Fulminant hepatic failure due to cardiac tamponade associated with mixed connective tissue disease

We describe a 42-year old Japanese woman with mixed connective tissue disease (MCTD) who developed fulminant hepatic failure and hepatic encephalopathy. Massive pericardial effusion accompanying cardiac tamponade was shown by echocardiography. The hepatic failure was considered to be caused by a low cardiac output state because of cardiac tamponade, which might be due to cardiac involvement of MCTD. This is a rare case, showing an unusual progressive course in MCTD.