Differences in Clinical Profile and Relapse Rate of Type 1 Versus Type 2 Autoimmune Pancreatitis

Raghuwansh P. Sah, Suresh T. Chari, Rahul Pannala, Aravind Sugumar, Jonathan E. Clain, Michael J. Levy, Randall K. Pearson, Thomas C. Smyrk, Bret T. Petersen, Mark D. Topazian, Naoki Takahashi, Michael B. Farnell, Santhi S. Vege

Research output: Contribution to journalArticlepeer-review

317 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)140-148
Number of pages9
Issue number1
StatePublished - Jul 2010


  • Autoimmunity
  • Chronic Pancreatitis
  • Corticosteroid Therapy
  • IgG4-Related Systemic Disease

ASJC Scopus subject areas

  • Hepatology
  • Gastroenterology


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