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Journal of Gastroenterology

, Volume 53, Issue 8, pp 967–977 | Cite as

Relapse rate and predictors of relapse in a large single center cohort of type 1 autoimmune pancreatitis: long-term follow-up results after steroid therapy with short-duration maintenance treatment

  • Hyun Woo Lee
  • Sung-Hoon Moon
  • Myung-Hwan KimEmail author
  • Dong Hui Cho
  • Jae Hyuck Jun
  • Kwangwoo Nam
  • Tae Jun Song
  • Do Hyun Park
  • Sang Soo Lee
  • Dong-Wan Seo
  • Sung Koo Lee
Original Article—Liver, Pancreas, and Biliary Tract
First Online:

Abstract

Background

Type 1 autoimmune pancreatitis (AIP), as a pancreatic manifestation of IgG4-related disease, shows a favorable prognosis in the short term. However, disease relapse is common in long-term follow-up, despite a successful initial treatment response. This study aimed to identify the predictors of relapse and long-term outcomes in patients with type 1 AIP.

Methods

Patients with more than 2 years of follow-up who met the International Consensus Diagnostic Criteria for type 1 AIP were included. Patients who had undergone pancreatic operations associated with AIP or who lacked sufficient clinical data were excluded.

Results

All 138 patients achieved clinical remission with initial steroid therapy, and 66 (47.8%) experienced relapse during a median 60 (range 24–197) months follow-up. Among the relapsed patients, about 74% (49/66) relapsed within 3 years. About 60% (82/138) had other organ involvement (OOI), most commonly in the proximal bile duct (26.8%). At first diagnosis, OOI, and especially OOI of the proximal bile duct, was a significant independent predictor of relapse (hazard ratio 2.65; 95% confidence interval 1.44–4.89; p = 0.002), according to multivariate analysis. During the follow-up period, 16 (11.6%) patients experienced endocrine/exocrine dysfunction and 32 (23.2%) patients developed de novo pancreatic calcifications/stones. No pancreatic cancer occurred in any patients.

Conclusions

Type 1 AIP has common relapses, and patients with OOI, especially OOI of the proximal bile duct, appear to be at increased risk for relapse. Long-term sequelae, including pancreatic insufficiency and pancreatic calcifications/stones, are common in patients with relapse. To reduce the relapse, longer maintenance treatment may be needed especially for patients at high risk for relapse.

Keywords

Autoimmune pancreatitis Serum IgG4 Relapse Calcification Long-term outcome 

Abbreviations

AIP

Autoimmune pancreatitis

OOI

Other organ involvement

IgG4

Immunoglobulin G4

IgG4-RD

IgG4-related disease

ICDC

International consensus of diagnostic criteria

CBD

Common bile duct

ERCP

Endoscopic retrograde cholangiopancreatography

CT

Computed tomography

MRI

Magnetic resonance imaging

MRCP

Magnetic resonance cholangiopancreatography

SD

Standard deviation

DM

Diabetes mellitus

HR

Hazard ratio

CI

Confidence interval

ULN

Upper limit of normal

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Notes

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Supplementary material

535_2018_1434_MOESM1_ESM.tif (508 kb)
Supplementary material 1 Organ involvement at initial diagnosis and relapse in type 1 AIP. OOI other organ involvement (TIFF 507 kb)

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Copyright information

© Japanese Society of Gastroenterology 2018

Authors and Affiliations

  • Hyun Woo Lee
    • 1
  • Sung-Hoon Moon
    • 2
  • Myung-Hwan Kim
    • 3
    Email author
  • Dong Hui Cho
    • 3
  • Jae Hyuck Jun
    • 3
  • Kwangwoo Nam
    • 4
  • Tae Jun Song
    • 3
  • Do Hyun Park
    • 3
  • Sang Soo Lee
    • 3
  • Dong-Wan Seo
    • 3
  • Sung Koo Lee
    • 3
  1. 1.Department of Internal Medicine, Digestive Disease Center and Research InstituteSoonChunHyang University School of MedicineBucheonSouth Korea
  2. 2.Department of Internal Medicine, Hallym University College of MedicineHallym University Sacred Heart HospitalAnyangSouth Korea
  3. 3.Division of Gastroenterology, Department of Internal Medicine, Asan Medical CenterUniversity of Ulsan College of MedicineSeoulRepublic of Korea
  4. 4.Department of Internal Medicine, Dankook University HospitalDankook University College of MedicineCheonanSouth Korea

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