Autoimmune Enteropathy in an Adult with Autoimmune Multisystemic Involvement

Abstract

We describe the case of a 58-year-old woman with autoimmune enteropathy associated with thyroiditis, gastritis, transitory neutropenia, sicca syndrome and severe axonal polyneuropathy of autoimmune origin. Enterocyte autoantibodies were not detected. However, predisposition to autoimmune disease was indicated by the presence of high titres of anti-gastric parietal cell, anti-thyroglobulin, anti-thyroid peroxidase and anti-neutrophil antibodies. CD4+ and CD8+ lymphocytes were equally distributed in the lamina propria of the small intestine, but CD8+ cells were highly represented among intraepithelial lymphocytes.

Autoimmune Enteropathy Associated with Cessation of Interferon-Alpha Therapy in Chronic Hepatitis C

“…The majority of patients with AIE have other underlying autoimmune diseases [12][13][14]. Our patient has a history of ulcerative colitis with a total colectomy 30 years prior to this presentation.…”

Enteropathy with loss of enteroendocrine and Paneth cells in a patient with immune dysregulation: a case of adult autoimmune enteropathy

“…Clinically, he was considered to have the IPEX syndrome. Autoimmune enteropathies are uncommon in young children and are very rare in adults, with only 7 identifiable cases in the literature (discussed in Leon et al [2] and in Biagi and Corazza's commentary on Daum et al [3]) [3][4][5][6] and one presentation (S.C. Abrahams, Gastrointestinal Specialty Conference, United States and Canadian Academy of Pathology 2005 Annual Meeting).…”

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

“…Parietal cell and/or intrinsic factor antibodies have been reported in a number of patients with autoimmune enteropathy, 3,5,17,18 but only rarely are gastric biopsies in these patients described. Some reports include ''mild lymphocytic infiltration'' of the stomach, 20 ''necrosis of the crypts,'' 2 ''slight increase of CD3 + CD8 + intraepithelial lymphocytes,'' 19 ''mild focal antral gastritis,'' 22 and ''dense lymphoid infiltration of the lamina propria.''…”

“…The first, a 58-year-old woman who also suffered from autoimmune thyroiditis, sicca syndrome, and autoimmune axonal polyneuropathy, had high-titer parietal cell antibodies and endoscopic atrophy involving the entire stomach. 3 Body biopsies were not described, but antral biopsies were said to show antral gland atrophy, lymphocytic gastritis, and more inflammatory cells near the gastric pits. The second patient, a 59-yearold man, underwent gastric resection which was reported to show atrophy, intestinal metaplasia, and complete loss of specialized glands throughout the stomach.…”