Background Systemic lupus erythematosus (SLE) is an autoimmune disease that is more severe in pediatric population than in adults. Biological therapy with anti-CD20 (rituximab) is an option in patient that do not respond to conventional therapy.
Objectives The aim of this study is to determine the clinical and immunological response in 9 patients with childhood-onset systemic lupus erythematosus (cSLE) that received treatment with rituximab in a third level hospital
Methods This is a retrospective observational study. 9 patients treated with Rituximab between November 2007 and October 2016 were included and their medical records were reviewed. The response to treatment at 6 months and one year after the first infusion of Rituximab were assessed. Patients with overlap syndromes were excluded. All patients fulfilled four or more of the 1982 revised American College of Rheumatology criteria for the diagnosis of SLE (<16 years).
Results Nine pediatric patients with SLE treated with rituximab were included, all of them were female. The age at diagnosis of SLE was a mean of 15,22 years. The mean time duration of disease was 87,55months (5–255m). 7 patients were caucasians. Rituximab was indicated in 6 patients with class IV of lupus nephritis (LN) 1/9 with class III LN, 1/9 with severe cutaneous lupus, and with severe hematological manifestations in 1 case (haemolytic anemia). In addition, 6/9 patients had mucocutaneous and articular manifestations. The disease activity of all patients was assessed using SELENA-SLEDAI index pre rituximab infusion, the mean was
17,11 (8–33). All patients had low level of complement C3 and C4 and 8/9 increased anti-DNA. In 8/9 patients Rituximab was used as a rescue treatment and in a single case as a first line.
3/6 patients with renal involvement were previously treated with cyclophosphamide (CF) iv and mycophenolate, 2/6 CF. In case of cutaneous involvement the previous treatment was methotrexate, azathioprine (AZA) and dapsone and in case of hemolytic anemia was AZA.
The treatment protocol was 1 gram x 2 (1 cycle) in 7/9 patients, 375mg/m2 x 4 in 1/9 cases and 600mg monthly for 5 months in the case of hemolytic anemia. Five patients received more than 1 cycle. After the administration of Rituximab, the SELENA-SLEDAI activity index was 4.5 points. At 6 months a complete response was obtained in the case of hematological and cutaneous manifestations, in 2 cases of lupus nephritis (proteinuria <0.5 g/day) and partial response was obtained in 2 cases. Data were not analyzed in 2 patients (death and less than 6 months of the first dose of rituximab). Patients with partial response and lack of response achieved complete response at 12 months. 2/9 patients had side effects (Rituximab pneumonitis in 1 case and infections in 2 cases). Mortality was 11.11% (1/9 patients, per infection and lupus activity, SLEDAI pre rituximab =33)
Conclusions In our study, although it consisted of few patients, it was objected that Rituximab therapy in patients with cSLE is effective, reduces lupus activity index, especially in cases of renal, cutaneous and hematologic involvement, that don't respond to conventional therapy. It may be consider in the future as an effective alternative treatment at first line treatment.
Disclosure of Interest None declared