Abstract
We report a case of progressive systemic sclerosis (PSS) complicated with chronic active hepatitis and esophageal ulcer. This 42 year-old male patient was admitted to our hospital because of oppressive sensation on swallowing. His present illness disclosed edema of the 4 extremities 17 years ago, Raynaud's phenomenon 12 years before, sclerotic change of the fingers 7 years ago and hematemesis 1 year previously. He had masked face, sclerotic change of the skin and arthralgia of the left wrist with morning stiffness.
Laboratory studies revealed positive RA test, increased γ-globulin levels, negative HBsAg and Ab, KICG 0.12, serum compliment 45.4 CH50 u, negative anti-mitochondrial antibody, T-cells 80.2%, B-cells 14.2%, urinary 4-hydroxyproline 42.96mg/day, etc. The histology of the biopsied skin disclosed marked fibrosis in the true skin, which was consistent with PSS. Two esophageal ulcers were demonstrated in endoscopic examination. The surface of the liver showed slight unevenness, increased white network patterns and moderate splenomegaly on peritoneoscopic examination.
The histology of the biopsied liver disclosed chronic active hepatitis (CAH, 2A according to the European classification) and the relation with autoimmune mechanism was conjectured.
