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• W2942194060 abstract "Autoimmune pancreatitis (AIP) is a chronic, steroid-responsive fibroinflammatory disease of the pancreas. The incidence and prevalence of AIP remain largely unknown, but they are increasingly being identified. One study from Japan reported an incidence of 1 per 100,000, whereas the prevalence was estimated to be 5%–6% of all patients with chronic pancreatitis.1Uchida K. Masamune A. Shimosegawa T. et al.Prevalence of IgG4-related disease in Japan based on nationwide survey in 2009.Int J Rheumatol. 2012; 2012: 358371Google Scholar AIP often mimics pancreatic cancer and is identified in 2%–3% of surgical resections for presumed pancreatic cancer.2Hardacre J.M. Iacobuzio-Donahue C.A. Sohn T.A. et al.Results of pancreaticoduodenectomy for lymphoplasmacytic sclerosing pancreatitis.Ann Surg. 2003; 237 (discussion 8–9): 853-858Google Scholar The international consensus diagnostic criteria (ICDC) classify AIP as type 1 AIP or lymphoplasmacytic sclerosing pancreatitis, type 2 AIP or idiopathic duct centric pancreatitis (IDCP), or AIP not otherwise specified.3Okazaki K. Chari S.T. Frulloni L. et al.International consensus for the treatment of autoimmune pancreatitis.Pancreatology. 2017; 17: 1-6Google Scholar Because of the significant differences among type 1 and type 2 AIP with regard to natural history, diagnostic criteria, and management, the term AIP is restricted to lymphoplasmacytic sclerosing pancreatitis, whereas type 2 AIP is termed IDCP. Long-term sequelae from persistent pancreatic inflammation include exocrine insufficiency and diabetes, similar to other causes of chronic pancreatitis. There is no clear evidence of an increased risk of pancreatic cancer in patients with AIP. There are data suggesting that the risk is similar to other causes of chronic pancreatitis.4Ikeura T. Miyoshi H. Uchida K. et al.Relationship between autoimmune pancreatitis and pancreatic cancer: a single-center experience.Pancreatology. 2014; 14: 373-379Google Scholar Patients with AIP most commonly present with obstructive jaundice, whereas 50% of patients with IDCP present with acute pancreatitis.5Sah R.P. Chari S.T. Pannala R. et al.Differences in clinical profile and relapse rate of type 1 versus type 2 autoimmune pancreatitis.Gastroenterology. 2010; 139 (quiz e12–e13): 140-148Google Scholar The obstructive jaundice in AIP can be due to distal biliary ductal compression from pancreatic head swelling or from a proximal biliary stricture. Despite the intense inflammation, patients typically have no or mild abdominal pain. Table 1 compares the key characteristics between AIP and IDCP. AIP belongs to the spectrum of immunoglobulin subclass 4 (IgG4)-related disorders, where other organ involvement includes bile duct stricture, interstitial nephritis, orbital pseudotumor, IgG4-related plasmacytic exocrinopathy of the salivary gland, pulmonary interstitial fibrosis or nodules, and mediastinal or retroperitoneal fibrosis. AIP can also present with isolated pancreatic involvement in 50% of the patients. Inflammatory bowel disease, especially ulcerative colitis (UC), is strongly associated with IDCP; a majority of patients are diagnosed with inflammatory bowel disease before or concurrently with IDCP. The cumulative probability of an IDCP diagnosis after the diagnosis of UC was reported to be 0.2% after 1 year, 0.6% after 5 years, and 0.8% after 10 years.6Park S.H. Kim D. Ye B.D. et al.The characteristics of ulcerative colitis associated with autoimmune pancreatitis.J Clin Gastroenterol. 2013; 47: 520-525Google Scholar Immunoglobulin subclass 4 is a distinctive serologic marker for AIP, and a level ≥2 times the upper limit of normal (280 mg/dL) is considered level 1 evidence per ICDC for the diagnosis of AIP, with sensitivity of 53% and specificity of 99%.3Okazaki K. Chari S.T. Frulloni L. et al.International consensus for the treatment of autoimmune pancreatitis.Pancreatology. 