Idiopathic portal hypertension(IPH) accompanied with chronic rheumatoid arthritis - Report of an autopsy case.

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The patient, 68 years old female, had rheumatoid arthritis for 24 years and splenomegaly was pointed out 14 years ago. Hematemesis due to rupture of esophageal varix occurred two years ago. She admitted to the hospital with a chief complaint of hematemesis. Laboratory examinations revealed; hypergammaglobulinemia, thrombocytopenia, ascites, splenomegaly and positive results of RA, ANA and ASMA. She died of severe pancytopenia. Post mortem examination revealed the atrophic noncirrhotic liver (560g) with thrombosis in the intrahepatic large portal vein, esophageal varicies and splenomegaly (300g). The histology of the liver showed peculiar findings such as; fibrous enlargement of Glisson's capsule with marked canstriction of small portal vein branches, development of ectopic portal vessels, lamellar concentric fibrosis around the bile duct and dislocation of both Glisson's capsule and hepatic vein. No pseudolobule was visible. Immunohistochemical stains failed to demonstrate deposition of immunoglobulins as well as compliments in the liver.<BR>The present case, being compatible with IPH in either the clinical course or the morphology of the liver, suggests that some immunological abnormality, which is common to rheumatoid arthritis, may be related to the development of IPH.
社団法人日本肝臓学会の論文