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Overall survival, renal survival and relapse in patients with microscopic polyangiitis: a systematic review of
Luis Corral-Gudino1, María Borao-Cengotita-Bengoa, Javier Del Pino-Montes
1Department of Internal Medicine, Hospital Universitaro de Salamanca, Hospital Los Montalvos s/n, E-37192 Salamanca, Spain. lcorral@saludcastillayleon.es
Objective:
There are limited data on the long-term prognosis of microscopic polyangiitis (MPA). A systematic review was performed to estimate the survival, renal survival and relapse rates in patients with MPA.
Methods:
Articles included in MEDLINE and EMBASE databases were reviewed. Randomized or non-randomized trials, cohort, case-control and cases-series studies of patients with MPA diagnosed according to Chapel Hill Consensus Conference definitions, a high rate of biopsy-confirmed diagnosis, follow-up >1 year and follow-up losses <10%. Two independent authors using a predefined questionnaire for evaluating the quality and risk of bias for each study extracted data.
Results:
Eighteen studies for MPA prognosis (n = 940) and six for MPA outcomes after transplantation (n = 65) were included. Survival rates were 77-100% at 1 year, 46-80% at 5 years and 60-80% at 10 years. Higher mortality density occurred within the first months after diagnosis. Vasculitis was the cause of death in 32-50% of patients. Relapses were detected in 19-39% of cases (median time to relapse 15-43 months). Renal graft survival was 85-94% at 1 year and 51-87% at 5 years. Age, renal involvement and immunosuppressive treatment were related to mortality. Lower relapse rate was achieved with 12 vs 6 CYC pulses.
Conclusion:
Evidence regarding MPA prognosis is weak. MPA mortality is mainly concentrated in the first months after diagnosis. Fewer than 50% of deaths are related to MPA activity. MPA long-term prognosis is less severe, although relapses are frequent. End-stage renal failure is a frequent complication of MPA, and renal transplantation could be an effective therapy in these patients. Early diagnosis, early initiation of a tailored therapy according to risk factors and a longer follow-up of the patients are needed.
Insights
Microscopic polyangiitis (MPA) has a less severe long-term prognosis than previously thought, though relapses are common. Early diagnosis and tailored therapy are crucial for managing this condition.
Area of Science:
- Rheumatology
- Nephrology
- Internal Medicine
Background:
- Limited data exist on the long-term prognosis of microscopic polyangiitis (MPA).
- Understanding survival, renal survival, and relapse rates is crucial for patient management.
- MPA is a severe autoimmune disease affecting small blood vessels.
Purpose of the Study:
- To systematically review and estimate the long-term survival, renal survival, and relapse rates in patients with microscopic polyangiitis (MPA).
- To identify factors influencing mortality and relapse in MPA patients.
- To evaluate MPA outcomes after renal transplantation.
Main Methods:
- Systematic review of MEDLINE and EMBASE databases.
- Inclusion of randomized trials, cohort, case-control, and case-series studies with >1 year follow-up.
- Data extraction by two independent authors using a predefined quality assessment questionnaire.
Main Results:
- Survival rates ranged from 46-80% at 5 years and 60-80% at 10 years.
- Relapse rates were 19-39%, with a median time to relapse of 15-43 months.
- Renal graft survival was 51-87% at 5 years post-transplantation.
Conclusions:
- MPA prognosis is less severe long-term, with mortality concentrated early after diagnosis.
- Relapses are frequent, and end-stage renal failure is a common complication.
- Early diagnosis, tailored therapy, and extended follow-up are essential for improving MPA outcomes.
