A Rare Case of Chronic Diarrhea and Weight Loss
Authors: Huiqin He, Sibin Mei, Leimin Sun
Journal: Clinical Gastroenterology and Hepatology (2016)

A 44-year-old man presented with chronic diarrhea. Over the past 5 years, the patient experienced intermittent bouts of watery diarrhea associated with fatigue and a 15-kg weight loss. Past medical history included a perianal fistula, which required surgery 5 years ago. He denied use of antibiotics or nonsteroidal anti-inflammatory drugs before the onset of his symptoms. On presentation, vital signs were within the normal range. Physical examination showed a pale appearance, hyperactive bowel sounds, and mild lower-extremity edema. Laboratory data were significant for a hemoglobin level of 7.1 g/dL, potassium level of 2.6 mmol/L, serum albumin level of 2.4 g/dL, C-reactive protein level of 16.8 mg/L, and an erythrocyte sedimentation rate of 58 mm/h. Other evaluations including tissue transglutaminase IgA, anti-endomysial IgA, autoantibody to nuclear antigen, perinuclear antineutrophil cytoplasmic antibody, anti-Saccharomyces cerevisiae antibody, and T cell spot Tuberculosis Test were unremarkable. Stool studies including bacterial culture, stool ova and parasite, and stool rotavirus culture showed no infectious source. An abdominal computed tomography study with contrast showed wall thickening in parts of the small and large intestines with multiple enlarged mesenteric lymph nodes (Figure A). Gastroscopy showed diffuse scalloping of duodenal folds (Figure B), and colonoscopy showed areas of healed longitudinal ulcers in the terminal ileum and ascending colon (Figure C) which were examined by biopsy. Capsule endoscopy was performed and showed numerous ulcers throughout the small intestine (Figure D). H&E stain showed marked blunting of villous architecture, chronic inflammatory cell infiltrate within the lamina propria, and intraepithelial infiltration by lymphocytes and polymorphs in the duodenum as well as the terminal ileum (Figures E, F, and G). Masson stain showed a diffusely thickened subepithelial collagenous band measuring up to 30 um in both the duodenum and terminal ileum, consistent with collagenous sprue (Figures F, G, and H). The patient initially was treated with a gluten-free diet for 2 weeks during hospitalization without relief of his symptoms. After receiving intravenous and oral corticosteroids, his diarrhea resolved. First described in 1947 by Schein,1Schein J. Syndrome on non tropical sprue with hitherto undescribed lesions of the intestine.Gastroenterology. 1947; 8: 438-460Google Scholar collagenous sprue is a rare malabsorptive disorder characterized by subepithelial collagen deposition, forming a band greater than 10-mm diameter in thickness in the small intestine. Classic presentation of collagenous sprue includes chronic diarrhea, insidious weight loss, and malabsorption. Although the etiology is unclear, inflammation, autoimmunity, dietary, environmental, and even resident commensal bacteria may contribute to the pathogenesis of collagenous sprue. Collagenous sprue is differentiated from celiac disease by the presence of a collagenous band greater than 5 mm,2Vakiani E. Arguelles-Grande C. Mansukhani M.M. et al.Collagenous sprue is not always associated with dismal outcomes: a clinicopathological study of 19 patients.Mod Pathol. 2010; 23: 12-26Google Scholar an absence of celiac antibody titers, and a lack of response to a gluten-free diet. Observational studies have suggested that glucocorticoids and immunomodulators may delay the progression of disease, especially when treatment is initiated in the early stages2Vakiani E. Arguelles-Grande C. Mansukhani M.M. et al.Collagenous sprue is not always associated with dismal outcomes: a clinicopathological study of 19 patients.Mod Pathol. 2010; 23: 12-26Google Scholar before the development of intestinal fibrosis.3Latella G. Sferra R. Speca S. et al.Can we prevent, reduce or reverse intestinal fibrosis in IBD?.Eur Rev Med Pharmacol Sci. 2013; 17: 1283-1304Google Scholar The authors would like to acknowledge Dr John Kim from Loma Linda University Medical Center for his assistance with the manuscript preparation.