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-cd9895bd7-8ctnn Total loading time: 0 Render date: 2024-12-26T21:23:52.133Z Has data issue: false hasContentIssue false

Chapter 2 - Gastrointestinal Involvement by Systemic Disease

Published online by Cambridge University Press:  06 June 2020

By
Adrian C. Bateman
Edited by
Roger M. Feakins
Roger M. Feakins
Affiliation:
Royal Free London NHS Foundation Trust, London, UK
Get access

Summary

Many diseases that have a systemic distribution may involve the gastrointestinal (GI) tract and liver. Furthermore, diseases that usually manifest within one extra-gastrointestinal organ or system may also involve the GI tract, either directly or as a result of treatment for the extra-GI disease. This chapter focuses initially on five systemic diseases that can have GI manifestations: sarcoidosis, amyloidosis, mast cell diseases, IgG4-related disease, and Behçet’s disease, and then discusses diseases with cutaneous manifestations and their effect(s) on the GI tract. Systemic diseases may become manifest within the GI tract in the context of a known condition. When this occurs, the cause of the GI disease may be obvious. Alternatively, the GI tract features may represent the presenting phase of the disease, which could already be active but subclinical at other sites. The differential diagnosis of certain histopathological features associated with GI tract manifestations of systemic disease, e.g. granulomas, may be wide. Therefore, careful clinicopathological correlation is essential. Finally, treatments for some extra-GI conditions may cause GI-related side effects, e.g. methotrexate therapy for psoriasis.

Keywords

gastrointestinal tractintestineliversarcoidosisamyloidosismast cell diseaseIgG4-related diseaseBehçet’s diseasecoeliac diseasepsoriasisbullous skin disease
Type
Chapter
Information
Non-Neoplastic Pathology of the Gastrointestinal Tract
A Practical Guide to Biopsy Diagnosis
 , pp. 13 - 29
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

