Rituximab therapy has a rapid and durable response for refractory cytopenia in childhood-onset systemic lupus erythematosus

Abstract

Rituximab appears to be a well-tolerated, safe and long-lasting therapy for cSLE patients with refractory AITP and/or AIHA. Caution should be exercised when considering rituximab for patients with preexisting hypogammaglobulinemia.

Tregopathies: Monogenic diseases resulting in regulatory T-cell deficiency

“…97 Indeed, in children with systemic lupus erythematosus (SLE)-associated cytopenia treated with rituximab, 3 of 4 patients with pre-existing low IgG levels developed persistent hypogammaglobulinemia after rituximab that required immunoglobulin replacement. 183 The efficacy of rituximab to control cytopenia in patients with Tregopathies is currently difficult to establish, first because of the small number of patients in some of these diseases and second because other therapies frequently coadministered in patients with Tregopathies (steroids, rapamycin, or calcineurin inhibitors) can also contribute to the beneficial effect of rituximab. 184,185 The abovementioned study on pediatric SLE demonstrated complete response in 23 of 24 children with cytopenia treated with rituximab.…”

“…184,185 The abovementioned study on pediatric SLE demonstrated complete response in 23 of 24 children with cytopenia treated with rituximab. 183 In an adult SLE study, of 71 patients treated with rituximab for cytopenia, approximately 60% displayed a complete response, and relapses were successfully treated in most with a second round of rituximab therapy. 186 Similarly, in patients with combined variable immunodeficiency-associated cytopenia, the complete response rate was 74%, and patients who relapsed also mostly responded to rituximab readministration.…”

Benefits of rituximab as a second‐line treatment for autoimmune haemolytic anaemia in children: a prospective French cohort study

“…This problem was described early on in adult patients receiving rituximab for rheumatoid arthritis and SLE (Edwards et al, 2006) or ITP (Levy et al, 2014) and more recently in 3 SLE children (Olfat et al, 2015) and in some children with auto-immune lymphoproliferative syndrome (Cooper et al, 2009;Teachey, 2012) treated with rituximab for ITP or AIHA. This problem was described early on in adult patients receiving rituximab for rheumatoid arthritis and SLE (Edwards et al, 2006) or ITP (Levy et al, 2014) and more recently in 3 SLE children (Olfat et al, 2015) and in some children with auto-immune lymphoproliferative syndrome (Cooper et al, 2009;Teachey, 2012) treated with rituximab for ITP or AIHA.…”

“…Finally, 4/50 (8%) cases of persistent hypogammaglobulinaemia at the end of follow-up that were unknown before rituximab were documented. This problem was described early on in adult patients receiving rituximab for rheumatoid arthritis and SLE (Edwards et al, 2006) or ITP (Levy et al, 2014) and more recently in 3 SLE children (Olfat et al, 2015) and in some children with auto-immune lymphoproliferative syndrome (Cooper et al, 2009;Teachey, 2012) treated with rituximab for ITP or AIHA. These conditions had been excluded for our 4 patients, as recommended.…”

According to the 2019 updated European League Against Rheumatism (EULAR) recommendations for the treatment of systemic lupus erythematosus: debatable issues and comments

“…Исключением из этого правила является тяжелая аутоиммунная тромбоцитопения или гемолитическая анемия, при которых РТМ продемонстрировал неоспоримый положительный эффект как у пациентов с СКВ, так и при изолированной иммунной тромбоцитопенической пурпуре. В лечении ВН РТМ применяется в отсутствие эффекта от препаратов первой линии (ЦФ и ММФ) или при рецидивирующем течении заболевании [74][75][76][77][78][79][80][81][82].…”