Adult onset Still's disease as a cause of acute liver failure.

ABSTRACT

A 29-year old lady presented to the hospital with high-grade intermittent fever, arthritis and macular skin rash. Investigations revealed anaemia, polymorphonuclear leucocytosis and raised erythrocyte sedimentation rate. Other tests including those for antinuclear antibody and rheumatoid factor were normal. The serum ferritin level however was raised. On the basis of these parameters a diagnosis of Still's disease was made. Treatment comprising oral steroids and anti-inflammatory agents was instituted. The patient recovered and was discharged only to present ten days later following 3 episodes of generalised tonic clonic seizures. Investigations revealed a persistently high serum ferritin with abnormal liver function test results. Jaundice developed and the patient went into grade IV hepatic encephalopathy following which she died. Still's disease is an idiopathic disease, diagnosed purely on the basis of the typical clinical features of the illness which include persistent arthritis, high fever, anaemia and an erythematous rash. Treatment for Still's disease mainly includes steroids and non-steroidal anti-inflammatory agents. Second-line treatment includes that used for controlling the arthritis and comprises gold, hydroxychloroquine, penicillamine, azathioprine, methotrexate, and cyclophosphamide.