Department of Gastroenterology, West China Hospital, Sichuan University, No. 37, Guo Xue Xiang, Chengdu, 610041, People's Republic of China.
BMC Infect Dis. 2023 Mar 1;23(1):125. doi: 10.1186/s12879-023-08090-6.
The etiopathogenesis of idiopathic non-cirrhotic portal hypertension (INCPH) is so far poorly understood. Altered immunity, blood diseases, infections, congenital defects and drug exposure have been documented in a part of patients with INCPH owing to increased recognition of the disorder in patients with HIV, or various haematological disorders or autoimmune diseases. We aim to discuss the possible etiopathogenesis of INCPH.
We reported that a patient with intestinal infection of T. Marneffei and hyper-IgE syndrome, a group of rare primary immunodeficiency disorders, was finally diagnosed with INCPH for gastroesophageal variceal bleeding. The diagnosis was mainly based on histopathological features. Transjugular intrahepatic portosystemic shunt was performed and there was no recurrence of melena during the six-month follow-up.
In the context of immunodeficiency, INCPH may associated with intestinal infections. Thus, screening for enterogenic infection and immunological disorders in patients with unexplained portal hypertension is necessary.
特发性非肝硬化性门静脉高压症（INCPH）的病因学至今仍知之甚少。由于人们对 HIV 患者、各种血液系统疾病或自身免疫性疾病患者中这种疾病的认识增加，部分 INCPH 患者的免疫改变、血液疾病、感染、先天性缺陷和药物暴露已被记录下来。我们旨在讨论 INCPH 的可能病因学。
我们报告了一例患有马尔尼菲青霉肠道感染和高免疫球蛋白 E 综合征的患者，这是一组罕见的原发性免疫缺陷疾病，最终因胃食管静脉曲张出血而被诊断为 INCPH。该诊断主要基于组织病理学特征。进行了经颈静脉肝内门体分流术，在六个月的随访期间没有再次出现黑便。
在免疫缺陷的情况下，INCPH 可能与肠道感染有关。因此，对于不明原因的门静脉高压症患者，有必要筛查肠源性感染和免疫性疾病。
