肝不全で死亡した全身性エリテマトーデスの1例

概要

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A 38-year-old man who had been treated as a patient with systemic lupus erythematosus (SLE) during the last 12 years was admitted to the Kawasaki Municipal Hospital in February, 1986, because of high fever. Anemia due to both autoimmune hemolytic anemia and follic acid deficiency was confirmed. After the administration of prednisolone together with follic acid, the patient was recovered from anemia.In July, 1986, jaundice appeared abruptly and liver function deteriorated. After the administration of prednisolon, jaundice and liver function improved slightly. But in 1987, total bilirubin increased up to 30-53mg/dl. Despite therapeutic trials including high dose of steroid and plasma exchange, liver dysfunction continued. Finally hemorrhagic tendency, hypoproteinemia and consciousness disturbance appeared and the patient died on February, 1987.An autopsy revealed; There were primary cholestatic liver fibrosis, focal segmental prolif-erative lupus nephritis and the Libman-Sacks endocarditis. Scince the severe liver dysfunction is unusual in the patient with SLE, we report this rare case.