Skip to main content Accessibility help

We use cookies to distinguish you from other users and to provide you with a better experience on our websites. Close this message to accept cookies or find out how to manage your cookie settings.

Close cookie message
×
Hostname: page-component-cd9895bd7-gxg78 Total loading time: 0 Render date: 2024-12-27T10:38:27.629Z Has data issue: false hasContentIssue false

Chapter 15 - Coeliac Disease

Published online by Cambridge University Press:  06 June 2020

By
Colan M. Ho-Yen  and
Fuju Chang
Edited by
Roger M. Feakins
Roger M. Feakins
Affiliation:
Royal Free London NHS Foundation Trust, London, UK
Get access

Summary

A common clinical indication for duodenal biopsy is the exclusion of coeliac disease / gluten sensitive enteropathy. However, a variety of inflammatory and infectious disorders may affect the duodenum, some of which are associated with subtle endoscopic findings. The indications for duodenal biopsy are often the same as the broader indications for upper gastrointestinal endoscopy and include chronic dyspepsia, unexplained anaemia, abdominal pain, bloating, nausea, and diarrhoea. Endoscopic findings associated with inflammatory duodenal biopsies range from normal-appearing duodenal mucosa to mild hyperaemia and congestion of the duodenal bulb to erosions, severe congestion, mucosal haemorrhage, mucosal contact bleeding, and luminal narrowing.

Keywords

duodenumbiopsyinflammationulcerduodenitismetaplasiaheterotopiainfection,giardiasisWhipple’s disease
Type
Chapter
Information
Non-Neoplastic Pathology of the Gastrointestinal Tract
A Practical Guide to Biopsy Diagnosis
 , pp. 215 - 235
Publisher: Cambridge University Press
Print publication year: 2020

