Idiopathic portal hypertension associated with systemic sclerosis and Sjögren's syndrome.

Abstract:

:We report a patient with idiopathic portal hypertension (IPH) associated with systemic sclerosis (SSc) and Sjögren's syndrome. A 72-year-old Japanese woman was admitted to our hospital because of Raynaud's phenomenon, sclerodactyly, and dyspnea. The patient had splenomegaly, esophageal varices in the absence of extrahepatic portal obstruction, and cirrhosis of the liver. Immunological studies revealed positive anti-nuclear antibodies and high titers of anti-Scl-70, anti-SS-A, anti-centromere, and anti-mitochondrial M2 antibodies. Histological examinations of the liver biopsy specimen revealed stenosis and loss of small portal veins without findings of primary biliary cirrhosis. The patient was diagnosed as having IPH associated with SSc and Sjögren's syndrome. These observations suggest an immunological role in the pathogenesis of IPH.

journal_name

Clin Rheumatol

journal_title

Clinical rheumatology

authors

Kogawa H,Migita K,Ito M,Takii Y,Daikoku M,Nakao M,Miyashita T,Kimura H,Ezaki H,Nakamura M,Yatsuhashi H,Eguchi K,Ishibashi H

doi

10.1007/s10067-005-1084-y

subject

Has Abstract

pub_date

2005-09-01 00:00:00

pages

544-7

issue

5

eissn

0770-3198

issn

1434-9949

journal_volume

24

pub_type

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