Abstract
High levels of IgG4-positive plasma cells are commonly seen in autoimmune pancreatitis. It has recently become evident that autoimmune pancreatitis is one component of a larger multi-system disease. IgG4-positive plasma cells have been identified in many extrapancreatic tissues, including the colon, biliary tract, liver, and lungs, and thus the term “IgG4-related sclerosing disease” has been proposed. Awareness of IgG4-related sclerosing disease is important, as it has been shown to mimic other conditions like malignancy. This review discusses IgG4-related colitis and its potential for mimicking inflammatory bowel disease.
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Yoshida K, Toki F, Takeuchi T, et al. Chronic pancreatitis caused by autoimmune abnormality. Proposal of concept of autoimmune pancreatitis. Dig Dis Sci. 1995;40:1561–1568.
Kim MH, Kwon S. Diagnostic criteria for autoimmune chronic pancreatitis. J Gastroenterol. 2007;42:42–49.
Nishimori I, Tamakoshi A, Otsuki M. Research committee on intractable diseases of the pancreas, Ministry of Health, Labour, and Welfare of Japan. Prevalence of autoimmune pancreatitis in Japan from a nationwide survey in 2002. J Gastroenterol. 2007;42:6–8.
Pearson RK, Longnecker DS, Chari ST, et al. Controversies in clinical pancreatology: autoimmune pancreatitis: does it exist? Pancreas. 2003;27:1–13.
Sah RP, Pannala R, Chari ST, et al. Prevalence, diagnosis, and profile of autoimmune pancreatitis presenting with features of acute or chronic pancreatitis. Clin Gastroenterol Hepatol. 2010;8:91–96.
Zamboni G, Lüttges J, Capelli P, et al. Histopathological features of diagnostic and clinical relevance in autoimmune pancreatitis: a study on 53 resection specimens and 9 biopsy specimens. Virchows Arch. 2004;445:552–563.
Weber SM, Cubukcu-Dimopulo O, Palesty JA, et al. Lymphoplasmacytic sclerosing pancreatitis: inflammatory mimic of pancreatic carcinoma. J Gastrointest Surg. 2003;7:129–137.
Notohara K, Burgart LJ, Yadav D, et al. Idiopathic chronic pancreatitis with periductal lymphoplasmacytic infiltration: clinicopathologic features of 35 cases. Am J Surg Pathol. 2003;27:1119–1127.
Klöppel G, Lüttges J, Löhr M, et al. Autoimmune pancreatitis: pathological, clinical, and immunological features. Pancreas. 2003;27:14–19.
Kim KP, Kim MH, Song MH, et al. Autoimmune chronic pancreatitis. Am J Gastroenterol. 2004;99:1605–1616.
Morselli-Labate AM, Pezzilli R. Usefulness of serum IgG4 in the diagnosis and follow up of autoimmune pancreatitis: a systematic literature review and meta-analysis. J Gastroenterol Hepatol. 2009;24:15–36.
Ghazale A, Chari ST, Smyrk TC, et al. Value of serum IgG4 in the diagnosis of autoimmune pancreatitis and in distinguishing it from pancreatic cancer. Am J Gastroenterol. 2007;102:1646–1653.
Frulloni L, Lunardi C, Simone R, et al. Identification of a novel antibody associated with autoimmune pancreatitis. NEJM. 2009;361:2135–2142.
Kamisawa T, Naoto E, Hitoshi N, et al. Comparison of radiological and histological features in autoimmune pancreatitis. Hepatogastroenterology. 2006;53:953–956.
Chang WI, Kim BJ, Lee JK, et al. The clinical and radiological characteristics of focal mass-forming autoimmune pancreatitis: comparison with chronic pancreatitis and pancreatic cancer. Pancreas. 2009;38:401–408.
Nakazawa T, Ohara H, Yamada T, et al. Atypical primary sclerosing cholangitis cases associated with unusual pancreatitis. Hepatogastroenterology. 2001;48:625–630.
