Is Collagenous Sprue Really a Combination of Collagenous Colitis and Celiac Sprue?

Abstract

Collagenous sprue is a rare condition of the small intestinal associated with severe diarrhea, malabsorption and weight loss. We report a case of collagenous sprue with overlapping features of collagenous colitis and celiac disease histologically but yet negative serologies and genetic testing for celiac disease. An 82 year old Caucasian female with a past medical history of coronary artery and ichemic stroke presented to the clinic complaining of severe diarrhea, nausea and weight loss over the past year. The diarrhea was watery and typically post prandial. She had a weight loss of more than 30 lbs in the past year. Physical examination was normal. Celiac serologies were negative. Genetic testing for HLA-DQ2 and HLA DQ-8 was negative. Colonoscopy revealed a grossly normal appearing mucosa. Biopsies from the terminal ileum showed partial villous atrophy, intraepithelial lymphocytosis and mild collagenous table thickening and biopsies from the colon revealed collagenous colitis. She had no significant improvement of her symptoms with a gluten free diet. Other dietary modifications were done and fiber supplementation as well as antidiarrheal medications were added without any significant improvement of diarrhea. She was then initiated on budesonide 9 mg daily which improved the frequency and consistency of the diarrhea. Collagenous sprue is characterized microscopically by small intestinal villi and crypt atrophy as well as a subepithelial collagen deposit that is thicker than 12 μm. It is histologically similar to celiac disease with intestinal villous and crypt atrophy as well as microscopic colitis, with increased subepithelial collagen deposition. Although it is similar to celiac disease, the serologies and genetic testing are negative and a gluten free diet does not help to resolve symptoms. In the evaluation of diarrhea, generally gastroenterologists obtain duodenal biopsies to rule out celiac disease and random colon biopsies in cases of normal colonoscopy to rule out microscopic colitis. We emphasize the importance of terminal ileal intubation and biopsies in order to establish the diagnosis of collagenous sprue. Systemic or luminal steroids are the treatment of choice once the diagnosis is established.