Early ReportAutoimmune enteropathy and villous atrophy in adults
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)
- et al.
Celiac sprue and refractory sprue
Gastroenterology
(1978) Sprue by any other name
Gastroenterology
(1970)- et al.
T-lymphocyte dysfunctions occurring together with apical gut epithelial cell autoantibodies
Gastroenterology
(1991) - et al.
Autoantibodies against gut epithelium in a child with small intestinal enteropathy
Lancet
(1982) - et al.
HLA product expression and lymphocyte subpopulations in jejunum biopsies of children with idiopathic protracted diarrhoea and enterocyte autoantibodies
J Autoimmun
(1988) - et al.
Classification of intractable diarrhea in infancy using clinical and immunohistological criteria
Gastroenterology
(1990) - et al.
Gut epithelial cell autoantibodies in AIDS pathogenesis
Lancet
(1993) - et al.
All that flattens is not “sprue”
Gastroenterology
(1979) Celiac sprue
N Engl J Med
(1991)- et al.
Quantitative assessment of the mucosal architecture of jejunal biopsy specimens: a comparison between linear measurement, stereology, and computer aided microscopy
J Clin Pathol
(1985)
Intraepithelial lymphocytes of the small intestine
Gut
Response of the jejunal mucosa in adult coeliac disease to oral prednisolone
Gut
Cited by (142)
Adult autoimmune enteropathy in autoimmune hepatitis patient. Case report and literature review
2021, Clinics and Research in Hepatology and GastroenterologyImmune-mediated enteropathies: From bench to bedside
2021, Journal of AutoimmunitySeronegative villous atrophy
2021, Coeliac Disease and Gluten-Related DisordersHedgehog-Activated Fat4 and PCP Pathways Mediate Mesenchymal Cell Clustering and Villus Formation in Gut Development
2020, Developmental CellCitation Excerpt :This approach could be extended to study human villification. Of note, patients with celiac disease commonly suffer from malnutrition due to villus atrophy, as apoptosis of enterocytes results in a flattened epithelium with markedly reduced surface area (Corazza et al., 1997). A better understanding of mesenchymal PCP mechanisms in human villification could form a basis for new therapies that promote villus regeneration.
Clinical manifestations and gastrointestinal pathology in 40 patients with autoimmune enteropathy
2019, Clinical Immunology