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Ciclitira, PJ, King, AL, Fraser, JS. AGA technical review on celiac sprue. American Gastroenterological Association. Gastroenterology. 2001;120:1526–40.Google Scholar
Brown, IS, Smith, J, Rosty, C. Gastrointestinal pathology in celiac disease: a case series of 150 consecutive newly diagnosed patients. Am J Clin Pathol. 2012;138(1):42–9. DOI: 10.1309/AJCPE89ZPVJTSPWL.Google Scholar
van Heel, DA, West, J. Recent advances in coeliac disease. Gut. 2006;55;1037–46.CrossRefGoogle ScholarPubMed
Clemente, MG, De Virgiliis, S, Kang, JS, et al. Early effects of gliadin on enterocyte intracellular signalling involved in intestinal barrier function. Gut. 2003;52:218–23.Google Scholar
Fasano, A, Not, T, Wang, W, et al. Zonulin, a newly discovered modulator of intestinal permeability, and its expression in coeliac disease. Lancet. 2000;355:1518–19.Google Scholar
Craig, D, Robins, G, Howdle, PD. Advances in celiac disease. Curr Opin Gastroenterol. 2007;23:142–8.Google Scholar
Rostom, A, Murray, JA, Kagnoff, MF. American Gastroenterological Association (AGA) Institute technical review on the diagnosis and management of celiac disease. Gastroenterology. 2006;131:1981–2002.Google Scholar
Koning, F, Schuppan, D, Cerf-Bensussan, N, Sollid, LM. Pathomechanisms in celiac disease. Best Pract Res Clin Gastroenterol. 2005;19:373–87.Google Scholar
Hüe, S, Mention, JJ, Monteiro, RC, et al. A direct role for NKG2D/MICA interaction in villous atrophy during celiac disease. Immunity. 2004;21(3):367–77.Google Scholar
Kelly, CP, Bai, JC, Liu, E, Leffler, DA. Advances in diagnosis and management of celiac disease. Gastroenterology. 2015;148(6):1175–86. DOI:10.1053/j.gastro.2015.01.044. Epub 2015 Feb 3.CrossRefGoogle ScholarPubMed
Ludvigsson, JF, Bai, JC, Biagi, F, et al. BSG Coeliac Disease Guidelines Development Group; British Society of Gastroenterology. Diagnosis and management of adult coeliac disease: guidelines from the British Society of Gastroenterology. Gut. 2014;63(8):1210–28. DOI:10.1136/gutjnl-2013–306578. Epub 2014 Jun 10.Google Scholar
Castillo, NE, Theethira, TG, Leffler, DA. The present and the future in the diagnosis and management of celiac disease. Gastroenterol Rep (Oxf). 2015;3(1):3–11. DOI:10.1093/gastro/gou065. Epub 2014 Oct 17.Google Scholar
ClinicalTrials.gov. http://www.clinicaltrials.gov. October 2016.Google Scholar
Elli, L, Branchi, F, Tomba, C, et al. Diagnosis of gluten related disorders: celiac disease, wheat allergy and non-celiac gluten sensitivity. World J Gastroenterol. 2015;21(23):7110–19. DOI:10.3748/wjg.v21.i23.7110.Google Scholar
Rubio-Tapia, A, Hill, ID, Kelly, CP, Calderwood, AH, Murray, JA; American College of Gastroenterology. ACG clinical guidelines: diagnosis and management of celiac disease. Am J Gastroenterol. 2013 May;108(5):656–76; quiz 677. DOI:10.1038/ajg.2013.79. Epub 2013 Apr 23.Google Scholar
Kwiecien, J, Karczewska, K, Lukasik, M, et al. Negative results of antiendomysial antibodies: long term follow up. Arch Dis Child. 2005;90(1):41–2.Google Scholar
Green, PH, Jabri, B. Coeliac disease. Lancet. 2003;362:383–91.Google Scholar
Bao, F, Green, PH, Bhagat, G. An update on celiac disease histopathology and the road ahead. Arch Pathol Lab Med. 2012;136(7):735–45. DOI:10.5858/arpa.2011–0572–RA.Google Scholar
Mino-Kenudson, M, Brown, I, Lauwers, GY. Histopathological diagnosis of gluten-sensitive enteropathy. Curr. Diagn. Pathol. 2005;11:274–83.Google Scholar
