Elsevier

The Lancet

Volume 350, Issue 9071, 12 July 1997, Pages 106-109
The Lancet

Early Report
Autoimmune enteropathy and villous atrophy in adults

https://doi.org/10.1016/S0140-6736(97)01042-8Get rights and content

Summary

Background

Autoimmune enteropathy is a condition described in children and characterised by villous atrophy, which is unresponsive to any dietary restrictions, and by the presence of enterocyte autoantibodies. We report two adult patients who fulfilled all the criteria for the diagnosis of this disorder.

Methods

Over the past 5 years we have seen four adult patients (all women, median age 51·5 [range 38–64] years) with subtotal villous atrophy, which was unresponsive to a gluten-free diet. The patients were HLA-DQ2 positive. IgA antigliadin and antiendomysial antibodies were not found in any of the patients. We did an indirect immunofluorescence search for enterocyte autoantibodies on monkey jejunum and for other autoantibodies for all four patients.

Findings

Of the four patients, two were positive for enterocyte autoantibodies and one of these two patients was positive for antiactin, antiparietal cell, and antithyroid microsomal autoantibodies.

Interpretation

To the best of our knowledge the two patients affected by severe enteropathy, who had never responded to any exclusion diet, and who were positive for enterocyte autoantibodies are the first cases of autoimmune enteropathy described in adults. We propose that adult patients whose disorders are unresponsive to a gluten-free diet should be tested for enterocyte autoantibodies.

Introduction

The key to the diagnosis of coeliac disease is the unequivocal demonstration of histological and clinical improvement after the patient is put on a gluten-free diet.1 However, in a minority of adult patients jejunal villous atrophy and malabsorption symptoms are unresolved despite strict adherence to a gluten-free diet.2 Since most of these patients are resistant to dietary therapy from its inception, unclassified coeliac disease is the preferred term for this disorder.1, 3

Here we describe four adult patients with small-bowel villous atrophy attributed to unclassified coeliac disease.

Section snippets

Case reports

During the past 5 years, we have seen four women with features of unclassified coeliac disease (table 1). They all proved to be HLA-DQ2 positive. Intestinal biopsy samples were taken because of severe malabsorption symptoms. Three of them (patients one, two, and three) were referred to us because of a lack of histological and clinical response to gluten withdrawal from the diet and were therefore already on gluten-free diet at the time of our first observation. Adherence to the gluten-free diet

Results

The serological pattern of our patients is shown in table 2. Patients one and two were positive for the presence of enterocyte autoantibodies. Only patients three and four were positive for IgG antigliadin antibodies, but IgA antigliadin and antiendomysial antibodies were absent in all four patients. Enterocyte autoantibodies belonged to IgA and IgG classes, whereas the test for IgM enterocyte autoantibodies was always negative. Only patient two, who had the highest enterocyte autoantibody

Discussion

We report four adult patients with villous atrophy initially diagnosed as unclassified coeliac disease. It was subsequently clear that two of these patients (one and two) fulfilled all the criteria proposed by Unsworth and Walker-Smith10 for the diagnosis of autoimmune enteropathy. In patient two predisposition to autoimmune disease, as well as the presence of circulating enterocyte autoantibodies, was also indicated by the presence of other organ-specific and non-organ-specific autoantibodies,

References (24)

  • A Ferguson

    Intraepithelial lymphocytes of the small intestine

    Gut

    (1977)
  • AJ Wall et al.

    Response of the jejunal mucosa in adult coeliac disease to oral prednisolone

    Gut

    (1970)
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