Matthias Papo1, Renato A Sinico2, Vítor Teixeira3,4, Nils Venhoff5, Maria-Letizia Urban6, Michele Iudici1,7, Juliane Mahrhold8, Francesco Locatelli9, Giulia Cassone10, Franco Schiavon11, Benjamin Seeliger12, Thomas Neumann13,14, Claus Kroegel15, Matthieu Groh16, Chiara Marvisi17, Maxime Samson18, Thomas Barba19, David Jayne4, Arianna Troilo5, Jens Thiel5, Bernhard Hellmich7, Sara Monti9, Carlomaurizio Montecucco9, Carlo Salvarani20, Jean-Emmanuel Kahn16, Bernard Bonnotte18, Cécile-Audrey Durel19, Xavier Puéchal1, Luc Mouthon1, Loïc Guillevin1, Giacomo Emmi6, Augusto Vaglio1,2, Benjamin Terrier1. 1. Department of Internal Medicine, Cochin Hospital, National Referral Center for Rare Systemic Autoimmune Diseases, Paris University, Paris, France. 2. Department of Medicine and Surgery, Università degli Studi di Milano-Bicocca, Milano/Monza, Italy. 3. Department of Medicine, University of Cambridge, Cambridge, UK. 4. Department of Rheumatology, Centro Hospitalar Universitário do Algarve, Portugal. 5. Department of Rheumatology and Clinical Immunology, Medical Center - University of Freiburg, Faculty of Medicine, Freiburg, Germany. 6. Department of Experimental and Clinical Medicine, University of Firenze, Firenze, Italy. 7. Rheumatology Unit, Geneva University Hospitals, Geneva, Switzerland. 8. Department of Internal Medicine, Rheumatology and Immunology, Vasculitis-Center Tübingen-Kirchheim, Medius Klinik Kirchheim, University of Tübingen, Kirchheim-Teck, Germany. 9. Department of Rheumatology, IRCCS Policlinico S. Matteo Foundation, University of Pavia, Pavia, Italy. 10. Clinical and Experimental Medicine PhD Program, Azienda USL-IRCCS di Reggio Emilia and Università di Modena and Reggio Emilia, Italy. 11. Operative Unit of Rheumatology, Department of Medicine DIMED, University of Padova, Padova, Italy. 12. Department of Respiratory Medicine, Hannover Medical School, Hannover, Germany. 13. Department of Rheumatology, Clinic of Internal Medicine II I, Jena University Hospital, Jena, Germany. 14. Division of Rheumatology, Immunology and Rehabilitation, Kantonsspital St Gallen, St Gallen, Switzerland. 15. Department of Pneumology and Allergology, Clinic of Internal Medicine I, Jena University Hospital, Jena, Germany. 16. Service de Médecine Interne, Centre de Référence des Syndromes Hyperéosinophiliques-CEREO, Hôpital Foch, Université Versailles-Saint-Quentin-en-Yvelines, Suresnes, France. 17. Rheumatology Unit Università di Modena and Reggio Emilia, Modena, Italy. 18. Department of Internal Medicine and Clinical Immunology, François-Mitterrand Teaching Hospital, University of Bourgogne-Franche-Comté, Dijon, France. 19. Department of Internal Medicine, Hôpital Edouard Herriot, Lyon, France. 20. Azienda USL-IRCCS di Reggio Emilia, Università di Modena and Reggio Emilia.
Abstract
OBJECTIVES: Only a third of patients with eosinophilic granulomatosis with polyangiitis (EGPA) are ANCA-positive, mainly directed against MPO. ANCA directed against PR3 are rarely found in EGPA. We aimed to examine the significance of PR3-ANCA in EGPA. METHODS: We set up a retrospective European multicentre cohort including 845 patients. Baseline characteristics and outcomes were analysed and compared according to ANCA status. RESULTS: ANCA status was available for 734 patients: 508 (69.2%) ANCA-negative, 210 (28.6%) MPO-ANCA and 16 (2.2%) PR3-ANCA. At baseline, PR3-ANCA patients, compared with those with MPO-ANCA and ANCA-negative, less frequently had active asthma (69% vs 91% and 93%, P = 0.003, respectively) and peripheral neuropathy (31% vs 71% and 47%, P < 0.0001), more frequently had cutaneous manifestations (63% vs 38% and 34%, P = 0.03) and pulmonary nodules (25% vs 10% and 8%, P = 0.046), and lower median eosinophil count (1450 vs 5400 and 3224/mm3, P < 0.0001). Vasculitis relapse-free survival was shorter for PR3-ANCA (hazard ratio 6.05, P = 0.005) and MPO-ANCA patients (hazard ratio 1.88, P = 0.0002) compared with ANCA-negative patients. CONCLUSION: PR3-ANCA EGPA patients differ from those with MPO-ANCA and negative ANCA, and share clinical features with granulomatosis with polyangiitis. This suggests that PR3-ANCA EGPA could be a particular form of PR3-ANCA-associated vasculitis.
