Collagenous Enteritis – An Alternative Cause of Malabsorptive Enteropathy

Document Type : Case Report

Authors

Department of General Internal Medicine, Cedars-Sinai Medical Center

10.30476/acrr.2021.91296.1100

Abstract

Introduction: Collagenous Enteritis (CE) is a less common cause of enteropathy presenting with malabsorption as its cardinal symptom. While historically considered a resistant form of celiac disease, newer evidence supports the interaction between genetic predispositions and environmental triggers as the pathophysiologic basis of CE.

Case Presentation: Herein we present the case of a 40-year-old woman with two-year history of diarrhea and 32 kg weight loss who was incorrectly diagnosed with celiac disease with refractory symptoms despite adherence to a gluten-free diet. Extensive infectious, inflammatory, secretory, autoimmune workup did not reveal an underlying etiology. Endoscopic evidence of severe villous blunting and biopsy with characteristic patchy enlarged subepithelial collagen layer solidified a diagnosis of CE. Treatment with steroids resulted in resolution of malabsorptive symptoms and gradual weight gain.

Conclusion: This case highlights the importance of considering CE in patients presenting with malabsorption especially given significant clinical overlap with other malasbsorptive conditions as well as its therapeutic implications.

Keywords


1.            Schein J. Syndrome on non tropical sprue with hitherto undescribed lesions of the intestine. Gastroenterology. 1947;8(4):438-460.
2.            Weinstein WM, Saunders DR, Tytgat GN, Rubin CE. Collagenous sprue--an unrecognized type of malabsorption. N Engl J Med. 1970;283(24):1297-1301.
3.            Cuoco L, Villanacci V, Salvagnini M, Bassotti G. Collagenous sprue with associated features of refractory celiac disease. Rev Esp Enferm Dig. 2012;104(4):223-225.
4.            Lan N, Shen B, Yuan L, Liu X. Comparison of clinical features, treatment, and outcomes of collagenous sprue, celiac disease, and collagenous colitis. J Gastroenterol Hepatol. 2017;32(1):120-127.
5.            Karakus E, Ekinci O, Kirsaclioglu CT, Ozaydin E, Atakan C, Dursun A. A rare cause of protein losing enteropathy: collagenous sprue. Fetal Pediatr Pathol. 2015;34(2):133-135.
6.            Zhao X, Johnson RL. Collagenous sprue: a rare, severe small-bowel malabsorptive disorder. Arch Pathol Lab Med. 2011;135(6):803-809.
7.            Holdstock DJ, Oleesky S. Successful treatment of collagenous sprue with combination of prednisolone and gluten-free diet. Postgrad Med J. 1973;49(575):664-667.
8.            Robert ME. Gluten sensitive enteropathy and other causes of small intestinal lymphocytosis. Semin Diagn Pathol. 2005;22(4):284-294.
9.            Daniels JA, Lederman HM, Maitra A, Montgomery EA. Gastrointestinal tract pathology in patients with common variable immunodeficiency (CVID): a clinicopathologic study and review. The American journal of surgical pathology. 2007;31(12):1800-1812.
10.          Freeman HJ, Berean KW. Resolution of paraneoplastic collagenous enterocolitis after resection of colon cancer. Can J Gastroenterol. 2006;20(5):357-360.
11.          Robert ME, Ament ME, Weinstein WM. The histologic spectrum and clinical outcome of refractory and unclassified sprue. The American journal of surgical pathology. 2000;24(5):676-687.
12.          Vakiani E, Arguelles-Grande C, Mansukhani MM, et al. Collagenous sprue is not always associated with dismal outcomes: a clinicopathological study of 19 patients. Mod Pathol. 2010;23(1):12-26.
13.          Cellier C, Delabesse E, Helmer C, et al. Refractory sprue, coeliac disease, and enteropathy-associated T-cell lymphoma. French Coeliac Disease Study Group. Lancet. 2000;356(9225):203-208.
14.          Nielsen OH, Riis LB, Danese S, Bojesen RD, Soendergaard C. Proximal collagenous gastroenteritides: clinical management. A systematic review. Ann Med. 2014;46(5):311-317.
15.          Yau AH, Xiong W, Ko HH. Collagenous enterocolitis manifesting as watery diarrhoea and iron-deficiency anaemia. BMJ Case Rep. 2015;2015.
16.          Kung VL, Liu TC, Ma C. Collagenous Enteritis is Unlikely a Form of Aggressive Celiac Disease Despite Sharing HLA-DQ2/DQ8 Genotypes. The American journal of surgical pathology. 2018;42(4):545-552.
17.          Desruisseaux C, Bensoussan M, Desilets E, et al. Adding Water to the Mill: Olmesartan-Induced Collagenous Sprue-A Case Report and Brief Literature Review. Can J Gastroenterol Hepatol. 2016;2016:4837270.
18.          Nielsen JA, Steephen A, Lewin M. Angiotensin-II inhibitor (olmesartan)-induced collagenous sprue with resolution following discontinuation of drug. World J Gastroenterol. 2013;19(40):6928-6930.
19.          Vasant DH, Hayes S, Bucknall R, Lal S. Clinical and histological resolution of collagenous sprue following gluten-free diet and discontinuation of non-steroidal anti-inflammatory drugs (NSAIDs). BMJ Case Rep. 2013;2013.
20.          van Gils T, van de Donk T, Bouma G, van Delft F, Neefjes-Borst EA, Mulder CJ. The first cases of collagenous sprue successfully treated with thioguanine. BMJ Open Gastroenterol. 2016;3(1):e000099.