Immunofluorescent Studies in Adult Celiac Disease*

Abstract

The etiology of childhood and adult celiac dis- ease is not known. It has been established, how- ever, that gluten, a complex heterogeneous pro- tein in wheat and other cereal grains, plays a significant role in the pathogenesis of the disease, and that the harmful factor resides in gliadin, the al- cohol soluble fraction of gluten (1-4). Treatment of celiac disease with a gluten-free diet results in clinical and biochemical improvement usually ac- companied by a decrease in the severity of the pathologic lesion in the proximal small intestinal mucosa. The ingestion of gluten by patients in remission on a gluten-free diet causes an exacerbation of symptoms, accompanied by malabsorption and acute inflammatory changes in the proximal jejunal mucosa (5). Furthermore, direct instil- lation of flour or gluten into the histologically normal distal small intestine of patients with adult celiac disease produces similar acute mucosal in- flammation (6), whereas the intestinal mucosa of a normal subject remains unchanged. The toxicity of gluten and gliadin is retained by mixtures of straight chain polypeptides that result from enzy- matic and chemical digestion of the protein (7-9). Further digestion of these toxic peptides by fresh extracts of hog small intestinal mucosa or by crude papain renders them inactive (7, 8). De- amidation or complete acid hydrolysis of gliadin also renders it inactive (1).