OBJECTIVES: Only a third of patients with eosinophilic granulomatosis with polyangiitis (EGPA) are ANCA-positive, mainly directed against MPO. ANCA directed against PR3 are rarely found in EGPA. We aimed to examine the significance of PR3-ANCA in EGPA. METHODS: We set up a retrospective European multicentre cohort including 845 patients. Baseline characteristics and outcomes were analysed and compared according to ANCA status. RESULTS: ANCA status was available for 734 patients: 508 (69.2%) ANCA-negative, 210 (28.6%) MPO-ANCA and 16 (2.2%) PR3-ANCA. At baseline, PR3-ANCA patients, compared with those with MPO-ANCA and ANCA-negative, less frequently had active asthma (69% vs 91% and 93%, P = 0.003, respectively) and peripheral neuropathy (31% vs 71% and 47%, P < 0.0001), more frequently had cutaneous manifestations (63% vs 38% and 34%, P = 0.03) and pulmonary nodules (25% vs 10% and 8%, P = 0.046), and lower median eosinophil count (1450 vs 5400 and 3224/mm3, P < 0.0001). Vasculitis relapse-free survival was shorter for PR3-ANCA (hazard ratio 6.05, P = 0.005) and MPO-ANCA patients (hazard ratio 1.88, P = 0.0002) compared with ANCA-negative patients. CONCLUSION:PR3-ANCA EGPA patients differ from those with MPO-ANCA and negative ANCA, and share clinical features with granulomatosis with polyangiitis. This suggests that PR3-ANCA EGPA could be a particular form of PR3-ANCA-associated vasculitis.
Authors: Allyson C Egan; Andreas Kronbichler; Irmgard Neumann; Alessandra Bettiol; Nicholas Carlson; Maria C Cid; Giacomo Emmi; Seerapani Gopaluni; Lorraine Harper; Thomas Hauser; Mark A Little; Raashid A Luqmani; Alfred Mahr; Mark McClure; Aladdin J Mohammad; Karl Emil Nelveg-Kristensen; Sophie Ohlsson; Chen Au Peh; Matthew Rutherford; Beatriz Sanchez Alamo; Jennifer Scott; Mårten Segelmark; Rona M Smith; Wladimir M Szpirt; Gunnar Tomasson; Giorgio Trivioli; Augusto Vaglio; Michael Walsh; Maria Wester Trejo; Kerstin Westman; Ingeborg M Bajema; David R W Jayne Journal: Kidney Int Rep Date: 2022-05-25
Authors: Edoardo Conticini; Miriana d'Alessandro; Laura Bergantini; Diego Castillo; Paolo Cameli; Bruno Frediani; Luca Cantarini; Elena Bargagli Journal: Biology (Basel) Date: 2022-01-08
Authors: Alessandra Bettiol; Maria Letizia Urban; Lorenzo Dagna; Vincent Cottin; Franco Franceschini; Stefano Del Giacco; Franco Schiavon; Thomas Neumann; Giuseppe Lopalco; Pavel Novikov; Chiara Baldini; Carlo Lombardi; Alvise Berti; Federico Alberici; Marco Folci; Simone Negrini; Renato Alberto Sinico; Luca Quartuccio; Claudio Lunardi; Paola Parronchi; Frank Moosig; Georgina Espígol-Frigolé; Jan Schroeder; Anna Luise Kernder; Sara Monti; Ettore Silvagni; Claudia Crimi; Francesco Cinetto; Paolo Fraticelli; Dario Roccatello; Angelo Vacca; Aladdin J Mohammad; Bernhard Hellmich; Maxime Samson; Elena Bargagli; Jan Willem Cohen Tervaert; Camillo Ribi; Davide Fiori; Federica Bello; Filippo Fagni; Luca Moroni; Giuseppe Alvise Ramirez; Mouhamad Nasser; Chiara Marvisi; Paola Toniati; Davide Firinu; Roberto Padoan; Allyson Egan; Benjamin Seeliger; Florenzo Iannone; Carlo Salvarani; David Jayne; Domenico Prisco; Augusto Vaglio; Giacomo Emmi Journal: Arthritis Rheumatol Date: 2021-12-30 Impact factor: 15.483