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 Christopher Smyrk, Bret Thomas Petersen, Mark Topazian, Naoki Takahashi, Michael B. Farnell, Santhi Swaroop Vege

Research output: Contribution to journalArticle

292 Citations (Scopus)

Abstract

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
JournalGastroenterology
Volume139
Issue number1
DOIs
StatePublished - Jul 2010

Fingerprint

Pancreatitis
Recurrence
Pancreaticoduodenectomy
Inflammatory Bowel Diseases
Immunoglobulins
Adrenal Cortex Hormones
Survival Rate
Kidney

Keywords

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

ASJC Scopus subject areas

  • Gastroenterology

Cite this

Differences in Clinical Profile and Relapse Rate of Type 1 Versus Type 2 Autoimmune Pancreatitis. / Sah, Raghuwansh P.; Chari, Suresh T; Pannala, Rahul; Sugumar, Aravind; Clain, Jonathan E.; Levy, Michael J.; Pearson, Randall K.; Smyrk, Thomas Christopher; Petersen, Bret Thomas; Topazian, Mark; Takahashi, Naoki; Farnell, Michael B.; Vege, Santhi Swaroop.

In: Gastroenterology, Vol. 139, No. 1, 07.2010, p. 140-148.

Research output: Contribution to journalArticle

Sah, Raghuwansh P. ; Chari, Suresh T ; Pannala, Rahul ; Sugumar, Aravind ; Clain, Jonathan E. ; Levy, Michael J. ; Pearson, Randall K. ; Smyrk, Thomas Christopher ; Petersen, Bret Thomas ; Topazian, Mark ; Takahashi, Naoki ; Farnell, Michael B. ; Vege, Santhi Swaroop. / Differences in Clinical Profile and Relapse Rate of Type 1 Versus Type 2 Autoimmune Pancreatitis. In: Gastroenterology. 2010 ; Vol. 139, No. 1. pp. 140-148.
@article{6fee0507d75445f598fb01c373a674f3,
title = "Differences in Clinical Profile and Relapse Rate of Type 1 Versus Type 2 Autoimmune Pancreatitis",
abstract = "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.",
keywords = "Autoimmunity, Chronic Pancreatitis, Corticosteroid Therapy, IgG4-Related Systemic Disease",
author = "Sah, {Raghuwansh P.} and Chari, {Suresh T} and Rahul Pannala and Aravind Sugumar and Clain, {Jonathan E.} and Levy, {Michael J.} and Pearson, {Randall K.} and Smyrk, {Thomas Christopher} and Petersen, {Bret Thomas} and Mark Topazian and Naoki Takahashi and Farnell, {Michael B.} and Vege, {Santhi Swaroop}",
year = "2010",
month = "7",
doi = "10.1053/j.gastro.2010.03.054",
language = "English (US)",
volume = "139",
pages = "140--148",
journal = "Gastroenterology",
issn = "0016-5085",
publisher = "W.B. Saunders Ltd",
number = "1",

}

TY - JOUR

T1 - Differences in Clinical Profile and Relapse Rate of Type 1 Versus Type 2 Autoimmune Pancreatitis

AU - Sah, Raghuwansh P.

AU - Chari, Suresh T

AU - Pannala, Rahul

AU - Sugumar, Aravind

AU - Clain, Jonathan E.

AU - Levy, Michael J.

AU - Pearson, Randall K.

AU - Smyrk, Thomas Christopher

AU - Petersen, Bret Thomas

AU - Topazian, Mark

AU - Takahashi, Naoki

AU - Farnell, Michael B.

AU - Vege, Santhi Swaroop

PY - 2010/7

Y1 - 2010/7

N2 - 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.

AB - 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.

KW - Autoimmunity

KW - Chronic Pancreatitis

KW - Corticosteroid Therapy

KW - IgG4-Related Systemic Disease

UR - http://www.scopus.com/inward/record.url?scp=77953890776&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=77953890776&partnerID=8YFLogxK

U2 - 10.1053/j.gastro.2010.03.054

DO - 10.1053/j.gastro.2010.03.054

M3 - Article

C2 - 20353791

AN - SCOPUS:77953890776

VL - 139

SP - 140

EP - 148

JO - Gastroenterology

JF - Gastroenterology

SN - 0016-5085

IS - 1

ER -