Liver Cirrhosis Due to Autoimmune Hepatitis Combined with Systemic Sclerosis.
- Author:
Byung Chul YOU
1
;
Soung Won JEONG
;
Jae Young JANG
;
So Mi GOO
;
Sang Gyune KIM
;
Young Seok KIM
;
Chan Hong JEON
;
Yoon Mi JEEN
Author Information
- Publication Type:Case Reports
- Keywords: Systemic scleroderma; Autoimmune hepatitis; Liver cirrhosis
- MeSH: Anti-Inflammatory Agents/therapeutic use; Antibodies, Antinuclear/blood; Esophageal and Gastric Varices; Female; Gastrointestinal Hemorrhage; Hepatitis, Autoimmune/complications/*diagnosis/drug therapy; Humans; Liver Cirrhosis/*diagnosis/etiology/pathology; Middle Aged; Prednisolone/therapeutic use; Raynaud Disease/diagnosis; Scleroderma, Systemic/complications/*diagnosis; Skin/pathology
- From:The Korean Journal of Gastroenterology 2012;59(1):48-52
- CountryRepublic of Korea
- Language:English
- 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.
