A case of primary biliary cirrhosis complicated by isolated ACTH deficiency

Abstract

A 58-year-old woman was diagnosed with primary biliary cirrhosis at a nearby hospital in 1997. In 2003 she was admitted to our hospital with erysipelas and acute poststreptococcal glomerulonephritis. On admission she was found to have pancytopenia and hypothyroidism, and was further diagnosed with isolated ACTH deficiency and also Hashimoto's disease as a result of a biochemical blood test. Both pancytopenia and hypothyroidism responded to hydrocortisone, and she was discharged. In 2007, however, she was readmitted to our hospital because of vertigo, vomiting and general fatigue, which are symptoms of acute adrenal insufficiency. After the administration of steroids, her condition improved temporarily, but she developed pneumonia and died of multiple organ failure due to respiratory failure and disseminated intravascular coagulation. Although primary biliary cirrhosis and isolated ACTH deficiency are considered autoimmune diseases, the combination of the two is extremely rare. To our knowledge this is the second reported case.