title: Liver cirrhosis due to autoimmune hepatitis combined with systemic sclerosis.
abstract: 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.
authors.affiliation: Department of Internal Medicine, Soon Chun Hyang University College of Medicine, Seoul, Korea.
authors.forename: Byung Chul
authors.initials: BC
authors.lastname: You
authors.forename: Soung Won
authors.initials: SW
authors.lastname: Jeong
authors.forename: Jae Young
authors.initials: JY
authors.lastname: Jang
authors.forename: So Mi
authors.initials: SM
authors.lastname: Goo
authors.forename: Sang Gyune
authors.initials: SG
authors.lastname: Kim
authors.forename: Young Seok
authors.initials: YS
authors.lastname: Kim
authors.forename: Chan Hong
authors.initials: CH
authors.lastname: Jeon
authors.forename: Yoon Mi
authors.initials: YM
authors.lastname: Jeen
mesh_terms: D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D000974:Antibodies, Antinuclear / Q000097:blood; D004932:Esophageal and Gastric Varices; D005260:Female; D006471:Gastrointestinal Hemorrhage; D019693:Hepatitis, Autoimmune / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D006801:Humans; D008103:Liver Cirrhosis / Q000175:diagnosis* / Q000209:etiology / Q000473:pathology; D008875:Middle Aged; D011239:Prednisolone / Q000627:therapeutic use; D011928:Raynaud Disease / Q000175:diagnosis; D012595:Scleroderma, Systemic / Q000150:complications / Q000175:diagnosis*; D012867:Skin / Q000473:pathology
chemical_list: D000893:Anti-Inflammatory Agents; D000974:Antibodies, Antinuclear; C538950:anticentromere antibody; D011239:Prednisolone