The Coexistence of Cystic Fibrosis and Celiac Disease

Abstract
Two children with cystic fibrosis (CF) who subsequently developed celiac disease (CD) are described. Since organ culture of intestinal mucosa was used to establish an in vitro model for the study of CD, this opportunity was used to determine whether duodenal mucosa obtained from each of the 2 patients and their immediate families differed in its organ culture behavior from mucosa obtained from patients with CD alone. As specific HL-A [histocompatibility antigen] types are associated with CD, HL-A typing was used to determine whether the 2 patients with CF-CD differed genetically from patients with CD alone. One of the patients was HL-A8, the most common type associated with CD; the other was HL-A12, as are many of the non-HL-A8 celiac patients. The response in organ culture of the mucosa of the 2 patients was the same as the response in organ culture of the mucosa from patients with CD alone. These and other data suggest that CD occurring in patients with CF is no different than CD occurring alone.