Gastroenterology

Gastroenterology

Volume 139, Issue 1, July 2010, Pages 140-148
Gastroenterology

Clinical Advances in Liver, Pancreas, and Biliary Tract
Differences in Clinical Profile and Relapse Rate of Type 1 Versus Type 2 Autoimmune Pancreatitis

https://doi.org/10.1053/j.gastro.2010.03.054Get rights and content

Background & Aims

Autoimmune pancreatitis (AIP) has been divided into subtypes 1 (lymphoplasmacytic sclerosing pancreatitis) and 2 (idiopathic duct centric pancreatitis). We compared clinical profiles and long-term outcomes of types 1 and 2 AIP.

Methods

We compared clinical presentation, relapse, and vital status of 78 patients with type 1 AIP who met the original HISORt criteria and 19 patients with histologically confirmed type 2 AIP.

Results

At presentation, patients with type 1 AIP were older than those with type 2 AIP (62 ± 14 vs 48 ± 19 years; P < .0001) and had a greater prevalence of increased serum levels of immunoglobulin G4 (47/59 [80%] vs 1/6 [17%]; P = .004). Patients with type 1 were more likely than those with type 2 to have proximal biliary, retroperitoneal, renal, or salivary disease (60% vs 0; P < .0001). Inflammatory bowel disease was associated with types 1 and 2 (6% vs 16%; P = .37). During median clinical follow-up periods of 42 and 29 months, respectively, 47% of patients with type 1 and none of those with type 2 experienced a relapse. In type 1 AIP, proximal biliary involvement (hazard ratio [HR], 2.12; P = .038) and diffuse pancreatic swelling (HR, 2.00; P = .049) were predictive of relapse, whereas pancreaticoduodenectomy reduced the relapse rate (vs the corticosteroid-treated group; HR, 0.15; P = .0001). After median follow-up periods of 58 and 89 months (types 1 and 2, respectively), the 5-year survival rates for both groups were similar to those of the age- and sex-matched US population.

Conclusions

Types 1 and 2 AIP have distinct clinical profiles. Patients with type 1 AIP have a high relapse rate, but patients with type 2 AIP do not experience relapse. AIP does not affect long-term survival.

Section snippets

Case Ascertainment

The institutional review board at the Mayo Clinic approved the study protocol and the contact of consenting patients. The database of patients with AIP is maintained prospectively by one of the authors (S.T.C.). The initial patients in the database were identified through a retrospective review of pancreatic resection specimens (19 with type 1 and 12 with type 2 AIP) reported earlier.5 All of these patients were on continuous clinical follow-up. Subsequently, we have prospectively identified a

Type 1 AIP

Of the 78 cases of type 1 AIP, 50 (63%) were histologically confirmed and 28 met original HISORt criteria for type 1 without having histologic confirmation. These 2 groups were similar in demography (mean age, 62 ± 14 years; 77% male), clinical presentation (12 [15%] as acute pancreatitis), and IgG4 seropositivity (47/59 [80%]). However, the patients with histologically confirmed type 1 AIP were more likely to have undergone surgery (50% vs 0%), more likely to have focal features on imaging

Discussion

Recently it has been suggested that the term “AIP” encompasses not only 2 distinct histologic subtypes but also 2 distinct clinical entities that have been designated as type 1 and type 2 AIP.7, 39 The use of the terms “type 1” and “type 2” AIP to describe clinical profiles of lymphoplasmacytic sclerosing pancreatitis and IDCP, respectively, was debated at a recent international consensus meeting held in Honolulu, Hawaii. Although some experts raised questions about whether IDCP was even an

References (45)

  • K. Notohara et al.

    Idiopathic chronic pancreatitis with periductal lymphoplasmacytic infiltration: clinicopathologic features of 35 cases

    Am J Surg Pathol

    (2003)
  • G. Zamboni 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)
  • D.H. Park et al.

    Recent advances in autoimmune pancreatitis

    Gut

    (2009)
  • T. Kamisawa et al.

    Lymphoplasmacytic sclerosing pancreatitis is a pancreatic lesion of igG4-related systemic disease

    Am J Surg Pathol

    (2004)
  • V. Deshpande et al.

    Autoimmune pancreatitis: a systemic immune complex mediated disease

    Am J Surg Pathol

    (2006)
  • A. Ghazale et al.

    Immunoglobulin G4-associated cholangitis: clinical profile and response to steroids

    Gastroenterology

    (2008)
  • N. Yamamoto et al.

    Mikulicz's disease and systemic IgG4-related plasmacytic syndrome (SIPS)

    Nihon Rinsho Meneki Gakkai Kaishi

    (2008)
  • N. Takahashi et al.

    Renal involvement in patients with autoimmune pancreatitis: CT and MR imaging findings

    Radiology

    (2007)
  • L.D. Cornell et al.

    Pseudotumors due to IgG4 immune complex tubulointerstitial nephritis associated with autoimmune pancreatitocentric disease

    Am J Surg Pathol

    (2007)
  • K. Okazaki et al.

    Autoimmune related pancreatitis

    Gut

    (2002)
  • M. Otsuki et al.

    Asian diagnostic criteria for autoimmune pancreatitis: consensus of the Japan-Korea Symposium on Autoimmune Pancreatitis

    J Gastroenterol

    (2008)
  • T. Kamisawa et al.

    Treating patients with autoimmune pancreatitis: results from a long-term follow-up study

    Pancreatology

    (2005)

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    This article has an accompanying continuing medical education activity on page page e12. Learning Objective: Upon completion of reading this article, successful learners will be able to recognize the existence of 2 distinct subtypes within AIP, namely Type 1 and Type 2 with different risks of relapse, as well as identify their characteristic clinical associations and pathology findings, and recognize factors associated with increased risk of relapse.

    Conflicts of interest The authors disclose no conflicts.

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