title: Autoimmune hepatitis associated with pulmonary arterial hypertension.
abstract: A 46-year-old woman presented with arthralgia. She had a history of fluctuating liver function impairment for 6 months. Laboratory investigations revealed elevated liver function test results, positive antinuclear antibodies and elevated serum IgG. The histological findings of a liver biopsy were interface hepatitis accompanied by plasmocytic infiltration with bridging fibrosis. There was no evidence of cirrhosis on pathological examination and no portal hypertension on endoscopic and radiographic studies. Autoimmune hepatitis was diagnosed, and treatment with prednisolone improved the liver dysfunction. After 6 months, she complained of dyspnea. Doppler echocardiography showed a dilated right ventricle, severe tricuspid insufficiency, and systolic pulmonary arterial pressure indicative of pulmonary arterial hypertension. We report this rare case of autoimmune hepatitis with pulmonary arterial hypertension.
authors.affiliation: Department of Internal Medicine, Kitasato Institute Medical Center Hospital, Kitasato University, Kitamoto.
authors.forename: Fumihiko
authors.initials: F
authors.lastname: Kaneko
authors.forename: Hiroaki
authors.initials: H
authors.lastname: Yokomori
authors.forename: Kumiko
authors.initials: K
authors.lastname: Tahara
authors.forename: Tomochika
authors.initials: T
authors.lastname: Takeshita
authors.forename: Hajime
authors.initials: H
authors.lastname: Takeuchi
authors.forename: Hide
authors.initials: H
authors.lastname: Yoshida
authors.forename: Kenta
authors.initials: K
authors.lastname: Hoshi
authors.forename: Hirobumi
authors.initials: H
authors.lastname: Kondo
authors.forename: Makoto
authors.initials: M
authors.lastname: Ohbu
authors.forename: Tooru
authors.initials: T
authors.lastname: Sato
authors.forename: Toshifumi
authors.initials: T
authors.lastname: Hibi
mesh_terms: D005260:Female; D019693:Hepatitis, Autoimmune / Q000150:complications* / Q000175:diagnosis* / Q000473:pathology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications* / Q000175:diagnosis* / Q000473:pathology; D008875:Middle Aged; D011651:Pulmonary Artery / Q000473:pathology*