Yann Nguyen1, Christian Pagnoux2, Alexandre Karras3, Thomas Quéméneur4, François Maurier5, Mohamed Hamidou6, Alain Le Quellec7, Noémie Jourde Chiche8, Pascal Cohen1, Alexis Régent1, François Lifermann9, Arsène Mékinian10, Chahéra Khouatra11, Eric Hachulla12, Jacques Pourrat13, Marc Ruivard14, Pascal Godmer15, Jean-François Viallard16, Benjamin Terrier1, Luc Mouthon1, Loïc Guillevin1, Xavier Puéchal17. 1. Department of Internal Medicine, National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, APHP, Université Paris Descartes, Paris, France. 2. Division of Rheumatology, Mount Sinai Hospital, Toronto, Canada. 3. Department of Nephrology, Hôpital Européen Georges Pompidou, APHP, Paris, France. 4. Department of Internal Medicine, CH, Valenciennes, France. 5. Department of Internal Medicine, Hôpitaux Privés, Metz, France. 6. Department of Internal Medicine, CHU, Nantes, France. 7. Department of Internal Medicine, Hôpital Saint-Eloi, CHU, Montpellier, France. 8. Department of Nephrology, AP-HM, CHU Conception, Marseille, France. 9. Department of Internal Medicine, CH, Dax, France. 10. Department of Internal Medicine, Hôpital Saint-Antoine, APHP, Paris, France. 11. Department of Respiratory Medicine, National Referral Center for Rare Pulmonary Diseases, Hôpital Louis-Pradel, CHU Lyon, France. 12. Department of Internal Medicine, National Referral Center for Systemic Sclerosis, CHRU Claude Huriez, Lille, France. 13. Department of Nephrology, CHU Rangueil, Toulouse, France. 14. Department of Internal Medicine, CHU Estaing, Clermont-Ferrand, France. 15. Department of Internal Medicine, CH Vannes, France. 16. Department of Internal Medicine, Hôpital Haut-Lévêque, CHU Bordeaux, France. 17. Department of Internal Medicine, National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, APHP, Université Paris Descartes, Paris, France. Electronic address: xavier.puechal@aphp.fr.
Abstract
OBJECTIVE: To describe characteristics and long-term outcomes of patients with microscopic polyangiitis (MPA), an antineutrophil cytoplasm antibody (ANCA)-associated small-vessel necrotizing vasculitis. METHODS: MPA patients from the French Vasculitis Study Group Registry satisfying the European Medicines Agency algorithm were analyzed retrospectively. Characteristics at diagnosis, treatments, relapses and deaths were analyzed to identify factors predictive of death or relapse. RESULTS: Between 1966 and 2017, 378 MPA patients (median age 63.7 years) were diagnosed and followed for a mean of 5.5 years. At diagnosis, the main clinical manifestations included renal involvement (74%), arthralgias (45%), skin (41%), lung (40%) and mononeuritis multiplex (32%), with less frequent alveolar hemorrhage (16%), cardiomyopathy (5%) and severe gastrointestinal signs (4%); mean serum creatinine was 217 μmol/L. ANCA were detected in 298/347 (86%) patients by immunofluorescence and/or enzyme-linked immunosorbent assay (ELISA). Among the 293 patients with available ELISA specificities, 272 (92.8%) recognized myeloperoxidase and 13 (4.4%) proteinase-3. During follow-up, 131 (34.7%) patients relapsed and 78 (20.6%) died, mainly from infections. Respective 5-year overall and relapse-free survival rates were 84.2% and 60.4%. Multivariable analyses retained age >65 years, creatinine >130 μmol/L, severe gastrointestinal involvement and mononeuritis multiplex as independent risk factors for death. Renal impairment was associated with a lower risk of relapse. CONCLUSION: Non-renal manifestations and several risk factors for death or relapse were frequent in this nationwide cohort. While mortality was low, and mainly due to treatment-related complications, relapses remained frequent, suggesting that MPA management can be further improved.
OBJECTIVE: To describe characteristics and long-term outcomes of patients with microscopic polyangiitis (MPA), an antineutrophil cytoplasm antibody (ANCA)-associated small-vessel necrotizing vasculitis. METHODS: MPA patients from the French Vasculitis Study Group Registry satisfying the European Medicines Agency algorithm were analyzed retrospectively. Characteristics at diagnosis, treatments, relapses and deaths were analyzed to identify factors predictive of death or relapse. RESULTS: Between 1966 and 2017, 378 MPA patients (median age 63.7 years) were diagnosed and followed for a mean of 5.5 years. At diagnosis, the main clinical manifestations included renal involvement (74%), arthralgias (45%), skin (41%), lung (40%) and mononeuritis multiplex (32%), with less frequent alveolar hemorrhage (16%), cardiomyopathy (5%) and severe gastrointestinal signs (4%); mean serum creatinine was 217 μmol/L. ANCA were detected in 298/347 (86%) patients by immunofluorescence and/or enzyme-linked immunosorbent assay (ELISA). Among the 293 patients with available ELISA specificities, 272 (92.8%) recognized myeloperoxidase and 13 (4.4%) proteinase-3. During follow-up, 131 (34.7%) patients relapsed and 78 (20.6%) died, mainly from infections. Respective 5-year overall and relapse-free survival rates were 84.2% and 60.4%. Multivariable analyses retained age >65 years, creatinine >130 μmol/L, severe gastrointestinal involvement and mononeuritis multiplex as independent risk factors for death. Renal impairment was associated with a lower risk of relapse. CONCLUSION: Non-renal manifestations and several risk factors for death or relapse were frequent in this nationwide cohort. While mortality was low, and mainly due to treatment-related complications, relapses remained frequent, suggesting that MPA management can be further improved.
Authors: Idris Boudhabhay; Florence Delestre; Guillaume Coutance; Viviane Gnemmi; Thomas Quemeneur; Cyrille Vandenbussche; Helene Lazareth; Guillaume Canaud; Leila Tricot; Clément Gosset; Aurélie Hummel; Benjamin Terrier; Marion Rabant; Emma E van Daalen; Maria A C Wester Trejo; Ingeborg M Bajema; Alexandre Karras; Jean-Paul Duong Van Huyen Journal: J Am Soc Nephrol Date: 2021-06-21 Impact factor: 14.978