Chang, F, Mahadeva, U, Deere, H. Pathological and clinical significance of increased intraepithelial lymphocytes (IELs) in small bowel mucosa. APMIS. 2005;113:385–99.Google Scholar
Hayat, M, Cairns, A, Dixon, MF, O’Mahony, S. Quantitation of intraepithelial lymphocytes in human duodenum: what is normal? J Clin Pathol. 2002;55:393–4.Google Scholar
Veress, B, Franzén, L, Bodin, L, Borch, K. Duodenal intraepithelial lymphocyte-count revisited. Scand J Gastroenterol. 2004;39:138–44.Google Scholar
Goldstein, NS, Underhill, J. Morphologic features suggestive of gluten sensitivity in architecturally normal duodenal biopsy specimens. Am J Clin Pathol. 2001;116:63–71.Google Scholar
Marsh, MN. Gluten, major histocompatibility complex, and the small intestine: a molecular and immunobiologic approach to the spectrum of gluten sensitivity (‘celiac sprue’). Gastroenterology. 1992;102(1):330–54.Google Scholar
Oberhuber, G, Granditsch, G, Vogelsang, H. The histopathology of coeliac disease: time for a standardized report scheme for pathologists. Eur J Gastroenterol Hepatol. 1999;11(10):1185–94.Google Scholar
Corazza, GR, Villanacci, V. Coeliac disease. J Clin Pathol. 2005;58(6):573–4.CrossRefGoogle ScholarPubMed
Zhao, X, Johnson, RL. Collagenous sprue: a rare, severe small-bowel malabsorptive disorder. Arch Pathol Lab Med. 2011;135(6):803–9. DOI:10.1043/2010-0028-RS.1.Google Scholar
Freeman, HJ. Update on collagenous sprue. World J Gastroenterol. 2010;16(3):296–8.Google Scholar
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. DOI:10.1038/modpathol.2009.151. Epub 2009 Oct 23.Google Scholar
Soendergaard, C, Riis, LB, Nielsen, OH. Collagenous sprue: a coeliac disease look-alike with different treatment strategy. BMJ Case Rep. 2014;2014. pii: bcr2014203721. DOI:10.1136/bcr-2014–203721.Google Scholar
Olaussen, RW, Løvik, A, Tollefsen, S, et al. Effect of elemental diet on mucosal immunopathology and clinical symptoms in type 1 refractory celiac disease. Clin. Gastroenterol. Hepatol. 2005; 3 :875–85.Google Scholar
Kung, VL, Liu, TC, Ma, C. Collagenous enteritis is unlikely a form of aggressive celiac Disease Despite Sharing HLA-DQ2/DQ8 Genotypes. Am J Surg Pathol. 2018;42(4):545–52.Google Scholar
Cuoco, L, Villanacci, V, Salvagnini, M, Bassotti, G. Collagenous sprue with associated features of refractory celiac disease. Rev Esp Enferm Dig. 2012;104(4):223–5.Google Scholar
Lynch, DA, Sobala, GM, Dixon, MF, et al. Lymphocytic gastritis and associated small bowel disease: a diffuse lymphocytic gastroenteropathy? J Clin Pathol. 1995;48(10):939–45.Google Scholar
Feeley, KM, Heneghan, MA, Stevens, FM, McCarthy, CF. Lymphocytic gastritis and coeliac disease: evidence of a positive association. J Clin Pathol. 1998;51(3):207–10.Google Scholar
Corazza, GR, Villanacci, V, Zambelli, C, et al. Comparison of the interobserver reproducibility with different histologic criteria used in celiac disease. Clin Gastroenterol Hepatol. 2007;5(7):838–43. Epub 2007 Jun 4.Google Scholar
Askling, J, Linet, M, Gridley, G, et al. Cancer incidence in a population-based cohort of individuals hospitalised with celiac disease or dermatitis herpetiformis. Gastroenterology. 2002;123:1428–35.Google Scholar
Catassi, C, Bearzi, I, Holmes, GK. Association of celiac disease and intestinal lymphomas and other cancers. Gastroenterology. 2005;128(4 Suppl 1):S79–86.Google Scholar
Wahab, PJ, Meijer, JW, Goerres, MS, Mulder, CJ. Coeliac disease: changing views on gluten-sensitive enteropathy. Scand J Gastroenterol Suppl. 2002;236:60–5.Google Scholar
