Rituximab for induction and maintenance therapy in granulomatosis with polyangiitis (Wegener's). Results of a single-center cohort study on 66 patients

The aim of this study was to evaluate the efficacy and safety of rituximab (RTX) associated with glucocorticoid treatment based on disease severity, as a remission induction treatment for granulomatosis with polyangiitis (GPA) (Wegener's) and to analyze the results of long-term maintenance therapy with low doses of RTX in a routine time-based protocol. This single-center retrospective study used standardized data collection from all CPA patients receiving RTX between 2002 and 2013. The remission induction regimen consisted of RTX and gluco-corticoids, adapted according to disease severity. Once remission was achieved, patients received RTX maintenance treatment (500 mg every 6 months) for 18 months. Sixty-six CPA patients received RTX for remission induction. After six months, a response had been achieved in 78.8% of these patients, with a moderate oral prednisone regimen (mean dose at baseline, 32.8 +/- 23.4 mg/day). Subglottic stenosis increased the risk of treatment failure (OR = 312, P = 0.0104). RTX maintenance treatment was continued for 18 months in 92% of the CPA patients, who were followed for 34.2 +/- 26.2 months (mean total cumulative RTX dose of 4.6 +/- 1.7 g). The relapse rate was 11.2/100 patient-years. The relapses occur a mean of 13.5 +/- 14.7 months after the last "RTX infusion. Twenty-one severe adverse events were recorded; 13.6% patients had severe infections. We conclude that in this single-center cohort, RTX associated with glucocorticoid treatment adapted for disease severity appeared to induce remission effectively in CPA patients. Maintenance treatment with low doses of RTX in a routine time-based protocol was safe and associated with low rates of relapse on treatment. (c) 2014 Elsevier Ltd. All rights reserved.