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Published on: August 26, 2017
Long-term follow-up of microscopic polyangiitis, 17-year experience at a single center
María Borao-Cengotita-Bengoa1, Luis Corral-Gudino, Javier Del Pino-Montes
1Servicio de Medicina Interna Virgen de la Vega, Hospital Universitario de Salamanca, Paseo de San Vicente, 58-182, 37007, Salamanca, Spain.
Background:
Long-term prognoses of Wegener granulomatosis (WG) and Churg-Strauss syndrome (CSS) are known; however, few data exist on long-term prognoses for microscopic polyangiitis (MPA). Our aim was to analyse the prognoses of MPA.
Methods:
Cohort study with retrospective selection of patients. Twenty-two patients admitted to our Hospital (1990-2006) with biopsy-proven MPA were studied. The start date for entry into the study was the date of diagnosis. Statistical analysis was performed to look for prognostic factors for survival.
Results:
MPA patients were followed-up for a median of 78 (5-131) months. MPA patients were treated with cyclophosphamide (Cy) plus corticosteroid (Cs) (59%) or Cs alone (41%). Seven MPA patients died. Cumulative MPA patient survival at 1, 5, and 10 years were 85% (75-95%), 85% (75-95%), and 74% (60-88%) in those treated with Cy plus Cs and 50% (32-68%), 36% (14-58%), and 0% (0-30%) in those treated with Cs alone, respectively (P=0.04). Disease extent index <5 (P=0.02) and age <65 years (P=0.02) were associated with improved survival rates in MPA patients treated with Cy. Five MPA (23%) patients relapsed after a median of 54 months (35-93). No variables were related to relapses. Despite treatment, 11MPA (50%) patients developed end-stage renal disease after a median of 9 months (0-53).
Conclusions:
Most MPA patients had life-threatening renal or lung involvement at diagnosis. Patients not treated with immunosuppressants had a poorer prognosis. The long-term prognosis of MPA patients who survived 6 months post diagnosis was good, although renal survival rates are low.
Insights
Microscopic polyangiitis (MPA) patients treated with cyclophosphamide and corticosteroids showed improved survival compared to those treated with corticosteroids alone. Early diagnosis and treatment are crucial for better outcomes in MPA patients, though renal survival remains a concern.
Area of Science:
- Rheumatology
- Nephrology
- Immunology
Background:
- Long-term prognoses for Wegener granulomatosis and Churg-Strauss syndrome are established, but data on microscopic polyangiitis (MPA) are limited.
- This study aimed to analyze the long-term prognoses of microscopic polyangiitis (MPA).
Purpose of the Study:
- To investigate the long-term prognosis of microscopic polyangiitis (MPA).
- To identify prognostic factors influencing survival in MPA patients.
Main Methods:
- A retrospective cohort study of 22 biopsy-proven MPA patients diagnosed between 1990 and 2006.
- Patients were followed up for a median of 78 months, with statistical analysis to determine prognostic factors for survival.
Main Results:
- MPA patients treated with cyclophosphamide plus corticosteroids (Cy+Cs) had significantly better 1, 5, and 10-year survival rates (85%, 85%, 74%) compared to those treated with corticosteroids alone (50%, 36%, 0%; P=0.04).
- Disease extent index <5 and age <65 were associated with improved survival in MPA patients treated with Cy.
- Despite treatment, 50% of MPA patients developed end-stage renal disease, and 23% experienced relapses.
Conclusions:
- Microscopic polyangiitis often presents with severe renal or lung involvement.
- Immunosuppressive treatment, particularly cyclophosphamide combined with corticosteroids, significantly improves survival in MPA patients.
- While long-term survival is possible for MPA patients who survive the initial 6 months, renal survival rates remain low.
