Collagenous sprue: a case report and literature review.

Abstract

Collagenous sprue (CS) is a rare disease of the small bowel that was first described in 1947 and histologically confirmed in 1970.1,2 This disease is characterized by complete atrophy of mucosal villi and excessive subepithelial collagen deposition that may replace the crypts. Other features that are distinctive, but not pathognomonic of CS, are the expansion of the lamina propria by subcryptal inflammation and a mucosa that appears thin.3 Some authors who doubt the existence and clinical significance of CS as a distinctive histologic picture have confused the prominent basement membrane seen in some patients with celiac disease (CD) with CS, but the degree of subepithelial collagen deposition is distinguishable from that of CD.3 That the subepithelial collagenous thickening can be variable and patchy along the small bowel also makes the diagnosis difficult.2,4,5 Aiding in the distinction between CS and CD is the response to gluten-free diet. As a general rule, the collagenous deposits in CD regress under gluten-free diet, whereas those of CS usually do not.6 This, however, is not true in all cases. Patients may or may not initially respond to a gluten-free diet.2,4 Thus far, the etiology of CS is not clear. Patients with documented CS have a clinical course that is usually, but not uniformly, dismal.7 This case report describes a young woman with intractable nausea, vomiting, diarrhea, and severe weight loss of 4 months’ duration who was diagnosed with CS of the small bowel and, shortly thereafter, collagenous colitis. The patient was unresponsive to a high dose of steroids and a trial of gluten-free diet and is currently on home parenteral nutrition (HPN). Our literature search revealed only 15 reported cases of collagen deposition involving the small and large intestines.