Studies of celiac disease. I. The apparent identical and specific nature of the duodenal and proximal jejunal lesion in celiac disease and idiopathic sprue.

Abstract

Summary In order to investigate systematically the possible relationship between celiac disease and idiopathic sprue, 230 suction biopsies of the duodenum and proximal jejunum were taken from 26 patients with celiac disease, 16 patients with idiopathic sprue, and 73 controls (3 months to 77 years old). The controls consisted of normals, patients with other types of malabsorption, and persons with situations which might theoretically cause an intestinal mucosal abnormality. A small diameter, highly flexible instrument was developed which enabled biopsies to be taken with equal facility from infants and adults. The absorptive surface of the fresh, unsectioned specimen was studied microscopically during fixation. The controls were covered with delicate villi, each of which contained numerous villous capillaries, whereas the abnormal specimens were more or less bald and had a disorganized, sparse vasculature. These findings were confirmed by the appearance of the tissue sections. The most constant diagnostic feature was the reduction in epithelial surface, with varying degrees of blunting or loss of villi. Infiltration of the lamina propria with round cells and abnormalities of the epithelial cells at the luminal surface were frequent but not invariable findings. Perfectly oriented serial sections were evaluated histologically by three observers independently and without clinical foreknowledge. A fourth observer evaluated them by a quantitative technique. This histologic interpretation and obj ective quantitativn agreed with the proved clinical diagnosis in every instance. The appearance of the proximal intestinal abnormality in celiac disease and idiopathic sprue is identical. The celiac-sprue lesion is apparently specific for it was not found in any of the controls. The normalcy of the ileal mucosa was demonstrated in 1 adult celiac patient and 1 idiopathic sprue patient, despite the fact that the proximal j ej unal biopsies in both cases were severely abnormal. The characteristic lesion in the duodenum and proximal jejunum in celiac disease and idiopathic sprue is probably pathognomonic.