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01.10.2005 | Case Report

Idiopathic portal hypertension associated with systemic sclerosis and Sjögren’s syndrome

verfasst von: Hiroyuki Kogawa, Kiyoshi Migita, Masahiro Ito, Yasushi Takii, Manabu Daikoku, Mikiko Nakao, Taichiro Miyashita, Hironori Kimura, Hironori Ezaki, Minoru Nakamura, Hiroshi Yatsuhashi, Katsumi Eguchi, Hiromi Ishibashi

Erschienen in: Clinical Rheumatology | Ausgabe 5/2005

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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.

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Titel: Idiopathic portal hypertension associated with systemic sclerosis and Sjögren’s syndrome
verfasst von: Hiroyuki Kogawa
Kiyoshi Migita
Masahiro Ito
Yasushi Takii
Manabu Daikoku
Mikiko Nakao
Taichiro Miyashita
Hironori Kimura
Hironori Ezaki
Minoru Nakamura
Hiroshi Yatsuhashi
Katsumi Eguchi
Hiromi Ishibashi
Publikationsdatum: 01.10.2005
Verlag: Springer-Verlag
Erschienen in: Clinical Rheumatology / Ausgabe 5/2005
Print ISSN: 0770-3198
Elektronische ISSN: 1434-9949
DOI: https://doi.org/10.1007/s10067-005-1084-y

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