중복증후군에서 병발한 원발쓸개관간경화 환자의 아달리무맙 치료 1예

Abstract

Primary biliary cirrhosis (PBC) is occasionally developed in patients with rheumatic diseases, such as systemic sclerosis or Sjogren’s syndrome. However, there are a few reports of overlap syndrome with PBC. The authors report a case of a 36 year-old female with PBC and overlap syndrome. Systemic sclerosis was diagnosed in 2007, and rheumatoid arthritis in 2010. Adalimumab stopped because of her pregnancy plan in January 2012. One month after delivery, she felt increased hand joint pain and fatigue. Laboratory findings were as follows: elevated AST, ALT, ALP, r-GTP and positive anti-mitochondrial antibody. Histology of a liver biopsy revealed moderate porto-periportal and mild lobular inflammation with bile duct inflammation, which was consistent with PBC. She was treated with prednisolone and UDCA (urosodeoxycholic acid), but her disease was not controlled. From May 2013, she has been treated with adalimumab. Her arthritis was improved and liver function test normalized up until now.