Seronegative autoimmune enteropathy with duodenal sparing and colonic clues in an adult female.

Document Type

Article

Publication Date

4-2021

Publication Title

Clin J Gastroenterol

Abstract

Autoimmune enteropathy (AIE) is a rare immune disorder of the gut seldom found in adults and characterized by uncontrollable diarrhea resulting in malabsorption. While AIE is known to be pan-enteric, virtually all cases have presented with altered duodenal histology following known patterns with or without macroscopic change. We describe a unique case of seronegative AIE lacking typical duodenal manifestations in a 43-year-old female. To our knowledge, this is the first report of AIE lacking usual duodenal histologic changes, which resulted in missed diagnosis for years. Ultimately, crypt epithelial apoptosis, mononuclear inflammation of the lamina propria, and goblet cell loss of intestinal mucosa besides the duodenum clinched the diagnosis of AIE. Colonic histologic abnormalities consistent with AIE in the setting of diarrhea with malnutrition despite duodenal sparing should prompt suspicion for AIE given the pan-enteric nature of this disease.

Volume

14

Issue

2

First Page

546

Last Page

550

DOI

10.1007/s12328-020-01336-9

ISSN

1865-7265

PubMed ID

33630282

Link to Full Text

Share

COinS