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Microscopic polyangiitis: Clinical characteristics and long-term outcomes of 378 patients from the French Vasculitis
Yann Nguyen1, Christian Pagnoux2, Alexandre Karras3
1Department of Internal Medicine, National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, APHP, Université Paris Descartes, Paris, France.
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.
Insights
Microscopic polyangiitis (MPA) patients often present with non-renal symptoms and risk factors for death. While mortality is low, relapses are common, indicating a need for improved MPA management strategies.
Area of Science:
- Rheumatology
- Nephrology
- Immunology
Background:
- Microscopic polyangiitis (MPA) is a type of antineutrophil cytoplasm antibody (ANCA)-associated small-vessel necrotizing vasculitis.
- Understanding MPA patient characteristics and long-term outcomes is crucial for effective disease management.
Purpose of the Study:
- To describe the clinical characteristics and long-term outcomes of patients diagnosed with microscopic polyangiitis (MPA).
- To identify factors that predict mortality and relapse in MPA patients.
Main Methods:
- Retrospective analysis of 378 MPA patients from the French Vasculitis Study Group Registry (1966-2017).
- Data collected included diagnosis characteristics, treatments, relapses, and mortality.
- Multivariable analyses were used to identify independent risk factors for death and relapse.
Main Results:
- The median age of MPA patients was 63.7 years, with common manifestations including renal involvement (74%), arthralgias (45%), and skin (41%) or lung (40%) involvement.
- ANCA, predominantly against myeloperoxidase (92.8%), were detected in 86% of patients.
- During a mean follow-up of 5.5 years, 34.7% of patients relapsed and 20.6% died, primarily from infections. Independent risk factors for death included age >65, creatinine >130 μmol/L, severe gastrointestinal involvement, and mononeuritis multiplex. Renal impairment was linked to a lower relapse risk.
Conclusions:
- Non-renal manifestations and risk factors for adverse outcomes are prevalent in MPA.
- Despite low overall mortality, frequent relapses highlight the need for optimized MPA treatment strategies to improve patient outcomes.