title: Idiopathic portal hypertension complicating systemic sclerosis: a case report.
abstract: BACKGROUND
  Patients with systemic sclerosis may develop mild abnormalities of liver function tests. More serious hepatic involvement has been well documented but is rare. Idiopathic portal hypertension had been reported only in a few female patients with systemic sclerosis.
  
  
  METHODS
  An 82-year-old man with known systemic sclerosis presented with melaena. Urgent gastroscopy revealed oesophageal varices, which re-started bleeding during the procedure and were treated ensocopically, with Sengstaken tube and glypressin. Liver function tests and coagulation were normal. Non-invasive liver screen (including hepatitis viral serology and autoantibodies) was negative. Ultrasound scan of the abdomen revealed a small liver with coarse texture and no focal lesion. Hepato-portal flow was demonstrated in the portal vein. The spleen was enlarged. A moderate amount of free peritoneal fluid was present. A CT scan confirmed the absence of portal vein thrombosis. One month following discharge the patient had a liver biopsy. Histological examination showed essentially normal liver tissue; there was no evidence of any excess inflammation and no features to suggest cirrhosis or drug-induced liver disease. Taking into account the above evaluation we concluded that the patient had idiopathic portal hypertension.
  
  
  CONCLUSIONS
  Both male and female patients with systemic sclerosis may -- rarely -- develop idiopathic portal hypertension.
authors.affiliation: Department of Gastroenterology, Queen Elizabeth Hospital, Sheriff Hill, Gateshead NE6 9SX, UK. gut@in.gr
authors.forename: John
authors.initials: J
authors.lastname: Moschos
authors.forename: Grigoris I
authors.initials: GI
authors.lastname: Leontiadis
authors.forename: Clive
authors.initials: C
authors.lastname: Kelly
authors.forename: James
authors.initials: J
authors.lastname: Henry
authors.forename: Savvas
authors.initials: S
authors.lastname: Kadis
mesh_terms: D000369:Aged, 80 and over; D004932:Esophageal and Gastric Varices / Q000150:complications; D006801:Humans; D006975:Hypertension, Portal / Q000150:complications* / Q000503:physiopathology; D008102:Liver Circulation; D008297:Male; D008551:Melena / Q000150:complications; D011168:Portal System / Q000503:physiopathology; D012595:Scleroderma, Systemic / Q000150:complications*