2017; 17: 1-6Google Scholar Pancreatic cancer patients can have elevated IgG4 levels, but only 1% have levels above 2 times the upper limit (280 mg/dL). However, an elevated IgG4 is not characteristic of IDCP, and no reliable serologic marker is currently available to aid in the diagnosis of IDCP. Characteristic radiologic features of AIP on computed tomography or magnetic resonance imaging include diffuse parenchymal enlargement (“sausage shape”), low attenuating capsule (“rim enhancement”), and, less commonly, a focal mass-like lesion or diffuse pancreatic atrophy. Magnetic resonance cholangiopancreatography or endoscopic retrograde cholangiopancreatography may reveal diffuse or multifocal narrowing of the pancreatic duct without upstream dilatation. Focal mass-like pancreatic lesions are more commonly identified in IDCP and are noted in approximately 35% of patients.7Hart P.A. Levy M.J. Smyrk T.C. et al.Clinical profiles and outcomes in idiopathic duct-centric chronic pancreatitis (type 2 autoimmune pancreatitis): the Mayo Clinic experience.Gut. 2016; 65: 1702-1709Google Scholar The distinctive histologic characteristics of AIP and IDCP are described in Table 1, but tissue procurement is not simple because endoscopic ultrasonography–guided fine-needle aspiration cytology has poor sensitivity, even with larger diameter needles.8Morishima T. Kawashima H. Ohno E. et al.Prospective multicenter study on the usefulness of EUS-guided FNA biopsy for the diagnosis of autoimmune pancreatitis.Gastrointest Endosc. 2016; 84: 241-248Google Scholar Endoscopic ultrasonography–guided fine-needle biopsy, which preserves the gland architecture, is needed for tissue diagnosis, and despite the concern for the development of post-procedure complications, smaller cohort studies demonstrated safety and efficacy of fine-needle biopsy in the diagnosis of AIP.9Detlefsen S. Joergensen M.T. Mortensen M.B. Microscopic findings in EUS-guided fine needle (SharkCore) biopsies with type 1 and type 2 autoimmune pancreatitis.Pathol Int. 2017; 67: 514-520Google Scholar The ICDC guidelines recommend use of core biopsy in the setting of focal mass and/or obstructive jaundice, when cancer is excluded and the diagnosis is unclear.10Shimosegawa T. Chari S.T. Frulloni L. et al.International consensus diagnostic criteria for autoimmune pancreatitis: guidelines of the International Association of Pancreatology.Pancreas. 2011; 40: 352-358Google ScholarTable 1Characteristic Differences Between Autoimmune Pancreatitis and Idiopathic Duct Centric PancreatitisAutoimmune pancreatitisIdiopathic duct centric pancreatitisAge at presentation7th decade5th decadeMale gender75%50%Other organ involvement/association with IgG4-related diseasePresent in ∼50%NoneAssociation with IBDLowHigh (10%–20%)Serum IgG4 elevationPresent in ∼66%Present in ∼25%Histologic findingsLymphoplasmacytic infiltrationPresentPresentStoriform fibrosisPresentPresentObliterative phlebitisCharacteristicRarely notedGranulocyte epithelial lesionAbsentCharacteristicPeriductal inflammationPresentPresentIgG4 staining≥10 cells/high-power field<10 cells/high-power fieldResponse to steroids∼100%∼100%Relapse riskUp to 60%<10%Adapted with permission from Hart PA et al., Recent Advances in Autoimmune Pancreatitis. Gastroenterology 2015.IBD, inflammatory bowel disease; IgG4, immunoglobulin subclass 4. Open table in a new tab Adapted with permission from Hart PA et al., Recent Advances in Autoimmune Pancreatitis. Gastroenterology 2015. IBD, inflammatory bowel disease; IgG4, immunoglobulin subclass 4. Numerous guidelines have been proposed to diagnose AIP and IDCP and differentiate this from pancreatic cancer on the basis of a combination of characteristics described in Table 1. ICDC has the greatest sensitivity among these guidelines and uses cardinal features of pancreatic imaging including parenchyma or ducts, serology, other organ involvement, histology of the pancreas, and response to steroid therapy.10Shimosegawa T. Chari S.T. Frulloni L. et al.International consensus diagnostic criteria for autoimmune pancreatitis: guidelines of the International Association of Pancreatology.Pancreas. 