Ho-Yen, C, Chang, F, van der Walt, J, Mitchell, T, Ciclitira, P. Recent advances in refractory coeliac disease: a review. Histopathology. 2009;54(7):783–95. DOI:10.1111/j.1365–2559.2008.03112.x. Epub 2009 Aug 12.Google Scholar
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;356:203–8.Google Scholar
Nijeboer, P, van Wanrooij, RL, Tack, GJ, Mulder, CJ, Bouma, G. Update on the diagnosis and management of refractory coeliac disease. Gastroenterol Res Pract. 2013;2013:518483. DOI:10.1155/2013/518483. Epub 2013 May 9.Google Scholar
Al-Toma, A, Verbeek, WH, Mulder, CJ. Update on the management of refractory coeliac disease. J Gastrointestin Liver Dis. 2007;16:57–63.Google Scholar
Daum, S, Cellier, C, Mulder, CJ. Refractory coeliac disease. Best Pract Res Clin Gastroenterol. 2005;19:413–24.Google Scholar
Brousse, N, Meijer, JW. Malignant complications of coeliac disease. Best Pract Res Clin Gastroenterol. 2005;19:401–12.CrossRefGoogle ScholarPubMed
Robert ME, Ament ME, Weinstein WM. The histologic spectrum and clinical outcome of refractory and unclassified sprue. Am. J. Surg. Pathol. 2000;24:676–87.Google Scholar
Goerres, MS, Meijer, JW, Wahab, PJ, et al. Azathioprine and prednisone combination therapy in refractory coeliac disease. Aliment Pharmacol Ther. 2003;18:487–94.CrossRefGoogle ScholarPubMed
Cellier, C, Patey, N, Mauvieux, L, et al. Abnormal intestinal intraepithelial lymphocytes in refractory sprue. Gastroenterology. 1998;114:471–81.Google Scholar
Verkarre, V, Asnafi, V, Lecomte, T, et al. Refractory coeliac sprue is a diffuse gastrointestinal disease. Gut. 2003;52:205–11.Google Scholar
Patey-Mariaud De Serre, N, Cellier, C, Jabri, B, et al. Distinction between coeliac disease and refractory sprue: a simple immunohistochemical method. Histopathology. 2000;37:70–7.Google Scholar
Nasr, I, Nasr, I, Beyers, C, Chang, F, Donnelly, S, Ciclitira, PJ. Recognising and managing refractory coeliac disease: a tertiary centre experience. Nutrients. 2015 Dec 1;7(12):9896–907. DOI:10.3390/nu7125506.Google Scholar
Meijer, JW, Mulder, CJ, Goerres, MG, Boot, H, Schweizer, JJ. Coeliac disease and (extra)intestinal T-cell lymphomas: definition, diagnosis and treatment. Scand J Gastroenterol Suppl. 2004; 241 :78–84.CrossRefGoogle Scholar
Jewell, DP. Ulcerative enteritis. Br Med J (Clin Res Ed). 1983;287:1740–1.Google Scholar
Mills, PR, Brown, IL, Watkinson, G. Idiopathic chronic ulcerative enteritis. Report of five cases and review of the literature. Q J Med. 1980;49:133–49.Google Scholar
Elsing, C, Placke, J, Gross-Weege, W. Ulcerative jejunoileitis and enteropathy-associated T-cell lymphoma. Eur J Gastroenterol Hepatol. 2005;17:1401–5.Google Scholar
Bagdi, E, Diss, TC, Munson, P, Isaacson, PG. Mucosal intra-epithelial lymphocytes in enteropathy-associated T-cell lymphoma, ulcerative jejunitis, and refractory celiac disease constitute a neoplastic population. Blood. 1999;94:260–4.CrossRefGoogle ScholarPubMed
Ashton-Key, M, Diss, TC, Pan, L, Du, MQ, Isaacson, PG. Molecular analysis of T-cell clonality in ulcerative jejunitis and enteropathy-associated T-cell lymphoma. Am J Pathol. 1997;151:493–8.Google Scholar
Arps, DP, Smith, LB. Classic versus type II enteropathy-associated T-cell lymphoma: diagnostic considerations. Arch Pathol Lab Med. 2013;137(9):1227–31. DOI:10.5858/arpa.2013–0242–CR.Google Scholar
