You Byung Chul, Jeong Soung Won, Jang Jae Young, Goo So Mi, Kim Sang Gyune, Kim Young Seok, Jeon Chan Hong, Jeen Yoon Mi
Department of Internal Medicine, Soon Chun Hyang University College of Medicine, Seoul, Korea.
Korean J Gastroenterol. 2012 Jan;59(1):48-52. doi: 10.4166/kjg.2012.59.1.48.
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.
系统性硬化症（SSc）是一种慢性全身性疾病，可累及皮肤、肺、心脏、胃肠道、肾脏和肌肉骨骼系统。尽管据估计高达90%的硬皮病患者有胃肠道受累，但肝病的报道却很少。一名51岁女性因食管静脉曲张破裂出血入院。她的血清抗核抗体和抗着丝点抗体呈阳性。双手出现指（趾）硬皮病，且她最近出现了雷诺综合征。手部皮肤打孔活检显示表皮角化过度、棘层规则肥厚和基底色素沉着增加，真皮可见增厚的淡色胶原束。肝活检显示慢性活动性肝炎伴桥接纤维化。因此，她被诊断为自身免疫性肝炎（AIH）合并SSc导致的肝硬化。每天服用40mg泼尼松龙后，AIH已消退。她作为门诊患者接受5-10mg/天的泼尼松龙治疗，病情一直保持稳定。AIH或SSc患者均应监测是否并发其他自身免疫性疾病。AIH合并SSc的早期诊断有助于实现最佳治疗。
