Background Rituximab (RTX) is a novel, recently approved therapeutic agent for the treatment of active ANCA-associated vasculitis (AAV, Granulomatosis with polyangiitis-GPA, Microscopic polyangiitis-MPA) with limited data available from daily clinical practice.
Objectives To study the efficacy and safety of RTX in patients with AAV in real life settings.
Methods Retrospective study of all patients with active AAV treated with RTX in our tertiary center between 2010 and 2015.
Results 16 patients with active AAV were included (GPA n=12, MPA n=4); 50% females with a mean age of 61.8±13.9 years and median disease duration of 33.5 months. The majority of patients had generalized disease (88%) and the most commonly involved organs were lungs (75%), kidney (63%), joints (50%) and ENT (31%). The patients were followed for a mean period of 21.5±16.1 months. Eleven (68%) patients were treated with RTX due to resistant or relapsing disease and 5 (32%) received RTX as initial treatment. Seven patients received 1 cycle, one patient 2 cycles and 8 patients received ≥3 cycles of RTX (1 gm x 2, 15 days apart). Among patients who received ≥1 cycle of RTX (n=9), 6 received RTX on a regular basis (every 6 months) and 3 at relapse. During treatment, a statistically significant improvement in disease activity was observed (BVAS/WG before RTX was 4.8 and decreased to 0.9 and 0.8 at 6 and 12 months, p=0.001 and 0.003 respectively). At 6 months, 87% of patients (13/15) responded to therapy (6 showed complete and 7 partial response). Additionally, one patient who had not responded at 6 months demonstrated complete response at 9 months. Among the 13 responders, 6 (46%) relapsed during follow up. The majority of relapses were minor (5/6, 83%) and occurred mainly in patients not receiving RTX in a regular basis (4/6, 67%). One patient with GPA and fulminant pulmonary-renal syndrome died from septic shock one month after RTX administration (6%). No other serious infections or allergic reactions were noticed during the follow up period.
Conclusions These real life data confirm the excellent efficacy and safety of RTX in patients with active newly diagnosed or relapsing AAV.
Acknowledgements This work was supported in part by research grants from the Special Account for Research Grants (S.A.R.G.), National and Kapodistrian University of Athens, Athens, Greece.
Disclosure of Interest None declared