Collagenous Sprue: A Case of Gastric and Colonic Involvement

Introduction: Collagenous sprue (CS), a rare disorder primarily aff ecting the small bowel and strongly associated with celiac disease, has rarely been reported. Patients historically present with persistent diarrhea, progressive weight loss and severe malabsorption causing multiple nutrient defi ciencies. While the disease fails to respond to a gluten free diet, it carries the burden of celiac disease associated small bowel malignancy. Here, we present the case of an otherwise asymptomatic patient who was noted to have collagenous gastroenteritis, with involvement of not only the small bowel, but the stomach and large intestines. Case: A 32-year-old male with a longstanding history of asymptomatic GERD and idiopathic gastroparesis presented electively for open access endoscopy. Endoscopic evaluation revealed nodular gastropathy, which histologically was compatible with collagenous sprue. Th is was confi rmed by a second pathologist at a tertiary referral center. A subsequent colonoscopy for evaluation of a change in bowel habits was unremarkable endoscopically, but random biopsies did reveal excess intraepithelial lymphocytes and architectural distortion with varying crypt size and spacing. Th e patient went on to have an upper EUS which revealed diff use wall thickening in the cardia, body and fundus with a maximal wall thickness of 6.1 mm noted, most notably in the superfi cial and deep mucosa. Th e patient has remained asymptomatic and continues to follow up as an outpatient. Discussion: Th e etiology and natural history of collagenous sprue has not been elucidated. Th e disease was fi st described in 1947 and histologically confi rmed in 1970. Some reports have estimated that the disease remains asymptomatic for decades. Histologically, atrophy of mucosal villi and excessive subepithelial collagen deposition that may replace crypts is noted. While histologically and clinically similar to celiac disease, CS does not respond to a gluten free diet. Management of CS remains diffi cult due to lack of long term follow up data. Hoerver proposed treatment regimens consist of total parenteral nutrition coupled with immunosuppressive therapy with cortocosteroids and biologic agents. Unfortunately, no clinical marker or pathological hallmark appears to predict which patients might respond to treatment. Patients die from lymphoma of the small bowel and diarrhea, as well as severe malnutrition. Here, we present a case of CS, a rare disease involving most of the gastrointestinal tract, including the stomach, small bowel and colon.