Kamisawa T, Funata N, Hayashi Y, et al. Close relationship between autoimmune pancreatitis and multifocal fibrosclerosis. Gut. 2003;52:683–687.
Saegusa H, Momose M, Kawa S, et al. Hilar and pancreatic gallium-67 accumulation is characteristic feature of autoimmune pancreatitis. Pancreas. 2003;27:20–25.
Hamano H, Kawa S, Ochi Y, et al. Hydronephrosis associated with retroperitoneal fibrosis and sclerosing pancreatitis. Lancet. 2002;359:1403–1404.
Uchiyama-Tanaka Y, Mori Y, Kimura T, et al. Acute tubulointerstitial nephritis associated with autoimmune-related pancreatitis. Am J Kidney Dis. 2004;43:18–25.
Hirano K, Kawabe T, Komatsu Y, et al. High-rate pulmonary involvement in autoimmune pancreatitis. Intern Med J. 2006;36:58–61.
Komatsu K, Hamano H, Ochi Y, et al. High prevalence of hypothyroidism in patients with autoimmune pancreatitis. Dig Dis Sci. 2005;50:1052–1057.
Yoshimura Y, Takeda S, Ieki Y, et al. IgG4-associated prostatitis complicating autoimmune pancreatitis. Intern Med. 2006;45:897–901.
Ravi K, Chari ST, Vege SS, et al. Inflammatory bowel disease in the setting of autoimmune pancreatitis. Inflamm Bowel Dis. 2009;15:1326–1330.
Ohara H, Nakazawa T, Sano H, et al. Systemic extrapancreatic lesions associated with autoimmune pancreatitis. Pancreas. 2005;31:232–237.
Kamisawa T, Nakajima H, Egawa N, et al. IgG4-related sclerosing disease incorporating sclerosing pancreatitis, cholangitis, sialadenitis and retroperitoneal fibrosis with lymphadenopathy. Pancreatology. 2006;6:132–137.
Kamisawa T, Okamoto A. Autoimmune pancreatitis; proposal of IgG4-related sclerosing disease. J Gastroenterol. 2006;41:613–625.
Kamisawa T, Funata N, Hayashi Y, et al. A new clinicopathological entity of IgG4-related autoimmune disease. J Gastroenterol. 2003;38:982–984.
Kasashima S, Zen Y, Kawashima A, et al. Inflammatory abdominal aortic aneurysm: close relationship to IgG4-related periaortitis. Am J Surg Pathol. 2008;32:197–204.
Yamashita K, Haga H, Kobashi Y, et al. Lung involvement in IgG4-related lymphoplasmacytic vasculitis and interstitial fibrosis. Am J Surg Pathol. 2008;32:1620–1626.
Deshpande V, Chicano S, Finkelberg D, et al. Autoimmune pancreatitis: a systemic immune complex mediated disease. Am J Surg Pathol. 2006;30:1537–1545.
Kojima M, Sipos B, Klapper W, et al. Autoimmune pancreatitis: frequency, IgG4 expression, and clonality of T and B cells. Am J Surg Pathol. 2007;31:521–528.
Zhang L, Notohara K, Levy MJ, et al. IgG4-positive plasma cell infiltration in the diagnosis of autoimmune pancreatitis. Mod Pathol. 2007;20:23–28.
Kawaguchi K, Koike M, Tsuruta K, et al. Lymphoplasmacytic sclerosing pancreatitis with cholangitis: a variant of primary sclerosing cholangitis extensively involving pancreas. Hum Pathol. 1991;22:387–395.
Comings DE, Skubi KB, Van Eyes J, et al. Familial multifocal fibrosclerosis. Findings suggesting that retroperitoneal fibrosis, mediastinal fibrosis, sclerosing cholangitis, Riedel’s thyroiditis, and pseudotumor of the orbit may be different manifestations of a single disease. Ann Intern Med. 1967;66:884–892.
Clark A, Zeman RK, Choyke PL, et al. Pancreatic pseudotumors associated with multifocal idiopathic fibrosclerosis. Gastrointest Radial. 1988;13:30–32.
