Autoimmune pancreatitis: the clinicopathological characteristics of the subtype with granulocytic epithelial lesions

Klöppel, G.; Detlefsen, Sönke; Chari, Suresh T.; Longnecker, Daniel S.; Zamboni, Giuseppe

doi:10.1007/s00535-010-0265-x

Cited by 110 publications

(77 citation statements)

References 51 publications

(78 reference statements)

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“…Cases associated with ulcerative colitis in young patients, mainly reported in Europe and the US, show typical pathological neutrophilic lesions called IDCP [22] or AIP with GEL [23,24]. In addition to histopathological findings, no hematological markers suggest that their pathological conditions are different from LPSP [11].…”

Section: Clinical Questions and Statementsmentioning

confidence: 99%

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Amendment of the Japanese Consensus Guidelines for Autoimmune Pancreatitis, 2013 I. Concept and diagnosis of autoimmune pancreatitis

et al. 2014

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Background In response to the proposal of the international consensus diagnostic criteria (ICDC) for autoimmune pancreatitis (AIP) and the Japanese diagnostic criteria in 2011, the 2009 Japanese consensus guidelines for managing AIP required revision. Methods Three committees [the professional committee for making clinical questions (CQs) and statements by Japanese specialists, the expert panelist committee for rating statements by the modified Delphi method, and the evaluating committee by moderators] were organized.

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Section: Clinical Questions and Statementsmentioning

confidence: 99%

“…However, in many cases, patients with type 1 AIP [9,22] or GEL [23,24]. Conversely, cases associated with ulcerative colitis or primary biliary cirrhosis are rarely seen in Japan [6][7][8].…”

Section: Cq-i-2 Are There Characteristic Clinical Symptoms In Aip?mentioning

confidence: 99%

Amendment of the Japanese Consensus Guidelines for Autoimmune Pancreatitis, 2013 I. Concept and diagnosis of autoimmune pancreatitis

et al. 2014

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“…Exact incidence and prevalence in the United States is unknown. AIP is generally divided into two types: Type 1 (Lymphoplasmacytic sclerosing pancreatitis/IgG4 related) and Type 2 (Idiopathic ductcentric pancreatitis) [4,5]. Systemic glucocorticoids remain the mainstay of treatment for AIP.…”

mentioning

confidence: 99%

Mycophenolate therapy in Autoimmune Pancreatitis

Jebakumar¹,

Udayakumar²,

Peterson³

et al. 2016

Gastroenterol Hepatol Endosc

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Autoimmune pancreatitis (AIP) is an uncommon cause of chronic pancreatitis with an estimated prevalence rate of 0.82/100,000 in Japan [1][2][3]. Exact incidence and prevalence in the United States is unknown. AIP is generally divided into two types: Type 1 (Lymphoplasmacytic sclerosing pancreatitis/IgG4 related) and Type 2 (Idiopathic ductcentric pancreatitis) [4,5]. Systemic glucocorticoids remain the mainstay of treatment for AIP. However, more than 50% of patients with AIP treated with steroids experience relapse with the steroid taper [6]. Remission maintenance in many patients requires chronic steroid use, which poses risk of adverse effects [7]. In order to minimize risk of chronic steroid use, drugs such as azathioprine (AZA), 6-mercaptopurine (6-MP) and rituximab (RTX) have been used in AIP [7]. Thiopurine Methyl Transferase (TPMT) activity is reduced in about 11% of general population, who therefore may not tolerate AZA or 6-MP [8]. Also, AZA is associated with drug-induced pancreatitis, which may mimic AIP flare and cause therapeutic confusion. Disadvantages of RTX include its requirement for intravenous infusion and its expense. Hence there is a need for alternative cheaper, effective and safer oral steroid-sparing agents for AIP. Mycophenolate mofetil (MMF), a powerful inhibitor of lymphocyte proliferation, is a commonly used immunosuppressive agent by rheumatologists. It is available in generic form and also the safety profile is known over few decades. It may be better tolerated than AZA or 6-MP in some patients. However data regarding efficacy and safety of MMF in AIP are lacking. As of 4-2016, less than 15 such cases have been reported in Englishlanguage literature [9][10][11][12]. AIP patients were identified by Rheumatology and Gastroenterology providers at the University of Minnesota (UMN), a tertiary referral center for patients with pancreatic diseases. On the basis of previous case series [13], UMN providers have used mycophenolate for AIP treatment in recent years. Here we present one center's experience with mycophenolate mofetil in 4 patients with AIP.Patient A is a 23-year-old female with history of celiac disease, type 1 diabetes mellitus, and chronic urticaria who presented with recurrent abdominal pain. Her abdominal pain was initially thought to be due to sphincter of Oddi dysfunction, but failed to respond to biliary and pancreatic sphincterotomies by endoscopic retrograde cholangio-pancreatography (ERCP). She was started on prednisone 40mg daily for chronic urticaria of unclear etiology (skin biopsy was non-specific) and the prednisone was quickly tapered off. Surprisingly, her abdominal pain improved dramatically. However, she was not a candidate for long-term prednisone therapy because of underlying type 1 diabetes mellitus and anxiety. MMF (750 mg twice daily) was started for her urticaria. However both urticarial rash and abdominal pain improved. When MMF dose was tapered down, her abdominal pain worsened. EUS showed hyper-echoic foci and lobularity in the pancreas. Ampullary ...

