Predictors at diagnosis of a first Wegener's granulomatosis relapse after obtaining complete remission

Rheumatology (Oxford). 2010 Nov;49(11):2181-90. doi: 10.1093/rheumatology/keq244. Epub 2010 Jul 31.


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.

MeSH terms

  • Adult
  • Aged
  • Female
  • Granulomatosis with Polyangiitis / drug therapy*
  • Granulomatosis with Polyangiitis / mortality
  • Granulomatosis with Polyangiitis / physiopathology
  • Humans
  • Immunosuppressive Agents / therapeutic use*
  • Male
  • Middle Aged
  • Predictive Value of Tests
  • Randomized Controlled Trials as Topic
  • Recurrence
  • Remission Induction
  • Risk Factors
  • Severity of Illness Index
  • Statistics as Topic
  • Treatment Outcome


  • Immunosuppressive Agents