2011; 40: 352-358Google Scholar The management of AIP and IDCP is primarily medical as summarized in Figure 1 but warrants close follow-up and monitoring for the possibility of underlying malignancy, because AIP and IDCP are relatively uncommon and have common imitators such as pancreatic cancer. Endoscopic interventions such as endoscopic retrograde cholangiopancreatography and biliary stenting are rarely indicated in the setting of obstructive jaundice. Although biliary stenting reduces the risk of cholangitis and facilitates obtaining cytologic brushings to differentiate benign from malignant strictures, ICDC recommend treating mild jaundice with no signs of infection with steroid therapy alone.3Okazaki K. Chari S.T. Frulloni L. et al.International consensus for the treatment of autoimmune pancreatitis.Pancreatology. 2017; 17: 1-6Google Scholar Surgical interventions are indicated in the setting of suspected malignancy. Symptomatic patients with obstructive jaundice, acute pancreatitis, abdominal pain, and back pain are considered for treatment, and asymptomatic patients are considered only in the setting of persistent pancreatic mass on imaging or with persistent liver function test abnormalities in the setting of IgG4-sclerosing cholangitis/OOI.3Okazaki K. Chari S.T. Frulloni L. et al.International consensus for the treatment of autoimmune pancreatitis.Pancreatology. 2017; 17: 1-6Google Scholar After treatment, there is no value to following serum IgG4 levels to predict relapse or diagnose recurrence.11Hart P.A. Kamisawa T. Brugge W.R. et al.Long-term outcomes of autoimmune pancreatitis: a multicentre, international analysis.Gut. 2013; 62: 1771-1776Google Scholar Despite the efficacy of corticosteroid therapy, normal pancreatic morphology and function may not be restored because the resolution of the inflammation will make the fibro-atrophic component of disease more prominent. Histologic reevaluation is therefore not recommended after treatment. Recurrence or relapse after initial response is noted in up to 60% of AIP patients during steroid tapering or after discontinuation of corticosteroids. Patients with elevated pre-treatment immunoglobulin E levels and OOI with proximal bile duct stricture or IgG4-sclerosing cholangitis are at higher risk of relapse and can be considered for long-term maintenance with steroids or immunomodulator regimens as described in Figure 1.11Hart P.A. Kamisawa T. Brugge W.R. et al.Long-term outcomes of autoimmune pancreatitis: a multicentre, international analysis.Gut. 2013; 62: 1771-1776Google Scholar, 12Culver E.L. Sadler R. Bateman A.C. et al.Increases in IgE, eosinophils, and mast cells can be used in diagnosis and to predict relapse of IgG4-related disease.Clin Gastroenterol Hepatol. 2017; 15: 1444-1452 e6Google Scholar AIP and IDCP are relatively rare causes of painless obstructive jaundice and recurrent acute pancreatitis but amenable to management with steroid therapy. Diagnosis of AIP and IDCP as well as the differentiation of these from pancreatic cancer is based on a combination of serologic, imaging, and histologic characteristics. Although IgG4 is the classically described serologic marker of AIP, it has limited utility in monitoring for risk of relapse or diagnosis of recurrence. Mild obstructive jaundice in the setting of AIP without signs of infection can be treated with corticosteroids alone and does not require interventions for biliary drainage. IgG4-sclerosing cholangitis or proximal biliary strictures and elevated immunoglobulin E levels may identify patients at higher risk of relapse. Intolerance to steroids or relapse is treated with a combination of steroids and immunomodulators or rituximab." @default.
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• W2942194060 title "Management of Autoimmune Pancreatitis" @default.
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