A Case of Chronic Lymphoplasmacellular Osteomyelitis with Autoimmune Hepatitis/Primary Sclerosing Cholangitis Overlap Syndrome in a Child

자가면역간염과 원발성 경화담관염을 가진 중복증후군 소아 환자에서 발생한 형질세포성 골수염 1예

The Overlap syndrome is characterized by a combination of the major hepatobiliary autoimmune diseases such as autoimmune hepatitis, primary biliary cirrhosis and primary sclerosing cholangitis. It is frequently accompanied by inflammatory bowel disease. Chronic lymphoplasmacellular osteomyelitis is characterized by recurrent episodes of bacterial osteomyelitis and is associated with autoimmune diseases (especially inflammatory bowel disease). We report the case of a girl who was diagnosed with ulcerative colitis and autoimmune hepatitis at 4 years of age and with the overlap syndrome with primary sclerosing cholangitis at 6 years. At 9 years, she was diagnosed with chronic lymphoplasmacellular osteomyelitis.

저자들은 4세에 진단된 자가면역간염과 궤양성 대장염 그리고 6세에 진단된 원발성 경화담관염을 가진 중 복증후군 환아에서 9세에 만성 림프형질세포성 골수염이 발병한 뒤 면역억제 치료에 잘 반응하지 않는 증례를 경험하였기에 보고하는 바이다.