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• W2895585224 abstract "Type 1 autoimmune pancreatitis (AIP) is a distinct form of pancreatitis characterized by enlargement of the gland, lymphoplasmacytic infiltration and fibrosis, and frequently increased serum IgG/IgG4 levels. Type 1 AIP typically presents in the elderly, more frequently in men, with diffuse enlargement of the pancreas and obstructive jaundice, increased serum IgG/IgG4 levels, and varying degree of other organ involvement (OOI). The frequent coexistence of OOI indicates that type 1 AIP is the pancreatic manifestation of a multiorgan IgG4-related disease (IgG4-RD). By contrast, a rarer form of AIP [idiopathic duct-centric pancreatitis (IDCP), AIP with granulocyte epithelial lesions (GEL), or type 2 AIP] has been described mainly in Western countries. Type 2 AIP differs from type 1 AIP in that it occurs at a younger age without sex predilection, and serum IgG4 elevation and extrapancreatic involvement are absent. In the Advances in Digestive Medicine, a cases series of type 1 AIP by Yen-Chun Chen and colleagues highlights the similarities in clinical manifestations between type 1 AIP and pancreatic ductal adenocarcinoma (PDAC). Differential diagnosis between type 1 AIP and PDAC is crucial and frequently challenging. Both typically present with painless jaundice in the elderly, and imaging findings may be indistinguishable between type 1 AIP with only focal involvement of pancreas and PDAC. However, it is worth noting that type 1 AIP is far less common than PDAC, and misdiagnosis of PDAC as AIP can lead to delayed treatment and unfavorable outcome. On the other hand, incorrect diagnosis of type 1 AIP as PDAC may lead to unnecessary pancreatic resection. In the diagnostic work-up for patients with suspected type 1 AIP, the first priority is to exclude pancreatobiliary malignancies. The International Consensus Diagnostic Criteria (ICDC) for Autoimmune Pancreatitis proposed by International Association of Pancreatology provides diagnostic criteria to enable correct diagnosis of AIP. Furthermore, ICDC provides algorithms for various clinical scenarios. In addition to diagnosis by histological confirmation, type 1 AIP can also be diagnosed with typical imaging studies or response to corticosteroid in the presence of strong collateral evidence. Adherence to ICDC is advised in evaluating patients who present with features suggestive of type 1 AIP to avoid misdiagnosis. Although AIP can also resolve spontaneously without treatment, corticosteroid therapy has been shown to be effective in achieving remission and reducing relapse and longterm unfavorable events. The Japanese consensus guidelines for management of AIP suggest 0.6 mg/kg/day oral prednisolone for 2 – 4 weeks to induce remission, followed by gradual reduction by 5 mg per 1 – 2 weeks, and then tapered over 2-3 months to a dose of 2.5 – 5 mg/day as maintenance. However, relapse after withdrawal of corticosteroid is common, as highlighted in the case series by Chen et al. Disease relapse can occur in approximately 40% of patients despite complete resolution after initial corticosteroid induction, and pancreas atrophy and pancreatic duct stones may develop in the long term. Recent evidence supports that maintenance corticosteroid reduces the risk of relapse, and relapse can be effectively treated by re-induction with corticosteroid. A Japanese multicenter randomized controlled trial showed that patients who received maintenance corticosteroid for 3 years had lower relapse rate compared with those who received corticosteroid for 26 weeks (23.3% vs 57.9%, p = 0.011); no serious corticosteroid-related complications were noted. For patients who require longterm immunosuppression to prevent relapse, immunosuppressant such as azathioprine can be used to avoid the side effects of chronic corticosteroid maintenance. In summary, type 1 AIP is a distinct entity that mimics pancreatobiliary malignancies. Correct identification of type 1 AIP is important to avoid unnecessary surgery, as corticosteroid treatment typically achieves complete remission with relatively favorable outcome. Misdiagnosis of other pancreatic diseases including PDAC as AIP is an emerging problem, which can lead to delayed treatment for cancers or unnecessary corticosteroid treatment for benign disorders other than AIP. Such mistakes usually result from misinterpretation of elevated IgG4 level as diagnostic for type 1 AIP or over-use of corticosteroid trial, and careful adherence to the diagnostic criteria is important to avoid such misdiagnosis. Once the diagnosis of AIP is confirmed or highly likely, corticosteroid treatment can be given with careful assessment of the treatment response; inadequate response prompts consideration of alternative diagnoses and possible need for surgery. Maintenance corticosteroid after initial remission may reduce relapse. The author declares no conflicts of interest." @default.
• W2895585224 created "2018-10-12" @default.
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• W2895585224 date "2017-06-01" @default.
• W2895585224 modified "2023-10-05" @default.
• W2895585224 title "Type 1 autoimmune pancreatitis" @default.
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• W2895585224 doi "https://doi.org/10.1002/aid2.12041" @default.
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