Autoimmune enteropathy with anti-goblet cell antibodies

doi:10.1016/0046-8177(95)90283-X

Human Pathology

Volume 26, Issue 10, October 1995, Pages 1162-1168

https://doi.org/10.1016/0046-8177(95)90283-X Get rights and content

Abstract

A 9-year-old boy with a 5-year history of severe protracted diarrhea requiring home parenteral nutrition and a 1 year history of abnormal liver function tests was admitted for duodenal, rectal, and liver biopsy. Duodenal biopsy results showed mild villus blunting, a mild lymphocytic infiltrate, and absent goblet cells. Paneth cells and endocrine cells could not be identified. Review of several previous biopsies showed an almost total absence of goblet cells by light microscopy. Anti-goblet cell antibodies of the immunoglobulin (Ig)G class were shown by immunofluorescence with a titer of 1:512. Histological examination of rectal mucosa also showed a total lack of goblet cells, orderly surface epithelial cells, and infiltration of the colonic crypts by lymphocytes. Immunoperoxidase staining of rectal mucosa showed increased numbers of lymphocytes with an excess of CD3+, CD45RO+ T cells, and increased numbers of B cells labeling with B1 and L26. Increased numbers of CD25+ (activated) lymphocytes were also observed. HLA/DR expression was striking and observed in both the crypt and surface enterocytes, as well as in the lamina propria. Immunological assessment of the patient showed an inverted CD4/CD8 ratio and IgA/IgG₄ deficiency. The liver biopsy and radiological investigation were in keeping with chronic sclerosing cholangitis. Although a slight and transient improvement in histological appearances was observed with prednisolone there was no significant improvement of diarrhea. Trials of azothiaprine and oral cyclosporin did not result in clinical or histological improvement.

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The antibodies are predominantly immunoglobulin (Ig) G, and have been described as complement fixing [104], although IgM and IgA have also been described [106,108]. Antibodies reacting against mucus or goblet cells have also been described, and intestinal biopsies in these cases have shown a marked depletion of goblet cells (see Fig. 6D) [109,110]. The target of the antienterocyte antibodies is still a matter of investigation, although a 75-kDa antigen reactive with autoantibody in the sera of patients with the IPEX syndrome has been reported [111].
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Case studyAutoimmune enteropathy with anti-goblet cell antibodies

Abstract

J Pediatr

Gastroenterology

J Pediatr

Protracted diarrhea of infancy: Evidence in support of an autoimmune variant

BMJ

Selective IgA deficiency associated with total villous atrophy of the small intestine and an organ-specific antiepithelial cell antibody

J Immunol

Chronic protracted diarrhea and jejunal atrophy in an infant: Cimetidine-associated stimulation of jejunal mucosal growth

Dig Dis Sci

Flat small intestinal mucosa and autoantibodies against the gut epithelium

J Pediatr Gastroenterol Nutr

Specific autoantibodies to gut epithelium in two infants with severe protracted diarrhoea

J Pediatr Gastroenterol Nutr

Autoimmunity in diarrhoeal disease

J Pediatr Gastroenterol Nutr

Case study
Autoimmune enteropathy with anti-goblet cell antibodies