title: Clinical presentation and long-term outcome of 144 patients with microscopic polyangiitis in a monocentric German cohort.
abstract: 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.
  
  
  CONCLUSIONS
  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.
authors: Department of Rheumatology, University Medical Center Schleswig-Holstein and Klinikum Bad Bramstedt, Bad Bramstedt, j.schirmer@klinikumbb.de.
authors: Jan H
authors: JH
authors: Schirmer
authors: Department of Medical Biometry and Statistics, University of Lübeck, University Medical Center Schleswig-Holstein, Campus Lübeck.
authors: Marvin N
authors: MN
authors: Wright
authors: Department of Medical Biometry and Statistics, University of Lübeck, University Medical Center Schleswig-Holstein, Campus Lübeck, Center for Clinical Trials Lübeck, University of Lübeck, Lübeck.
authors: Reinhard
authors: R
authors: Vonthein
authors: Department of Rheumatology, University Medical Center Schleswig-Holstein and Klinikum Bad Bramstedt, Bad Bramstedt.
authors: Kristine
authors: K
authors: Herrmann
authors: Department of Ophthalmology.
authors: Bernhard
authors: B
authors: Nölle
authors: Department of Radiology and Neuroradiology, University Medical Center Schleswig-Holstein, Campus Kiel, Kiel.
authors: Marcus
authors: M
authors: Both
authors: Clinic and Policlinic for Diagnostic and Interventional Radiology, University Medical Center Hamburg-Eppendorf, Hamburg.
authors: Frank O
authors: FO
authors: Henes
authors: Department of Medical and Neurological Rehabilitation, Klinikum Bad Bramstedt, Bad Bramstedt and.
authors: Andreas
authors: A
authors: Arlt
authors: University Medical Center Schleswig-Holstein, Campus Lübeck, Lübeck, Germany.
authors: Wolfgang L
authors: WL
authors: Gross
authors: Department of Rheumatology, University Medical Center Schleswig-Holstein and Klinikum Bad Bramstedt, Bad Bramstedt.
authors: Susanne
authors: S
authors: Schinke
authors: Department of Rheumatology, University Medical Center Schleswig-Holstein and Klinikum Bad Bramstedt, Bad Bramstedt.
authors: Eva
authors: E
authors: Reinhold-Keller
authors: Department of Rheumatology, University Medical Center Schleswig-Holstein and Klinikum Bad Bramstedt, Bad Bramstedt.
authors: Frank
authors: F
authors: Moosig
authors: Department of Rheumatology, University Medical Center Schleswig-Holstein and Klinikum Bad Bramstedt, Bad Bramstedt.
authors: Julia U
authors: JU
authors: Holle
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D005858:Germany / Q000453:epidemiology; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D015994:Incidence; D008297:Male; D055953:Microscopic Polyangiitis / Q000175:diagnosis* / Q000188:drug therapy / Q000453:epidemiology; D008875:Middle Aged; D012074:Remission Induction / Q000379:methods; D012189:Retrospective Studies; D015996:Survival Rate / Q000639:trends; D013997:Time Factors; D055815:Young Adult
chemical_list: D005938:Glucocorticoids; D007166:Immunosuppressive Agents
keywords: ANCA; MPA; interstitial lung disease; pulmonary fibrosis; vasculitis