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.
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.
Authors: David R W Jayne; Annette N Bruchfeld; Lorraine Harper; Matthias Schaier; Michael C Venning; Patrick Hamilton; Volker Burst; Franziska Grundmann; Michel Jadoul; István Szombati; Vladimír Tesař; Mårten Segelmark; Antonia Potarca; Thomas J Schall; Pirow Bekker Journal: J Am Soc Nephrol Date: 2017-04-11 Impact factor: 10.121
Authors: Jan Henrik Schirmer; Peer M Aries; Kirsten de Groot; Bernhard Hellmich; Julia U Holle; Christian Kneitz; Ina Kötter; Peter Lamprecht; Ulf Müller-Ladner; Eva Reinhold-Keller; Christof Specker; Michael Zänker; Frank Moosig Journal: Z Rheumatol Date: 2017-11 Impact factor: 1.372
Authors: Javier Villacorta; Francisco Diaz-Crespo; Mercedes Acevedo; Teresa Cavero; Carmen Guerrero; Manuel Praga; Gema Fernandez-Juarez Journal: Rheumatol Int Date: 2017-03-13 Impact factor: 2.631
Authors: Joshua D Ooi; Janet Chang; Michael J Hickey; Dorin-Bogdan Borza; Lars Fugger; Stephen R Holdsworth; A Richard Kitching Journal: Proc Natl Acad Sci U S A Date: 2012-09-05 Impact factor: 11.205