Delabie, J, Holte, H, Vose, JM, Ullrich, F, et al. Enteropathy-associated T-cell lymphoma: clinical and histological findings from the international peripheral T-cell lymphoma project. Blood. 2011;118(1):148–55. DOI:10.1182/blood-2011–02–335216. Epub 2011 May 12.Google Scholar
Rooney, N, Dogan, A. Gastrointestinal lymphoma. Curr Diagn Pathol. 2004;10:69–78.Google Scholar
Deleeuw, RJ, Zettl, A, Klinker, E, et al. Whole-genome analysis and HLA genotyping of enteropathy-type T-cell lymphoma reveals 2 distinct lymphoma subtypes. Gastroenterology. 2007;132:1902–11.Google Scholar
Isaacson, P, Wright, D, Ralfkiaer, E, Jaffe, ES. Enteropathy-type T-cell lymphoma. In Jaffe, JS, Harris, NL, Stein, H, Vardiman, JW JW (eds.), Pathology and Genetics of Tumours of the Haematopoietic and Lymphoid Tissues. Lyon, France: IARC Press; World Health Organisation Classification of Tumours; 2001, 208–9.Google Scholar
Murray, A, Cuevas, EC, Jones, DB, Wright, DH. Study of the immunohistochemistry and T cell clonality of enteropathy-associated T cell lymphoma. Am J Pathol. 1995;146:509–19.Google Scholar
Catassi, C, Bearzi, I, Holmes, GK. Association of celiac disease and intestinal lymphomas and other cancers. Gastroenterology. 2005;128(4 Suppl 1):S79–86.Google Scholar
Smedby, KE, Akerman, M, Hildebrand, H, et al. Malignant lymphomas in coeliac disease: evidence of increased risks for lymphoma types other than enteropathy-type T cell lymphoma. Gut. 2005; 54:54–9.Google Scholar
Howdle, PD, Jalal, PK, Holmes, GK, Houlston, RS. Primary small-bowel malignancy in the UK and its association with coeliac disease. QJM. 2003;96:345–53.Google Scholar
Rampertab, SD, Forde, KA, Green, PH. Small bowel neoplasia in coeliac disease. Gut. 2003; 52 :1211–14.CrossRefGoogle ScholarPubMed
Askling, J, Linet, M, Gridley, G, et al. Cancer incidence in a population-based cohort of individuals hospitalised with celiac disease or dermatitis herpetiformis. Gastroenterology. 2002;123:1428–35.Google Scholar
Brown, I, Mino-Kenudson, M, Deshpande, V, Lauwers, GY. Intraepithelial lymphocytosis in architecturally preserved proximal small intestinal mucosa: an increasing diagnostic problem with a wide differential diagnosis. Arch Pathol Lab Med. 2006;130:1020–5.Google Scholar
Mino, M, Lauwers, GY. Role of lymphocytic immunophenotyping in the diagnosis of gluten-sensitive enteropathy with preserved villous architecture. Am J Surg Pathol. 2003;27:1237–42.Google Scholar
Akram, S, Murray, JA, Pardi, DS, et al. Adult autoimmune enteropathy: Mayo Clinic Rochester experience. Clin Gastroenterol Hepatol. 2007 Nov;5(11):1282–90; quiz 1245. Epub 2007 Aug 1.Google ScholarPubMed
Corazza, GR, Biagi, F, Volta, U, Andreani, ML, De Franceschi, L, Gasbarrini, G. Autoimmune enteropathy and villous atrophy in adults. Lancet. 1997;350(9071):106–9.Google Scholar
Masia, R, Peyton, S, Lauwers, GY, Brown, I. Gastrointestinal biopsy findings of autoimmune enteropathy: a review of 25 cases. Am J Surg Pathol. 2014;38(10):1319–29. DOI:10.1097/PAS.0000000000000317.Google Scholar
Volta, U, Mumolo, MG, Caio, G, et al. Autoimmune enteropathy: not all flat mucosa mean coeliac disease. Gastroenterol Hepatol Bed Bench. 2016;9(2):140–5.Google Scholar
Gabrielli, M, D’Angelo, G, Di Rienzo, T, carpellini, E, Ojetti, V. Diagnosis of small intestinal bacterial overgrowth in the clinical practice. Eur Rev Med Pharmacol Sci. 2013;17(Suppl 2):30–5.Google Scholar
Bures, J, Cyrany, J, Kohoutova, D, et al. Small intestinal bacterial overgrowth syndrome. World J Gastroenterol. 2010;16(24):2978–90.Google Scholar
