A case of adult onset Still's disease complicated with adult respiratory distress syndrome and disseminated intravascular coagulation

Abstract

A 71 year-old man with adult onset Still's disease was admitted to our hospital because of fever, sore throat, myalgia and macular nonpruritic salmon pink eruption. He was treated with prednisolone, 40mg daily and these symptoms disappeared. When the dose of prednisolone was reduced to 30mg daily, he began to notice fever. 5 days later he developed adult respiratory distress syndrome (ARDS). The dose of prednisolone was increased to 50mg daily and oxygen administration was started. All symptoms began to improve immediately and the dose of prednisolone was decreased to 40mg daily. 10 days later he noticed fever and skin rash. Laboratory investigation showed platelet counts of 69, 000/mm3, a ferritin of 37, 000ng/ml, and increased fibrinogen degradation product, indicating increased activity of adult onset Still's disease associated with disseminated intravascular coagulation (DIC). The dose of prednisolone was again increased to 60mg daily, and 100mg of nafamostat mesilate was administrated intravenously. All above symptoms associated with adult onset Still's disease and DIC disappeared. The dose of prednisolone was gradually decreased and the clinical course was uneventful with daily administration of 10mg of prednisolone. Although there are a couple of case report which described the association of adult onset Still's disease with either ARDS or DIC, the association of adult onset Still's disease with both ARDS and DIC have not been reported yet. © 1995, The Japan Society for Clinical Immunology. All rights reserved.