Paediatric Systemic lupus erythematosus (SLE) constitutes 10 to 20% of cases of SLE with more severe disease and higher mortality. We report a case of an adolescent girl with SLE with multisystem involvement who was started on hydroxychloroquine and oral prednisolone. However, due to persistent worsening of skin lesions and falling cell counts, pulsed dexamethasone was initiated which showed improvement in the skin lesions, cell counts, proteinuria and pleural effusion but there was a persistent fall in the haemoglobin. The patient was diagnosed with refractory autoimmune hemolytic anemia (AIHA) and treated with Rituximab which showed marked improvement in AIHA. Therefore, Rituximab can be considered in managing childhood SLE with refractory AIHA.
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