Open-access Macrophage activation syndrome following the use of Leflunomide in a patient with adult-onset Still disease: case report

A 32-year-old white female, with adult-onset Still disease, was admitted following a 15-day course of leflunomide, with persistent fever and a generalized tonic-clonic seizure. She quickly developed liver and spleen enlargement, impairment of liver function, thrombocytopenia, elevation of lactate-dehydrogenase, trylicerides and ferritin, and renal failure, being diagnosed with the macrophage activation syndrome, a rare complication of rheumatic diseases due to activation and uncontrolled proliferation of T lymphocytes and excessive macrophage activation. The syndrome may lead to disseminated intravascular coagulation, renal impairment being a rare event. Pulse therapy with methylprednisolone and cyclosporine are the therapeutic options.

macrophage activation syndrome; Still disease; leflunomide


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