전신성 경화증과 동반된 자가면역성 간염에 의한 간경변

• Published in: The Korean journal of gastroenterology Vol. 59; no. 1; pp. 48 - 52
• Main Authors: 유병철, Byung Chul You, 정승원, Soung Won Jeong, 장재영, Jae Young Jang, 구소미, So Mi Goo, 김상균, Sang Gyune Kim, 김영석, Young Seok Kim, 전찬홍, Chan Hong Jeon, 진윤미, Yoon Mi Jeen
• Format: Journal Article
• Language: English; Korean
• Published: 대한소화기학회 30.01.2012
• Subjects: Autoimmune hepatitis; Liver cirrhosis; Systemic scleroderma; 내과학
• ISSN: 1598-9992; 2233-6869

Systemic sclerosis (SSc) is a chronic systemic disease that affects the skin, lungs, heart, gastrointestinal tract, kidneys, and musculoskeletal system. Although up to 90% of patients with scleroderma have been estimated to have gastrointestinal involvement, liver disease has been reported only rarely. A 51-year-old woman was hospitalized due to esophageal variceal bleeding. Her serum was positive for anti-nuclear antibody and anti-centromere antibody. Sclerodactyly was noted on both hands, and she had recently developed Raynaud`s syndrome. Punch biopsy of the hand showed hyperkeratosis, regular acanthosis, and increased basal pigmentation in the epidermis, and thick pale collagenous bundles in the dermis. Liver biopsy showed chronic active hepatitis with bridging fibrosis. Consequently, she was diagnosed with liver cirrhosis due to autoimmune hepatitis (AIH) combined with SSc. AIH had subsided after administration of prednisolone at 40 mg per day. She received 5-10 mg/day of prednisolone as an outpatient, and her condition has remained stable. Patients with either AIH or SSc should be monitored for further development of concurrent autoimmune diseases. The early diagnosis of AIH combined with SSc will be helpful in achieving optimal management. (Korean J Gastroenterol 2012;59:48-52).