A case of adult-onset Still's disease with macrophage activation syndrome and acute liver failure

Abstract

A 26-year-old Japanese woman was admitted to our hospital because of spiking fever, eruption, and liver dysfunction. High fever continued after admission, and antibiotics were not effective. The patient was diagnosed as adult-onset Still's disease after excluding leukemia and malignant lymphoma. Because the patient was complicated with acute liver failure, steroid pulse therapy was started. Although steroid pulse therapy was transiently effective for clinical symptoms, oral predonisolone was not. Moreover, the patient was complicated with severe cytopenia. We diagnosed with macrophage activation syndrome complicated with adult-onset Still's disease. She was treated with cyclosporine A and liposteroid, resulted in complete remission for macrophage activation syndrome and adult-onset Still's disease. In summary of our case, combination of cyclosporine A and liposteroid was effective for the treatment of macrophage activation syndrome with acute liver failure in a patient with adult-onset Still's disease.