Intestinal lymphangiectasia associated with deep vein thrombosis. First results in treatment with octreotide

Abstract

Background & Aim: Intolerance to dietary gluten in celiac disease has traditionally been considered to persist lifelong, although the possibility of transient intolerance has been proposed. Whether patients can 'grow out' of true celiac disease and the frequency with which this may occur, is unclear. We performed a detailed review of clinical, serologic and histologic evidence for gluten intolerance in adult subjects over two decades after they were diagnosed as celiac in childhood. Methods: 103 patients diagnosed with celiac disease in childhood in a pediatric unit with special interest in the condition were contacted and reviewed 23-39 years after their original diagnosis. Original diagnostic criteria used at the time were histologic evidence of celiac disease above the age of 24 months, a clinical response to a gluten-free diet, and proven clinical and histologic response to gluten exclusion. After reassessment in adulthood, we offered endoscopic distal duodenal re-biopsy to patients who were not adhering to a gluten-free diet and in whom endomysial antibodies were negative. Results: Six patients have been identified who initially fulfilled traditional criteria for celiac disease but now have no evidence for gluten sensitivity on reassessment in adulthood, while on a full gluten-containing diet. Each patient presented with malabsorption within the first 2 years of life and had a subsequent jejunal biopsy that showed moderate (l/6) to severe villous atrophy (5/6). Evidence for malabsorption resolved on a gluten-free diet, and each had a subsequent small bowel biopsy 1-11 years later which, although improved, was still abnormal (partial villous atrophy in 3 cases and increased intraepithelial lymphocytes in the other 3 cases). In adulthood, while taking a full gluten-containing diet, none of the patients now have evidence of malabsorption, serum endomysial antibodies are negative, and distal duodenal biopsies have been normal in all. Conclusion: The observations provide evidence in favor of the occurrence of true transient gluten intolerance in some patients with apparent classical childhood celiac disease. That each patient had two abnormal biopsies at different intervals during childhood (albeit with improvement on gluten restriction) supports the validity of the original diagnosis and suggests that the initial abnormality was not due to one of the mimics of celiac disease in childhood. The results also support the view that the requirement for rigid adherence to a gluten-free diet may diminish and resolve in adulthood in a proportion of patients.