title: Predictors at diagnosis of a first Wegener's granulomatosis relapse after obtaining complete remission.
abstract: OBJECTIVE
  Although in most patients with WG, induction therapy leads to complete remission (CR), the high relapse rate remains a major problem. This study was undertaken to identify potential predictors of these relapses.
  
  
  METHODS
  WG outcomes of patients included in two randomized trials were analysed. Patients were categorized into a predominant form of the disease using a scoring system composed of three granulomatous criteria (ear, nose and throat manifestations; lung nodules; and orbital pseudotumour) and three vascular criteria (serum creatinine >125 µmol/l, haematuria or proteinuria and alveolar haemorrhage).
  
  
  RESULTS
  Among 174 patients, 152 (87%) entered CR with CSs and CYC. The risk of death was higher for the vascular form [odds ratio (OR) 3.5 (95% CI 1.1, 11.4)]. With a median follow-up of 50 months, 66 out of 152 patients experienced a relapse (5-year relapse rate: 49%). Multivariate analysis retained the following variables at diagnosis as independent predictors of relapse: specific cardiac involvement [hazard ratio (HR) 2.9 (95% CI 1.3, 6.5)], ANCA with cytoplasmic-labelling pattern (c-ANCA) [HR 2.1 (95% CI 1.1, 4.3)] and higher age [HR 1.4 (95% CI 1.1, 1.7)]. Conversely, severe renal insufficiency was associated with a lower relapse rate [HR 0.4 (95% CI, 0.2-0.8)]. Comparing predominant granulomatous vs predominant vascular disease, relapses were more frequent and earlier with decreasing risk over time.
  
  
  CONCLUSIONS
  Increased risk of first WG relapse after initial remission appears to be related to heart involvement, age and c-ANCA positivity at onset. Predominant granulomatous presentation disease seems to be associated with poorer outcomes after CR. These findings may help adapt treatment strategies.
authors.affiliation: Department of Internal Medicine, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Université Paris Descartes, 27, Paris, France.
authors.forename: Carole
authors.initials: C
authors.lastname: Pierrot-Deseilligny Despujol
authors.forename: Jacques
authors.initials: J
authors.lastname: Pouchot
authors.forename: Christian
authors.initials: C
authors.lastname: Pagnoux
authors.forename: Joël
authors.initials: J
authors.lastname: Coste
authors.forename: Loïc
authors.initials: L
authors.lastname: Guillevin
mesh_terms: D000328:Adult; D000368:Aged; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000188:drug therapy* / Q000401:mortality / Q000503:physiopathology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D016032:Randomized Controlled Trials as Topic; D012008:Recurrence; D012074:Remission Induction; D012307:Risk Factors; D012720:Severity of Illness Index; D013223:Statistics as Topic; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents