Clinical presentation and long-term outcome of 144 patients with microscopic polyangiitis in a monocentric German cohort

Jan H. Schirmer*, Marvin N. Wright, Reinhard Vonthein, Kristine Herrmann, Bernhard Nölle, Marcus Both, Frank O. Henes, Andreas Arlt, Wolfgang L. Gross, Susanne Schinke, Eva Reinhold-Keller, Frank Moosig, Julia U. Holle

*Corresponding author for this work
29 Citations (Scopus)


Objective. To evaluate the clinical presentation and long-term outcome of a vasculitis centre cohort of patients with microscopic polyangiitis (MPA) with respect to organ manifestations, treatment, chronic damage and mortality. Methods. We performed a retrospective chart review at our vasculitis referral centre. MPA patients admitted between 1991 and 2013 classified by a modified European Medicines Agency algorithm were diagnosed and treated according to a standardized interdisciplinary approach. Results. Comprehensive data from standardized interdisciplinary workups was available for 144 patients (median follow-up 72 months). The overall standardized mortality ratio was 1.40 (95% CI 0.91, 2.07; P = 0.13). We observed a higher mortality [hazard ratio (HR) 4.04 (95% CI 1.21, 13.45), P = 0.02] in 17 patients with MPA-associated fibrosing interstitial lung disease (ILD) and 56 patients with peripheral nervous system involvement [HR 5.26 (95% CI 1.10, 25.14), P = 0.04] at disease onset. One hundred and fifteen patients (79.9%) responded to the initial treatment. Sixty-one (42.3%) achieved complete remission and 54 (37.5%) achieved partial remission. Twenty (13.9%) showed a refractory disease course. Conclusion. MPA patients at our tertiary rheumatology referral centre seemed to have a less severe phenotype resulting in a less severe disease course and better outcome than reported in other cohorts. Fibrosing ILD was significantly associated with mortality in this cohort.

Original languageEnglish
Article numberkev286
JournalRheumatology (United Kingdom)
Issue number1
Pages (from-to)71-79
Number of pages9
Publication statusPublished - 01.01.2016

Research Areas and Centers

  • Academic Focus: Center for Infection and Inflammation Research (ZIEL)


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