Korean J Gastroenterol.  2012 Jan;59(1):48-52. 10.4166/kjg.2012.59.1.48.

Liver Cirrhosis Due to Autoimmune Hepatitis Combined with Systemic Sclerosis

• You BC
• Jeong SW
• Jang JY
• Goo SM
• Kim SG
• Kim YS
• Jeon CH
• Jeen YM

• KMID: 1775833
• DOI: http://doi.org/10.4166/kjg.2012.59.1.48

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.

Keyword

Systemic scleroderma; Autoimmune hepatitis; Liver cirrhosis

MeSH Terms

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

Figure

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  Fig. 1 Skin manifestations and biopsy. (A) Sclerodactyly development at the tips of the fingers, skin indurations, and fixed flexion contractures at the proximal interphalangeal joints. (B) Section of the skin biopsy specimen showing collagen bundles of the reticular dermis appear to be thickened, hypocellular and deeply eosinophilic (H&E, ×200).

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  Fig. 2 Liver biopsy. (A) Multiple drop out of hepatocytes replaced by lymphoid cells and acidophilic bodies were seen in the acini (H&E, ×200). (B) Portal and periportal infiltration of lymphoid cells and a few plasma cells were seen. The bile ducts seemed to be intact (H&E, ×400). (C) Masson's trichrome staining revealed portal and periportal fibrosis with bridging (×100).

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