title: Long-term outcome of patients with antineutrophil cytoplasmic autoantibody-associated vasculitis with renal involvement.
abstract: BACKGROUND
  Despite treatment, renal involvement in antineutrophil cytoplasmic autoantibody (ANCA)-positive vasculitis is still associated with significant long-term mortality and remains an important cause of end-stage renal failure.
  
  
  METHODS
  We retrospectively analyzed a series of 61 consecutive patients with newly diagnosed ANCA-associated renal vasculitis (54.1% Wegener's granulomatosis, 23% renal-limited vasculitis, 16.4% microscopic polyangiitis, 4.9% Churg-Strauss syndrome) diagnosed between 1986 and 1997.
  
  
  RESULTS
  The median creatinine level at diagnosis was 221.5 (63-762) micromol/l, i.e. 2.5 (0.7-8.6) mg/dl, 32.8% were dialysis-dependent. All patients were treated with cyclophosphamide. Remission was achieved in 87% of patients. Relapses occurred in 44.7%. The median renal disease-free interval was 62.5 (0-138) months. The estimated patient survival at 5 and 10 years was 78.3 and 62.2%, respectively. Mortality was associated with age (p = 0.04 when age limit 50 years) and advanced renal failure (p = 0.038 when compared dialysis-dependent and independent patients). Estimated renal survival time at 5 and 10 years was 69.2 and 55.8%, respectively. At the end of follow-up, 50.8% of patients were in complete remission, 31% had died. The median serum creatinine level was 137.5 (77-469) micromol/l, i.e. 1.56 (0.87-5.3) mg/dl, 24.6% of patients were on regular dialysis treatment.
  
  
  CONCLUSIONS
  Patient survival, relapse rate and mortality were comparable to similar reports. In view of the severity of the renal disease and the length of follow-up, renal survival was very good. Despite effective treatment, the long-term outcome of patients with ANCA-associated renal vasculitis remains unsatisfactory.
authors.affiliation: Nephrology Unit, 1st Medical Faculty, Charles University, Prague, Czech Republic. zrihova@centrum.cz
authors.forename: Zuzana
authors.initials: Z
authors.lastname: Rihova
authors.forename: Eva
authors.initials: E
authors.lastname: Jancova
authors.forename: Miroslav
authors.initials: M
authors.lastname: Merta
authors.forename: Romana
authors.initials: R
authors.lastname: Rysava
authors.forename: Jana
authors.initials: J
authors.lastname: Reiterova
authors.forename: Jiri
authors.initials: J
authors.lastname: Zabka
authors.forename: Vladimir
authors.initials: V
authors.lastname: Tesar
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D019268:Antibodies, Antineutrophil Cytoplasmic; D003520:Cyclophosphamide / Q000627:therapeutic use; D018572:Disease-Free Survival; D004341:Drug Evaluation; D006801:Humans; D007674:Kidney Diseases / Q000188:drug therapy* / Q000209:etiology / Q000401:mortality; D007676:Kidney Failure, Chronic / Q000209:etiology; D008875:Middle Aged; D012008:Recurrence; D012074:Remission Induction; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome; D014657:Vasculitis / Q000188:drug therapy* / Q000276:immunology / Q000401:mortality*
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D003520:Cyclophosphamide