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Severe refractory SLE — SCE Rheumatology MCQ

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HardConnective tissue diseasesSevere refractory SLESCE Rheumatology

A 41-year-old woman with systemic lupus erythematosus has recurrent severe immune thrombocytopenia despite hydroxychloroquine and mycophenolate mofetil. She has bruising and gum bleeding, with a platelet count of 18 × 10^9/L. Blood film shows no schistocytes, and haemoglobin, lactate dehydrogenase, haptoglobin, coagulation studies and renal function are normal. Infection and drug-induced thrombocytopenia have been excluded. Intravenous methylprednisolone and intravenous immunoglobulin produce only a transient platelet response. Which biologic is the most appropriate next steroid-sparing treatment?

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Correct answer: E — Rituximab

Rituximab is the best answer for recurrent, severe SLE-associated immune thrombocytopenia that has not achieved durable control with glucocorticoids, intravenous immunoglobulin and conventional immunosuppression. The absence of schistocytes, haemolysis and renal impairment argues against thrombotic microangiopathy, while the transient response to intravenous immunoglobulin supports immune thrombocytopenia. EULAR specifically includes rituximab among treatment options for severe autoimmune thrombocytopenia in SLE and as a maintenance option after induction. Belimumab and anifrolumab may be used for persistently active non-renal SLE but are not the preferred targeted treatment for this severe haematological phenotype. Obinutuzumab has principally been evaluated in lupus nephritis. Eculizumab is relevant to complement-mediated thrombotic microangiopathy rather than isolated immune thrombocytopenia. Rituximab use for SLE thrombocytopenia is specialist and off-label in the UK.

Reference: European Alliance of Associations for Rheumatology. EULAR recommendations for the management of systemic lupus erythematosus: 2023 update, haematological disease recommendations, 2023. https://www.eular.org/document/download/724/fe9e2ce7-b682-4f2f-aa41-7983eeb7a05c/697