title: 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.
authors.affiliation: Department of General Internal Medicine, NHO Nagasaki Medical Center, Kubara 2-1001-1 Omura, Nagasaki, 856-8562, Japan.
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Kogawa
authors.forename: Kiyoshi
authors.initials: K
authors.lastname: Migita
authors.forename: Masahiro
authors.initials: M
authors.lastname: Ito
authors.forename: Yasushi
authors.initials: Y
authors.lastname: Takii
authors.forename: Manabu
authors.initials: M
authors.lastname: Daikoku
authors.forename: Mikiko
authors.initials: M
authors.lastname: Nakao
authors.forename: Taichiro
authors.initials: T
authors.lastname: Miyashita
authors.forename: Hironori
authors.initials: H
authors.lastname: Kimura
authors.forename: Hironori
authors.initials: H
authors.lastname: Ezaki
authors.forename: Minoru
authors.initials: M
authors.lastname: Nakamura
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Yatsuhashi
authors.forename: Katsumi
authors.initials: K
authors.lastname: Eguchi
authors.forename: Hiromi
authors.initials: H
authors.lastname: Ishibashi
mesh_terms: D000368:Aged; D015897:Comorbidity; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D006975:Hypertension, Portal / Q000175:diagnosis / Q000209:etiology / Q000473:pathology / Q000503:physiopathology; D007564:Japan; D012595:Scleroderma, Systemic / Q000150:complications / Q000175:diagnosis / Q000473:pathology / Q000503:physiopathology; D012859:Sjogren's Syndrome / Q000150:complications / Q000175:diagnosis / Q000473:pathology / Q000503:physiopathology