Zamani Farhad, Amiri Afsaneh, Shakeri Ramin, Zare Ali, Mohamadnejad Mehdi
Gastrointestinal and Liver Disease Research Center, Firouzgar Hospital, Iran University of Medical Sciences, Tehran, Iran.
J Med Case Rep. 2009 Feb 16;3:68. doi: 10.1186/1752-1947-3-68.
Idiopathic portal hypertension is a disorder of unknown etiology, clinically characterized by portal hypertension, splenomegaly and anemia secondary to hypersplenism.
A 54-year-old man was admitted to our hospital for evaluation of malaise, weight loss, abdominal swelling and lower limb edema. His paraclinical tests revealed pancytopenia, large ascites, splenomegaly and esophageal varices consistent with portal hypertension. Duodenal biopsy and serologic findings were compatible with celiac disease. His symptoms improved on a gluten-free diet, but his clinical course was further complicated with ulcerative jejunoileitis, and intestinal T-cell lymphoma.
It seems that celiac disease, by an increased immune reaction in the splenoportal axis, can result in the development of idiopathic portal hypertension in susceptible affected patients.
特发性门静脉高压是一种病因不明的疾病，临床特征为门静脉高压、脾肿大以及继发于脾功能亢进的贫血。
一名54岁男性因全身乏力、体重减轻、腹部肿胀和下肢水肿入院接受评估。他的辅助检查显示全血细胞减少、大量腹水、脾肿大以及与门静脉高压相符的食管静脉曲张。十二指肠活检和血清学检查结果与乳糜泻相符。他的症状在无麸质饮食后有所改善，但临床病程因溃疡性空回肠炎和肠道T细胞淋巴瘤而进一步复杂化。
乳糜泻似乎通过脾门静脉轴免疫反应增强，可导致易感患者发生特发性门静脉高压。
