyngitis, General Medicine, Syndrome, Middle Aged, Settore MED/38 - PEDIATRIA GENERALE E SPECIALISTICA, Italy, Recurrent fever, Autoinflammation, medicine.symptom, Human, Lymphadeniti, Adult, medicine.medical_specialty, PFAPA syndrome, Adolescent, Fever, Interviews as Topic, 03 medical and health sciences, Young Adult, Rheumatology, Lymphadenitis, medicine, Internal Medicine, Humans, Medical history, Symptom onset, 030203 arthritis & rheumatology, business.industry, medicine.disease, Stomatiti, Interleukin-1, stomatognathic diseases, 030104 developmental biology, Pharyngiti, Physical therapy, business
Abstract

The pediatric syndrome characterized by periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) and adult Behçet's disease share some clinical manifestations and are both polygenic autoinflammatory disorders with interleukin-1β showing to play a pivotal role. However, the diagnosis is mostly clinical and we hypothesize that specific criteria may be addressed differently by different physicians. To determine the diagnostic variability, we compared the answers of 80 patients with a definite diagnosis of Behçet's disease (age 42.1 ± 13.7 years) obtained by separate telephone interviews conducted by a rheumatologist, a pediatrician, and an internist working largely in the field of autoinflammatory disorders. Questions were related to the age of symptom onset, the occurrence of recurrent fevers during childhood, and the association with oral aphthosis, cervical adenitis and/or pharyngitis, previous treatments, possible growth impairment, the time lapse between PFAPA-like symptoms and the onset of Behçet's disease, and the occurrence of Behçet-related manifestation during childhood. The rheumatologist identified 30 % of patients with Behçet's disease fulfilling PFAPA syndrome diagnostic criteria, compared to the pediatrician and the internist identifying 10 and 7.5 %, respectively. Most of the patients suffered from recurrent oral aphthosis in childhood also without fever (50, 39, and 48 % with each interviewer), yet no patient fulfilled the Behçet's disease diagnostic criteria. Our data suggest that physician awareness and expertise are central to the diagnosis of autoinflammatory disorders through an accurate collection of the medical history.

Published
2014

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