Jafri SZH, Bree RL, Agha FP, et al. Inflammatory pseudotumor from sclerosing cholangitis. J Comput Assist Tomogr. 1983;7:902–904.
Laitt RD, Hubscher SG, Buckels JA, et al. Sclerosing cholangitis associated with multifocal fibrosis: a case report. Gut. 1992;33:1430–1432.
Levey JM, Mathai J. Diffuse pancreatic fibrosis: an uncommon feature of multifocal idiopathic fibrosclerosis. Am J Gastroenterol. 1998;93:640–642.
Hastier P, Buckley M, Gall Pt, et al. First report of association of chronic pancreatitis, primary biliary cirrhosis, and systemic sclerosis. Dig Dis Sci. 1998;43:2426–2428.
Church NI, Pereira SP, Deheragoda MG, et al. Autoimmune pancreatitis: clinical and radiological features and objective response to steroid therapy in a UK series. Am J Gastroenterol. 2007;102:2417–2425.
Czako L, Hegykozi E, Palinkas A, et al. Autoimmune pancreatitis: functional and morphological recovery after steroid therapy. World J Gastroenterol. 2006;12:1810–1812.
Erkelens GW, Vleggaar FP, Lesterhuis W, et al. Sclerosing pancreato-cholangitis responsive to steroid therapy. Lancet. 1999;354:43–44.
Gardner TB, Chari ST. Autoimmune pancreatitis. Gastroenterol Clin North Am. 2008;37:439–460.
Chari ST. Current concepts in the treatment of autoimmune pancreatitis. JOP. 2007;8:1–3.
Members of the Criteria Committee for Autoimmune Pancreatitis of the Japan Pancreas Society. Diagnostic criteria for autoimmune pancreatitis by the Japan Pancreas Society. J Jpn Pan Soc. 2002;17:585–587.
Chari ST, Smyrk TC, Levy MJ, et al. Autoimmune pancreatitis: diagnosis using histology, imaging, serology, other organ involvement and response to steroids. Clin Gastroenterol Hepatol. 2006;4:1010–1016.
Chari ST, Takahashi N, Levy MJ, et al. A diagnostic strategy to distinguish autoimmune pancreatitis from pancreatic cancer. Clin Gastroenterol Hepatol. 2009;7:1097–1103.
Ball WP, Baggenstoss AH, Bargen JA. Pancreatic lesions associated with chronic ulcerative colitis. Arch Pathol (Chic). 1950;50:345–358.
Altman HS, Philips G, Bank S, et al. Pancreatitis associated with duodenal Crohn’s disease. Am J Gastroenterol. 1983;78:174–177.
Legge DA, Hofman HN, Carlson HC. Pancreatitis as a complication of regional enteritis of the duodenum. Gastroenterology. 1971;61:834–837.
Barthet M, Hastier P, Bernard JP, et al. Chronic pancreatitis and inflammatory bowel disease: true or coincidental association? Am J Gastroenterol. 1999;94:2141–2148.
Seyrig JA, Jian R, Modigliani R, et al. Idiopathic pancreatitis associated with inflammatory bowel disease. Dig Dis Sci. 1985;30:1121–1126.
Hegnhoj J, Hansen CP, Rannem T, et al. Pancreatic function in Crohn’s disease. Gut. 1990;31:1076–1079.
Heikius B, Niemela S, Lehtola J, et al. Pancreatic duct abnormalities and pancreatic function in patients with chronic inflammatory bowel disease. Scand J Gastroenterol. 1996;31:517–523.
Katz S, Bank S, Greenberg RE, et al. Hyperamylasemia in inflammatory bowel disease. J Clin Gastroenterol. 1988;10:627–630.
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Narula, N., Vasudev, M. & Marshall, J.K. IgG4-Related Sclerosing Disease: A Novel Mimic of Inflammatory Bowel Disease. Dig Dis Sci 55, 3047–3051 (2010). https://doi.org/10.1007/s10620-010-1287-1
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DOI: https://doi.org/10.1007/s10620-010-1287-1