[Idiopathic portal hypertension associated with connective tissue disease similar to systemic lupus erythematosus].
A case of idiopathic portal hypertension associated with connective disease resembling systemic lupus erythematosus is described. The patient was a 50-year-old woman with splenomegaly, ascites, esophageal varices, and pancytopenia, but without extrahepatic portal obstruction or cirrhosis of the liver. Electron microscopy of the liver showed perisinusoidal fibrosis. High titers of autoantibodies against proliferating cell nuclear antigen (PCNA) were found in the sera as well as in ascites; anti-DNA antibodies appeared after anti-PCNA antibodies and remained thereafter at a moderate titer. The possibility of an immunological process in the pathogenesis of idiopathic portal hypertension is discussed.
Drouhin
F
F
Département d'Hépato-Gastroentérologie, Centre Hospitalier Louise-Michel, Evry.
Fischer
D
D
Vadrot
J
J
Denis
J
J
Johanet
C
C
Abuaf
N
N
Feldmann
G
G
Labayle
D
D
D000974:Antibodies, Antinuclear / Q000032:analysis; D003240:Connective Tissue Diseases / Q000150:complications* / Q000276:immunology; D005260:Female; D006801:Humans; D006975:Hypertension, Portal / Q000150:complications*; D008099:Liver / Q000648:ultrastructure; D008180:Lupus Erythematosus, Systemic / Q000276:immunology; D008875:Middle Aged; D009687:Nuclear Proteins / Q000032:analysis; D018809:Proliferating Cell Nuclear Antigen
D000974:Antibodies, Antinuclear; D009687:Nuclear Proteins; D018809:Proliferating Cell Nuclear Antigen
Hypertension portale idiopathique associée à une collagénose proche du lupus érythémateux disséminé.