Background There are only few reports of rituximab (RTX) effects in children with autoimmune diseases refractory to conventional therapy protocols.
Objectives Authors present their work on the effects of RTX therapy in chidren with systemic lupus erythematosus (SLE) + lupus nephritis (LN) and ANCA positive vasulitis, refractory to standard therapy.
Methods Retrospective chart rewiev of all patients treated with RTX at the Departement of Paediatrics, University of Zagreb School of Medicine, University Hospital Centre Zagreb, during the period 2009–2014.
Results Nine children were treated with RTX: 6 with SLE + LN (3 boys, 3 girls) and 3 with ANCA positive vasulitis (3 girls). Median age of patients at the disease onset was 11.6 years (5 – 15 years). Conventional therapy included methylprednisolon, cyclophosphamide (CYC), mycophenolate mofetil, azathyoprine, in some cases plasmapheresis. In all cases RTX was introduced due to ineffectivness of conventional therapy in achiving the remission of autoimune disease. Median time between the beginnig of conventional therapy and introduction of RTX was 10.3 months (1 – 48 months). In 8 cases RTX (750 mg/m2, two doses, two weeks apart) was combined with mini pulses of CYC (350 mg/m2), in 1 case RTX was applied in dose of 375 mg/m2 per week, for 4 weeks. After RTX introduction complete, prolonged remission was achived in 6 children. In 2 patients relapses of disease required repeating of RTX therapy. One patient (girl (5 yrs) with ANCA possitive vasculitis) died due to initially severe presentation at disease onset and fulminant, progressive multiorgan failure despite all conventional therapy and early introduction of RTX.
Conclusions Complete or partial remission after RTX introduction was achived in 8/9 (88,8%) of our patients with severe autoimmune diseases. To our expirience, RTX represents powerful tool in controlling SLE + LN and ANCA positive vasculitis refractory to conventional therapy protocols.
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Disclosure of Interest None declared