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

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 mu mol/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.