Cystic Fibrosis and Celiac Disease: Both Can Occur Together

Cystic fibrosis (CF) is the most common life-threatening autosomal recessive genetic disease in Caucasians and is noted to have an incidence of 1:3,200 in the US. Although CF is associated with progressive pulmonary disease, it also predisposes patients to malnutrition due to a multitude of factors, including exocrine pancreatic insufficiency, increased caloric requirements associated with impaired lung function, and potential cellular defects affecting energy utilization. Celiac disease also can cause symptoms of malabsorption, including diarrhea, abdominal distention and failure to thrive. Screening serum antibody tests have an excellent sensitivity and specificity in celiac disease screening; however, duodenal biopsies obtained by esophagogastroduodenoscopy are necessary to confirm this diagnosis. As celiac disease is relatively common, there is a risk that it can occur in children with CF. The occurrence of these 2 diseases concurrently can complicate determining malnutrition etiology in such patients. Here, Pohl et al examine the case of 2 patients who were diagnosed with CF and celiac disease.