Lappinga, PJ, Abraham, SC, Murray, JA, Vetter, EA, Patel, R, Wu, TT. Small intestinal bacterial overgrowth: histopathologic features and clinical correlates in an underrecognized entity. Arch Pathol Lab Med. 2010;134(2):264–70. DOI:10.1043/1543–2165–134.2.264.CrossRefGoogle Scholar
Agarwal, S, Smereka, P, Harpaz, N, Cunningham-Rundles, C, Mayer, L. Characterization of immunologic defects in patients with common variable immunodeficiency (CVID) with intestinal disease. Inflamm Bowel Dis. 2011;17(1):251–9. DOI:10.1002/ibd.21376.Google Scholar
Biagi, F, Bianchi, PI, Zilli, A, et al. The significance of duodenal mucosal atrophy in patients with common variable immunodeficiency: a clinical and histopathologic study. Am J Clin Pathol. 2012;138(2):185–9. DOI:10.1309/AJCPEIILH2C0WFYE.CrossRefGoogle ScholarPubMed
Ramakrishna, BS, Venkataraman, S, Mukhopadhya, A. Tropical malabsorption. Postgrad Med J. 2006;82(974):779–87.Google Scholar
Babbin, BA, Crawford, K, Sitaraman, SV. Malabsorption work-up: utility of small bowel biopsy. Clin Gastroenterol Hepatol. 2006;4(10):1193–8. Epub 2006 Sep 18.Google Scholar
Batheja, MJ, Leighton, J, Azueta, A, Heigh, R. The face of tropical sprue in 2010. Case Rep Gastroenterol. 2010;4(2):168–72.Google Scholar
Ghoshal, UC, Mehrotra, M, Kumar, S, et al. Spectrum of malabsorption syndrome among adults & factors differentiating celiac disease & tropical malabsorption. Indian J Med Res. 2012;136(3):451–9.Google Scholar
Jeffers, MD, Hourihane, DO. Coeliac disease with histological features of peptic duodenitis: value of assessment of intraepithelial lymphocytes. J Clin Pathol. 1993;46(5):420–4.CrossRefGoogle ScholarPubMed
Gdalevich, M, Cohen, D, Ashkenazi, I, Mimouni, D, Shpilberg, O, Kark, JD. Helicobacter pylori infection and subsequent peptic duodenal disease among young adults. Int J Epidemiol. 2000;29(3):592–5.Google Scholar
Leonard, N, Feighery, CF, Hourihane, DO. Peptic duodenitis: does it exist in the second part of the duodenum? J Clin Pathol. 1997;50(1):54–8.Google Scholar
Wright, CL, Riddell, RH. Histology of the stomach and duodenum in Crohn’s disease. Am J Surg Pathol. 1998;22(4):383–90.Google Scholar
Culliford, A, Markowitz, D, Rotterdam, H, Green, PH. Scalloping of duodenal mucosa in Crohn’s disease. Inflamm Bowel Dis. 2004;10(3):270–3.Google Scholar
Kocsis, D, Tóth, Z, Csontos, ÁA, et al. Prevalence of inflammatory bowel disease among coeliac disease patients in a Hungarian coeliac centre. BMC Gastroenterol. 2015;15:141. DOI:10.1186/s12876-015-0370-7.Google Scholar

Save book to Kindle

To save this book to your Kindle, first ensure no-reply@cambridge.org is added to your Approved Personal Document E-mail List under your Personal Document Settings on the Manage Your Content and Devices page of your Amazon account. Then enter the ‘name’ part of your Kindle email address below. Find out more about saving to your Kindle.

Note you can select to save to either the @free.kindle.com or @kindle.com variations. ‘@free.kindle.com’ emails are free but can only be saved to your device when it is connected to wi-fi. ‘@kindle.com’ emails can be delivered even when you are not connected to wi-fi, but note that service fees apply.

Find out more about the Kindle Personal Document Service.

Available formats
×

Save book to Dropbox

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Dropbox.

Available formats
×

Save book to Google Drive

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Google Drive.

Available formats
×