title: Response to steroids in Budd-Chiari syndrome caused by idiopathic granulomatous venulitis.
abstract: A 31-yr-old man presented with the Budd-Chiari syndrome. A liver biopsy specimen demonstrated noncaseating centrilobular granulomas and an idiopathic necrotizing granulomatous venulitis involving hepatic vein radicles with superimposed thrombotic occlusion. High-dose prednisone therapy produced dramatic clinical improvement with resolution of the hepatomegaly, ascites, and portal hypertension and normalization of the hepatic venous angiogram. A repeat liver biopsy specimen showed resolution of the venulitis and disappearance of the hepatic vein thrombosis. Idiopathic granulomatous venulitis that is reversible with prednisone therapy should be considered in the differential diagnosis of the Budd-Chiari syndrome.
authors.affiliation: Department of Pathology, Queen's University, Kingston, Ontario, Canada.
authors.forename: I D
authors.initials: ID
authors.lastname: Young
authors.forename: R N
authors.initials: RN
authors.lastname: Clark
authors.forename: P N
authors.initials: PN
authors.lastname: Manley
authors.forename: A
authors.initials: A
authors.lastname: Groll
authors.forename: J B
authors.initials: JB
authors.lastname: Simon
mesh_terms: D000328:Adult; D006502:Budd-Chiari Syndrome / Q000209:etiology* / Q000473:pathology; D006099:Granuloma / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D006503:Hepatic Veins / Q000473:pathology*; D006801:Humans; D008099:Liver / Q000473:pathology; D008107:Liver Diseases / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D008297:Male; D009336:Necrosis; D011241:Prednisone / Q000627:therapeutic use*; D014657:Vasculitis / Q000150:complications / Q000188:drug therapy / Q000473:pathology
chemical_list: D011241:Prednisone