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“…Type 2 AIP is regarded as a specific pancreatic disease, characterized histologically by duct-centric pancreatitis with a GEL [21,22,27,35] . Type 2 AIP patients are more frequently diagnosed in western countries, with a younger age of onset and without gender deviation, compared to type 1 [36] .…”

Section: Type 2 Aipmentioning

confidence: 99%

Diagnosis Autoimmune Pancreatitis

Hart¹,

Chari²

The Pancreapedia: Exocrine Pancreas Knowledge Base

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Autoimmune pancreatitis (AIP) is a distinct form of chronic pancreatitis that is increasingly being reported. The presentation and clinical image findings of AIP sometimes resemble those of several pancreatic malignancies, but the therapeutic strategy differs appreciably. Therefore, accurate diagnosis is necessary for cases of AIP. To date, AIP is classified into two distinct subtypes from the viewpoints of etiology, serum markers, histology, other organ involvements, and frequency of relapse: type 1 is related to IgG4 (lymphoplasmacytic sclerosing pancreatitis) and type 2 is related to a granulocytic epithelial lesion (idiopathic duct-centric chronic pancreatitis). Both types of AIP are characterized by focal or diffuse pancreatic enlargement accompanied with a narrowing of the main pancreatic duct, and both show dramatic responses to corticosteroid. Unlike type 2, type 1 is characteristically associated with increasing levels of serum IgG4 and positive serum autoantibodies, abundant infiltration of IgG4-positive plasmacytes, frequent extrapancreatic lesions, and relapse. These findings have led several countries to propose diagnostic criteria for AIP, which consist of essentially similar diagnostic items; however, several differences exist for each country, mainly due to differences in the definition of AIP and the modalities used to diagnose this disease. An attempt to unite the diagnostic criteria worldwide was made with the publication in 2011 of the international consensus diagnostic criteria for AIP, established at the 2010 Congress of the International Association of Pancreatology (IAP).

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Autoimmune pancreatitis: the clinicopathological characteristics of the subtype with granulocytic epithelial lesions

Cited by 110 publications

References 51 publications

Amendment of the Japanese Consensus Guidelines for Autoimmune Pancreatitis, 2013 I. Concept and diagnosis of autoimmune pancreatitis

Amendment of the Japanese Consensus Guidelines for Autoimmune Pancreatitis, 2013 I. Concept and diagnosis of autoimmune pancreatitis

Mycophenolate therapy in Autoimmune Pancreatitis

Diagnosis Autoimmune Pancreatitis

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