Atrophic autoimmune pangastritis: A distinctive form of antral and fundic gastritis associated with systemic autoimmune disease

Atrophic autoimmune pangastritis: A distinctive form of antral and fundic gastritis associated with systemic autoimmune disease
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DOI:
10.1097/01.pas.0000213337.25111.37
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发表时间:
2006-11-01
影响因子:
5.6
通讯作者:
Abraham, Susan C.
Abraham, Susan C.
中科院分区:
医学1区
文献类型:
--
作者:
Jevremovic, Dragan;Torbenson, Michael;Abraham, Susan C.

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公认的萎缩性胃炎的两种主要形式是自身免疫性和环境性萎缩性胃炎。它们在胃中的地形分布、组织学特征和病因学上都不同。自身免疫性萎缩性胃炎是由免疫介导的特异性含氧腺体破坏引起的,局限于身体和眼底,表现为特征性的神经内分泌增生。环境萎缩性胃炎与长期存在的幽门螺杆菌感染有关,并优先累及胃窦和移行区粘膜。在这项研究中,我们描述了一种独特的萎缩性胃炎,它与这两种经典变种都有明显的不同。本病的特点是:(1)强烈的粘膜炎性浸润,直至腺体严重萎缩;(2)胃壁分布弥漫性累及体腔,(3)与幽门螺杆菌缺乏联系,(4)神经内分泌缺乏。8例患者年龄从1岁到75岁不等,女性略占优势(5F:3M)。均有全身性自身免疫性和/或结缔组织病,包括自身免疫性小肠结肠炎(4例)、系统性红斑狼疮、难治性流行性出血热、自身免疫性溶血性贫血和致残纤维肌痛。8例患者中有7例自身免疫标志物阳性(87%),但仅1例患者进行了抗壁细胞抗体和抗固有因子抗体的血清检测,结果均为阴性。我们认为,这种形式的萎缩性硬化性胃炎的独特组织学及其与全身性自身免疫性疾病的关联表明,针对胃中的多个细胞系的自身免疫过程。1例19岁女性患者发生多灶性低度异型增生,提示这种疾病可能具有肿瘤潜能。
The 2 major recognized forms of atrophic gastritis are autoimmune and environmental atrophic gastritis. These differ in their topographical distribution in the stomach, histologic features, and etiology. Autoimmune atrophic gastritis results from immune-mediated destruction of specialized oxyntic glands, is restricted to the body and fundus, and shows characteristic neuroendocrine hyperplasia. Environmental atrophic gastritis is associated with long-standing Helicobacter pylori infection and preferentially involves antrum and transition zone mucosa. In this study, we describe a distinctive form of atrophic gastritis that differs markedly from both of these classic variants. This gastritis is characterized by: (1) intense mucosal inflammatory infiltrates, persisting even into the phase of severe glandular atrophy, (2) pangastric distribution with diffuse involvement of both body and antrum, (3) lack of association with H. pylori, and (4) lack of neuroendocrine hyperplasia. The 8 patients presented ranged from 1 to 75 years and showed a slight female predominance (5F:3M). All had systemic autoimmune and/or connective tissue diseases including autoimmune enterocolitis (4 cases), systemic lupus erythematosus, refractory sprue, autoimmune hemolytic anemia, and disabling fibromyalgia. Positive serum autoimmune markers were documented in 7 of 8 (87%) patients, but serologies for antiparietal cell and anti-intrinsic factor antibodies were undertaken in only 1 patient each and were negative. We propose that the distinctive histology of this form of atrophic pangastritis and its association with systemic autoimmune disease suggests an autoimmune process directed against multiple cell lineages in the stomach. The development of multifocal low-grade dysplasia in 1 patient, a 19-year-old woman, suggests that this condition might have neoplastic potential.