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146 Clinical and immunological response of childhood-onset systemic lupus erythematosus patients treated with rituximab
  1. Alina Boteanu1,
  2. Maria Angeles Blazquez Cañamero2,
  3. Sandra Garrote2 and
  4. Mariluz Gamir2
  1. 1Hospital Ramón y Cajal
  2. 2University Hospital Ramón y Cajal, Madrid


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 does not respond to conventional therapy.

Methods The aim of this study is to determine the clinical and immunological response in 10 patients with cSLE that received treatment with rituximab in a third level hospital. 10 patients treated with Rituximab between November 2007 and October 2018 was included in a retrospective observational study. The response to treatment at 6 months and one year after the first infusion of Rituximab were assessed. All patients fulfilled four or more of the 1982 revised American College of Rheumatology criteria for the diagnosis of SLE (<16 years).

Results Ten cSLE patients treated with rituximab were included, all of them were female. The age at diagnosis of SLE was a mean of 15,61 years. The mean time duration of disease was 87,55 months (5255 m). 8 patients were caucasians. Rituximab was indicated in 60% of patients for class IV of lupus nephritis (LN), in 10% for class III LN, 10% for Class II LN, 10% for severe cutaneous lupus, and for severe hematological manifestations in 10% of cases (haemolytic anemia). In addition, 60% of the patients had mucocutaneous and articular manifestations. The disease activity of all patients was assessed using SELENA-SLEDAI index pre rituximab infusion, the mean was 17,31 (833). All patients had low level of complement and 90% increased anti-DNA. In 90% of cases the Rituximab was used as a rescue treatment and in a single case as a first line.

90% of patients with renal involvement were previously treated with CF iv and/or mycophenolate. In case of cutaneous involvement the previous treatment was methotrexate, azathioprine (AZA) and Dapsone and in case of hemolytic anemia the treatments was AZA.

The treatment protocol was 1 gram ×2 ×6 m in 8/10 patients, 375 mg/m2×4 in 1/10 cases and 600 mg monthly for 5 m in the case of HAI. Five patients received more than 1 cycle. After the administration of Rituximab, the SELENA-SLEDAI activity index was 5,1. At 6 months a complete response was obtained in the case of hematological and cutaneous manifestations, in 3 cases of LN (proteinuria <0.5 g/day) and partial response was obtained in 2 cases. Data were not analysed 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 10% (1/10 patients: 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 considered in the future as an effective alternative treatment at first line treatment.

Funding Source(s): None

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