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

Rheumatology (Oxford). 2016 Jan;55(1):71-9. doi: 10.1093/rheumatology/kev286. Epub 2015 Aug 20.


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.

Keywords: ANCA; MPA; interstitial lung disease; pulmonary fibrosis; vasculitis.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Disease Progression
  • Female
  • Follow-Up Studies
  • Germany / epidemiology
  • Glucocorticoids / therapeutic use
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Incidence
  • Male
  • Microscopic Polyangiitis / diagnosis*
  • Microscopic Polyangiitis / drug therapy
  • Microscopic Polyangiitis / epidemiology
  • Middle Aged
  • Remission Induction / methods
  • Retrospective Studies
  • Survival Rate / trends
  • Time Factors
  • Young Adult


  • Glucocorticoids
  • Immunosuppressive Agents