Daniels Jason A, Torbenson Michael, Vivekanandan Perumal, Anders Robert A, Boitnott John K
Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
Hum Pathol. 2009 Apr;40(4):484-8. doi: 10.1016/j.humpath.2008.09.008. Epub 2008 Dec 11.
Common variable immunodeficiency is an important form of primary immunodeficiency disease. The most recognized histologic manifestation of common variable immunodeficiency is a paucity of plasma cells in gut biopsies. However, chronic inflammation can affect other organs including the liver. This study was designed to characterize the histologic findings in liver biopsies of individuals with common variable immunodeficiency. Thirteen liver biopsies from 10 patients were identified. The most common indication for biopsy was elevated liver enzymes, hepatomegaly, and/or splenomegaly. The biopsies typically showed mild portal and mild-to-moderate lobular chronic inflammation with minimal or absent interface activity. Plasma cells were absent in all cases. The biopsy specimens showed no fibrosis (n = 5) or mild portal fibrosis (n = 5). In 2 patients with follow-up biopsies, no fibrosis progression was identified. Four individuals showed small numbers of scattered portal and/or lobular granulomas, 3 of whom had diagnoses of coexistent sarcoidosis. Overall, the inflammatory changes in the biopsies were reminiscent of those seen in individuals with chronic inflammation of the gut, which can lead to translocation of intestinal luminal antigens to the liver and a mild hepatitis. Subsequent review of concurrent intestinal biopsies available in 7 individuals showed intestinal inflammation in 5 of 7 cases. In conclusion, liver biopsies in individuals with common variable immunodeficiency show mild portal and lobular inflammation with no or mild portal fibrosis. The etiology of the common variable immunodeficiency hepatitis remains unclear but, in some cases, may be secondary to mucosal inflammation in the gastrointestinal tract.
普通可变免疫缺陷是原发性免疫缺陷病的一种重要形式。普通可变免疫缺陷最公认的组织学表现是肠道活检中浆细胞数量稀少。然而，慢性炎症可影响包括肝脏在内的其他器官。本研究旨在描述普通可变免疫缺陷患者肝活检的组织学发现。从10例患者中获取了13份肝活检样本。活检最常见的指征是肝酶升高、肝肿大和/或脾肿大。活检通常显示轻度门静脉和轻度至中度小叶慢性炎症，界面活动轻微或无。所有病例均无浆细胞。活检标本未见纤维化（n = 5）或轻度门静脉纤维化（n = 5）。在2例接受随访活检的患者中，未发现纤维化进展。4例患者出现少量散在的门静脉和/或小叶肉芽肿，其中3例诊断为合并结节病。总体而言，活检中的炎症变化让人联想到肠道慢性炎症患者的炎症变化，肠道慢性炎症可导致肠腔内抗原转移至肝脏并引起轻度肝炎。随后对7例患者同时进行的肠道活检复查显示，7例中有5例存在肠道炎症。总之，普通可变免疫缺陷患者的肝活检显示轻度门静脉和小叶炎症，无门静脉纤维化或轻度门静脉纤维化。普通可变免疫缺陷性肝炎的病因尚不清楚，但在某些情况下，可能继发于胃肠道黏膜炎症。