Reich, JM. Epidemiology of sarcoidosis. Mayo Clin Proc. 2016;91:822–3.Google Scholar
Pietinalho, A, Hiraga, Y, Hosoda, Y, Lofroos, AB, Yamaguchi, M, Selroos, O. The frequency of sarcoidosis in Finland and Hokkaido, Japan: a comparative epidemiological study. Sarcoidosis. 1995;12:61–7.Google Scholar
Swigris, JJ, Olson, AL, Huie, TJ, et al. Sarcoidosis-related mortality in the United States from 1988 to 2007. Am J Respir Crit Care Med. 2011;183:1524–30.CrossRefGoogle ScholarPubMed
Rybicki, BA, Maliarik, M, Major, M, Popovich, J Jr, Iannuzzi, MC. Epidemiology, demographics, and genetics of sarcoidosis. Semin Respir Infect. 1998;13:166–73.Google Scholar
Judson, MA. The clinical features of sarcoidosis: a comprehensive review. Clin Rev Allergy Immunol. 2015;49:63–78.CrossRefGoogle ScholarPubMed
Ghozzi, A, Azouz, H, Chelly, I, et al. Unusual presentation of sarcoidosis: solitary intracranial mass lesion mimicking an intracranial neoplasm: a case report. Pan Afr Med J. 2014;18:236.CrossRefGoogle ScholarPubMed
Hammen, I, Sherson, DL, Davidsen, JR. Systemic sarcoidosis mimicking malignant metastatic disease. Eur Clin Respir J. 2015. DOI:10.3402/ecrj.v2.26761. eCollection 2015.Google Scholar
Tadros, M, Forouhar, F, Wu, GY. Hepatic sarcoidosis. J Clin Transl Hepatol. 2013;1:87–93.Google Scholar
Ebert, EC, Kierson, M, Hagspiel, KD. Gastrointestinal and hepatic manifestations of sarcoidosis. Am J Gastroenterol. 2008;103:3184–92.Google ScholarPubMed
Palmer, ED. Note on silent sarcoidosis of the gastric mucosa. J Lab Clin Med. 1958;52:231.Google ScholarPubMed
Attou, R, Duttmann, R, Devriendt, A, De Bels, D. Oesophageal lesions as first manifestations of necrotising sarcoid granulomatosis. BMJ Case Rep. 2015. DOI:10.1136/bcr-2015-209471.CrossRefGoogle Scholar
Gourevitch, A, Cunningham, IJ. Sarcoidosis of the sigmoid colon. Postgrad Med J. 1959;35:689.CrossRefGoogle ScholarPubMed
Yasar, Z, Ozgul, MA, Cetinkaya, E, et al. Angiotensin-converting enzyme as a predictor of extrathoracic involvement of sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis. 2016;32:318–24.Google Scholar
Mostard, RLM, Voo, S, van Kroonenburgh, MJPG, et al. Inflammatory activity assessment by F18 FDG-PET/CT in persistent symptomatic sarcoidosis. Respir Med. 2011;105:1917–24.Google Scholar
James, DG, Williams, WJ. Kveim-Siltzbach test revisited. Sarcoidosis. 1991;8:6–9.Google Scholar
Rosen, Y. Four decades of necrotizing sarcoid granulomatosis: what do we know now? Arch Pathol Lab Med. 2015;139:252–62.Google Scholar
Navasakulpong, A, Auger, M, Gonzalez, AV. Yield of EBUS-TBNA for the diagnosis of sarcoidosis: impact of operator and cytopathologist experience. BMJ Open Resp Res. 2016;3:e000144. DOI:10.1136/bmjresp-2016-000144.Google Scholar
Ebert, EC, Nagar, M. Gastrointestinal manifestations of amyloidosis. Am J Gastroenterol. 2008;103:776–87.Google Scholar
Lim, AY, Lee, JH, Jung, KS, et al. Clinical features and outcomes of systemic amyloidosis with gastrointestinal involvement: a single-center experience. Kor J Int Med. 2015;30:496–505.Google Scholar
Said, SM, Grogg, KL, Smyrk, TC. Gastric amyloidosis: clinicopathological correlations in 79 cases from a single institution. Hum Pathol. 2015;46:491–8.Google Scholar
Freudenthaler, S, Hegenbart, U, Schonland, S, et al. Amyloid in biopsies of the gastrointestinal tract – a retrospective observational study on 542 patients. Virchows Arch. 2016;468:569–77.Google Scholar
Pozzi, C, D’Amico, M, Fogazzi, GB, et al. Light chain deposition disease with renal involvement: clinical characteristics and prognostic factors. Am J Kidney Dis. 2003;42:1154–63.CrossRefGoogle ScholarPubMed
Valent, P. Mastocytosis: a paradigmatic example of a rare disease with complex biology and pathology. Am J Cancer Res. 2013;3:159–72.Google Scholar
Horan, RF, Austen, KF. Systemic mastocytosis: retrospective review of a decade’s clinical experience at the Brigham and Women’s Hospital. J Invest Dermatol. 1991;96;5S.CrossRefGoogle ScholarPubMed
Horny, H-P, Kaiserling, E, Campbell, M, Parwaresch, MR, Lennert, K. Liver findings in generalised mastocytosis: a clinicopathological study. Cancer. 1989;63:532–8.Google Scholar
