Lin Emily, Lee Brian T
Department of Medicine, Loma Linda University Medical Center, Loma Linda, CA.
Department of Gastroenterology and Transplant Institute, Loma Linda University Health, Loma Linda, CA.
ACG Case Rep J. 2022 Jul 6;9(7):e00812. doi: 10.14309/crj.0000000000000812. eCollection 2022 Jul.
A 69-year-old man with no history of liver disease presented with massive ascites. Imaging demonstrated diffuse wall thickening of the entire aorta, renal pelvis, and ureters along with an enlarged main portal vein, portosystemic collaterals, and peritoneal thickening concerning for large vessel vasculitis. Liver biopsy was consistent with obliterative portal venopathy. The patient was started on corticosteroid therapy with improvement in his ascites. This case study reveals a rare association between vasculitis and portal-sinusoidal vascular disease and idiopathic non-cirrhotic portal hypertension, highlighting the heterogenous clinical presentation of this disease entity.
一名无肝脏疾病史的69岁男性出现大量腹水。影像学检查显示整个主动脉、肾盂和输尿管弥漫性壁增厚，同时主门静脉增粗、门体侧支循环形成以及腹膜增厚，提示大血管血管炎。肝活检符合闭塞性门静脉病。患者开始接受皮质类固醇治疗，腹水有所改善。本病例研究揭示了血管炎与门静脉窦血管疾病及特发性非肝硬化门静脉高压之间罕见的关联，凸显了该疾病实体的异质性临床表现。
