Atrophic Autoimmune Pangastritis: A Distinctive Form of Antral and Fundic Gastritis Associated With Systemic Autoimmune Disease

Abstract

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.

A novel model might predict the risk of chronic atrophic gastritis: a multicenter prospective study in China

“…Some reports have shown that individuals with congenital or acquired immunodeficiencies carry an increased susceptibility to gastric cancer which is theorized to relate to a background of altered immune surveillance and CAG. Unlike classic autoimmune body gastritis, it may present a distinctive type of atrophic pangastritis [35]. Although, our questionnaire contains a variety of combined diseases, not a single disease, including immunodeficiency diseases, is significantly related to CAG.…”

Pathology of non-Helicobacter pylori gastritis: extending the histopathologic horizons

“…The inflammation is not confined merely to the body/fundus region [75]. The infiltrate is mixed, composed of polyclonal plasma cells and numerous CD3?…”

“…The mechanisms mediating the changes are not fully established, but anti-parietal cell and/or anti-intrinsic factor antibodies have been detected [72][73][74][75].…”

“…The infiltrate is mixed, composed of polyclonal plasma cells and numerous CD3? T lymphocytes, with variable numbers of CD4 and CD8 positive T cells [75]. Notably, secondary neuroendocrine cell hyperplasia is absent, suggesting that all gastric cellular phenotypes are equally damaged [75].…”

Atrophic Body Gastritis: A Challenge for the Presumptive Endoscopic and Histologic Diagnosis of Autoimmune Gastritis

“…This pathological condition was not associated with H. pylori or endocrine cell hyperplasia and may affect a especific group of patients with a particular type of autoimmune gastritis. Hypothetically, these patients would develop the production of antibodies directed against multiple cell lines of the gastric mucosa (Jevremovic, 2006).…”