Kristensen, T, Broesby-Olsen, S, Vestergaard, H, et al. Serum tryptase correlates with the KIT D816V mutation burden in adults with indolent systemic mastocytosis. Eur J Haematol. 2013;91:106–11.CrossRefGoogle ScholarPubMed
Erben, P, Schwaab, J, Metzgeroth, G, et al. The KIT D816V expressed allele burden for diagnosis and disease monitoring of systemic mastocytosis. Ann Hematol. 2014;93:81–8.CrossRefGoogle ScholarPubMed
Yadav, D, Notohara, K, Smyrk, TC, et al. Idiopathic tumefactive chronic pancreatitis: clinical profile, histology, and natural history after resection. Clin Gastroenterol Hepatol. 2003;1:129–35.Google Scholar
Uchida, K, Masamume, A, Shimosegawa, T, Okazaki, K. Prevalence of IgG4-related disease in Japan based on nationwide survey in 2009. Int J Rheumatol. 2012;2012:358371. http://dx.doi.org/10.1155/2012/358371. Epub 2012 Jul 31.Google Scholar
Culver, EL, Bateman, AC. IgG4-related disease. In Probert, C(ed), Recent Advances in Gastroenterology 12. London: JP Medical; 2013, 93–111.Google Scholar
Kamisawa, T, Funata, N, Hayashi, Y, et al. A new clinicopathological entity of IgG4-related autoimmune disease. J Gastroenterol. 2003;38:982–84.CrossRefGoogle ScholarPubMed
Della Torre, E, Mattoo, H, Mahajan, VS, Carruthers, M, Pillai, S, Stone, JH. Prevalence of atopy, eosinophilia, and IgE elevation in IgG4-related disease. Allergy. 2014;69:269–72.Google Scholar
Chetty, R, Serra, S, Gauchotte, G, Markl, B, Agaimy, A. Sclerosing nodular lesions of the gastrointestinal tract containing large numbers of IgG4 plasma cells. Pathology. 2011;43:31–5.Google Scholar
Hiyoshi, Y, Oki, E, Zaitsu, Y, et al. IgG4-related disease of the ileocaecal region mimicking malignancy: a case report. Int J Surg Case Rep. 2014;5:669–72.Google Scholar
Rebours, V, Le Baleur, Y, Cazals-Hatem, D, et al. Immunoglobulin G4 immunostaining of gastric, duodenal or colonic biopsies is not helpful for the diagnosis of autoimmune pancreatitis. Clin Gastroenterol Hepatol. 2012;10:91–4.CrossRefGoogle Scholar
Moon, SH, Kim, MH, Park, DH, et al. IgG4 immunostaining of duodenal papillary biopsy specimens may be useful for supporting a diagnosis of autoimmune pancreatitis. Gastrointestinal Endosc. 2010;71:960–6.Google Scholar
Tsen, A, Alishahi, Y, Rosenkranz, L. Autoimmune pancreatitis and inflammatory bowel disease: an updated review. J Clin Gastroenterol. 2017;51:208–14.CrossRefGoogle ScholarPubMed
Virk, R, Shinagare, S, Lauwers, GY, Yajnik, V, Stone, JH, Deshpande, V. Tissue IgG4-positive plasma cells in inflammatory bowel disease: a study of 88 treatment-naïve biopsies of inflammatory bowel disease. Mod Pathol. 2014;27:454–9.CrossRefGoogle ScholarPubMed
Ferry, J. IgG4-related lymphadenopathy and IgG4-related lymphoma: moving targets. Diagn Histopathol. 2013;19:128–39.Google Scholar
Chari, ST. Diagnosis of autoimmune pancreatitis using its five cardinal features: introducing the Mayo Clinic’s HISORt criteria. J Gastroenterol. 2007; 42(Suppl 18):39–41.Google Scholar
Umehara, H, Okazaki, K, Masaki, Y, et al. Comprehensive diagnostic criteria for IgG4-related disease, 2011. Mod Rheumatol. 2012;22:21–30.Google Scholar
Culver, EL, Sadler, R, Simpson, D, et al. Elevated serum IgG4 levels in diagnosis, treatment response, organ involvement, and relapse in a prospective IgG4-related disease UK cohort. Am J Gastroenterol. 2016;111:733–43.CrossRefGoogle Scholar
Wallace, ZS, Mattoo, H, Carruthers, M, et al. Plasmablasts as a biomarker for IgG4-related disease, independent of serum IgG4 concentrations. Ann Rheum Dis. 2015;74:190–5.Google Scholar
Deshpande, V, Zen, Y, Chan, JYC, et al. Consensus statement on the pathology of IgG4-related disease. Mod Pathol. 2012;25:1181–92.Google Scholar
Strehl, J, Hartmann, A,Agaimy, A. Numerous IgG 4-positive plasma cells are ubiquitous in diverse localised non-specific inflammatory conditions and need to be distinguished from IgG4-related systemic disorders. J Clin Pathol. 2011;64:237–43.Google Scholar
Bateman, AC, Culver, EL, Sommerlad, M, Chetty, R. Intraduct papillary-mucinous neoplasm of the pancreas: a tumour linked with IgG4-related disease? J Clin Pathol. 2013;66:671–5.Google Scholar
