title: A vasculitis centre based management strategy leads to improved outcome in eosinophilic granulomatosis and polyangiitis (Churg-Strauss, EGPA): monocentric experiences in 150 patients.
abstract: OBJECTIVE
  To evaluate a vasculitis centre based management strategy for eosinophilic granulomatosis and polyangiitis (Churg-Strauss, EGPA).
  
  
  METHODS
  A retrospective cohort study at a vasculitis referral centre was performed. All EGPA patients admitted from 1990 to 2009 were included. A structured interdisciplinary work-up for proof of diagnosis, Disease Extent Index and Birmingham Vasculitis Activity Score was performed. Immunosuppressive therapy was initiated and regularly adapted. Treatment targets were induction and maintenance of remission according to definitions given by the European League Against Rheumatism and the European Vasculitis Study Group. Outcomes were mortality, rate of remission, relapses, adverse events and prednisolone-dose.
  
  
  RESULTS
  Out of 269 patients with suspected EGPA 150 fulfilled the inclusion criteria. Of those, 104 had more than one follow-up visit resulting in a mean follow up of 53±4.9 months. By using additional data sources the follow-up concerning survival was extended to 92±5 month. Severe organ manifestations occurred at heart (46%), kidney (18%) and lungs (10%). Cyclophosphamide was used in 107 patients (71%). The prednisolone-doses of all patients were within the targeted range (i.e. ≤7.5 mg) in 69% of the total follow-up time; the median dose at end of follow-up was 5mg/d. The 10-year survival rate was 89% resulting in mortality comparable to the general population (SMR 1.29). Only patients with cardiac failure associated with EGPA had an increased mortality (SMR 3.06).
  
  
  CONCLUSIONS
  Regular re-evaluation and target-orientated adaption of therapy may lead to normalization of life expectancy and attenuation of disease progression. Continued centre based interdisciplinary treatment should be standard of care.
authors.affiliation: Department of Rheumatology, University Hospital Schleswig-Holstein and Klinikum Bad Bramstedt, Bad Bramstedt, Germany. f.moosig@klinikumbb.de
authors.forename: Frank
authors.initials: F
authors.lastname: Moosig
authors.forename: Jan Phillip
authors.initials: JP
authors.lastname: Bremer
authors.forename: Bernhard
authors.initials: B
authors.lastname: Hellmich
authors.forename: Julia Ulrike
authors.initials: JU
authors.lastname: Holle
authors.forename: Konstanze
authors.initials: K
authors.lastname: Holl-Ulrich
authors.forename: Martin
authors.initials: M
authors.lastname: Laudien
authors.forename: Christine
authors.initials: C
authors.lastname: Matthis
authors.forename: Claudia
authors.initials: C
authors.lastname: Metzler
authors.forename: Bernhard
authors.initials: B
authors.lastname: Nölle
authors.forename: Gert
authors.initials: G
authors.lastname: Richardt
authors.forename: Wolfgang L
authors.initials: WL
authors.lastname: Gross
mesh_terms: D000893:Anti-Inflammatory Agents / Q000627:therapeutic use*; D001379:Azathioprine / Q000627:therapeutic use; D015267:Churg-Strauss Syndrome / Q000188:drug therapy* / Q000401:mortality / Q000503:physiopathology; D015331:Cohort Studies; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007165:Immunosuppression Therapy / Q000379:methods; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007555:Isoxazoles / Q000627:therapeutic use; D000077339:Leflunomide; D008297:Male; D008727:Methotrexate / Q000627:therapeutic use; D008875:Middle Aged; D011239:Prednisolone / Q000627:therapeutic use*; D012074:Remission Induction / Q000379:methods; D012189:Retrospective Studies; D055502:Secondary Prevention; D016896:Treatment Outcome
chemical_list: D000893:Anti-Inflammatory Agents; D007166:Immunosuppressive Agents; D007555:Isoxazoles; D003520:Cyclophosphamide; D011239:Prednisolone; D000077339:Leflunomide; D001379:Azathioprine; D008727:Methotrexate