title: Overlap of idiopathic portal hypertension and scleroderma: report of two autopsy cases and a review of literature.
abstract: Idiopathic portal hypertension (IPH) is characterized by dense fibrosis of portal tracts and portal venous obliteration. Little is known about the etiopathogenesis of IPH. Association of various autoimmune diseases such as systemic lupus erythematosus in IPH suggests that IPH may share immunological disturbances with such autoimmune diseases. We recently experienced two autopsy cases presenting with both diffuse scleroderma and IPH. Dense fibrosis was found in both the dermis and intrahepatic portal tract of these cases. In addition, small vascular damages were commonly observed to various degrees in these fibrotic areas of both organs. The activation of fibroblasts and vascular damages mediated by various growth factors and cytokines reportedly involved in the dermis in scleroderma might have also been operative in portal tracts in these two cases of IPH. A review of literature disclosed eight overlapping cases of IPH and scleroderma (middle- to old-aged females), and scleroderma was diagnosed earlier than IPH. These findings suggest that similar pathogenetic processes are operative in the dermis as well as in the portal tracts of the liver in these cases.
authors.affiliation: Department of Pathology, Kanazawa University School of Medicine, Japan.
authors.forename: Koichi
authors.initials: K
authors.lastname: Tsuneyama
authors.forename: Kenichi
authors.initials: K
authors.lastname: Harada
authors.forename: Kazuyoshi
authors.initials: K
authors.lastname: Katayanagi
authors.forename: Kishichiro
authors.initials: K
authors.lastname: Watanabe
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Kurumaya
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Minato
authors.forename: Yasuni
authors.initials: Y
authors.lastname: Nakanuma
mesh_terms: D005260:Female; D006801:Humans; D006975:Hypertension, Portal / Q000150:complications* / Q000473:pathology; D008099:Liver / Q000473:pathology; D008875:Middle Aged; D011169:Portal Vein / Q000473:pathology; D012595:Scleroderma, Systemic / Q000150:complications* / Q000473:pathology; D012867:Skin / Q000473:pathology