Cottom, H, Mighell, AJ, High, A, Bateman, AC. Are plasma cell-rich inflammatory conditions of the oral mucosa manifestations of IgG4-related disease? J Clin Pathol. 2015;68:802–7.Google Scholar
Bateman, AC, Culver, EL. IgG4-related disease: experience of 100 consecutive cases from a specialist centre. Histopathology. 2016. DOI:10.1111/his.13136. [Epub ahead of print].Google Scholar
Brito-Zeron, P, Kostov, B, Bosch, X, Acar-Denizli, N, Ramos-Casals, M, Stone, JH. Therapeutic approach to IgG4-related disease: a systematic review. Medicine (Baltimore). 2016;95(26):e4002. DOI:10.1097/MD.0000000000004002.Google Scholar
Kamisawa, T, Shimosegawa, T, Okazaki, K, et al. Standard steroid treatment for autoimmune pancreatitis. Gut. 2009;58:1504–7.Google Scholar
Collin, P, Salmi, TT, Hervonen, K, Kaukinen, K, Reunala, T. Dermatitis herpetiformis: a cutaneous manifestation of coeliac disease. Ann Med. 2017;49:23–31.CrossRefGoogle ScholarPubMed
Faias, S, Lage, P, Sachse, F, et al. Pemphigus vulgaris with exclusive involvement of the esophagus: case report and review. Gastrointest Endosc. 2004;60:312–15.Google Scholar
Fine, J-D, Johnson, LB, Weiner, M, Suchindran, C. Gastrointestinal complications of inherited epidermolysis bullosa: cumulative experience of the National Epidermolysis Bullosa Registry. J Paediatr Gastroenterol Nutr. 2008;46:147–58.Google Scholar
Neilsen, JA, Law, RM, Firman, KH, Roberts, CA. Esophageal lichen planus: a case report and review of the literature. World J Gastroenterol. 2013;19:2278–81.Google Scholar
Shimizu, H, Kinoshita, T, Suzuki, H. Darier’s disease with esophageal carcinoma. Eur J Dermatol. 2000;10:470–2.Google Scholar
Zweiban, B, Cohen, H, Chandrasoma, P. Gastrointestinal involvement complicating Stevens-Johnson syndrome. Gastroenterology. 1986;91:469–74.CrossRefGoogle ScholarPubMed
Martyn-Simmons, CL, Rosenberg, WM, Cross, R, Wong, T, Smith, CH, Barker, JN. Validity of noninvasive markers of methotrexate-induced hepatotoxicity: a retrospective cohort study. Br J Dermatol. 2014;171:267–73.Google Scholar
Kaffenberger, BH, Kaffenberger, JA, Wong, H, Jarjour, W, Levin, D, Bechtel, MA. Magnetic resonance elastography and transient elastography as non-invasive analyses for liver fibrosis: can they obviate the need for liver biopsy in psoriasis patients treated with methotrexate? Int J Dermatol. 2015;54:752–6.Google Scholar
Miele, L, Vallone, S, Cefalo, C, et al. Prevalence, characteristics and severity of non-alcoholic fatty liver disease in patients with chronic plaque psoriasis. J Hepatol. 2009;51:778–86.Google Scholar
Poulin, Y, Thérien, G. Drug-induced hepatitis and lupus during infliximab treatment for psoriasis: case report and literature review. J Cutan Med Surg. 2010;14:100–4.CrossRefGoogle ScholarPubMed
Fallon, MB, Boyer, JL. Hepatic toxicity of vitamin A and synthetic retinoids. J Gastroenterol Hepatol. 1990;5:344–2.Google Scholar
Behçet, H. Uber rezidivierende aphthöse, durch ein Virus verursachte geschwure, am Mund, am Auge, und an den Genitalien. Dermatol Wochenschr. 1937;105:1152–7.Google Scholar
Hatemi, G, Seyahi, E, Fresko, I, Talicaro, R, Hamuryudan, V. One year in review 2016: Behçet’s syndrome. Clin Exp Rheumatol. 2016;45(6 Suppl 102):10–22.Google Scholar
Alpsoy, E. Behçet’s disease: a comprehensive review with a focus on epidemiology, etiology and clinical features, and management of mucocutaneous lesions. J Dermatol. 2016;43:620–32.Google ScholarPubMed
Akman-Demir, G, Serdaroglu, P, Tasci B and the Neuro-Behçet Study Group. Brain. 1999;122:2171–81.Google Scholar
Mizushima, Y. Recent research into Behçet’s disease in Japan. Int J Tissue React. 1988;10:59–65.Google Scholar
International Study Group for Behçet’s disease. Criteria for diagnosis of Behçet’s disease. Lancet. 1990;335:1078–80.Google Scholar
International Team for the Revision of the International Criteria for Behçet’s Disease (ITR-ICBD). The International Criteria for Behçet’s Disease (ICBD): a collaborative study of 27 countries on the sensitivity and specificity of the new criteria. J Eur Acad Dermatol Venereol. 2014;28:338–47.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
×