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Observational Study
. 2024 Apr;12(3):319-325.
doi: 10.1002/ueg2.12504. Epub 2024 Jan 12.

Clinical features and long-term outcomes of patients with type 2 autoimmune pancreatitis

Nicolò de Pretis  1 , Michele Carlin  1 , Emilia Calderini  1 , Federico Caldart  1 , Maria Cristina Conti Bellocchi  1 , Antonio Amodio  1 , Giulia De Marchi  1 , Pietro Campagnola  1 , Stefano Francesco Crinò  1 , Laura Bernardoni  1 , Armando Gabbrielli  1 , Luigi Martinelli  2 , Luca Frulloni  1
Affiliations
Observational Study

Clinical features and long-term outcomes of patients with type 2 autoimmune pancreatitis

Nicolò de Pretis et al. United European Gastroenterol J. 2024 Apr.

Abstract

Objectives: Type 2 is a rare form of autoimmune pancreatitis (AIP). Despite being considered a benign disease, only few studies with limited sample size and short follow-up have been published on type 2 AIP. The aim of this observational study was to evaluate long-term outcomes, such as the risk of relapse, pancreatic insufficiency and cancer in a large type 2 AIP cohort with long follow-up.

Methods: Patients with definitive or probable diagnosis of type 2 AIP by International Consensus Diagnostic Criteria (ICDC) present in our prospectively maintained database since 1995 at 31.12.2021 were identified. All patients were clinically evaluated during the year 2022. Clinical, radiological, serological, and pathological data were evaluated.

Results: Eighty-eight out of 420 patients present in the database (21%) were diagnosed with type 2 AIP (mean age 33.5 ± 13.5 years). According to the ICDC, 21 patients (23.8%) had a definitive and 67 (76.2%) a probable diagnosis of type 2 AIP. The mean follow-up was 9.2 ± 7.1 years (range 1-27 years). No differences were observed when comparing patients with definitive and probable type 2 AIP diagnosis. Concomitant IBD was reported in 77 patients (87.5%). The probability of disease relapse was lower in patients treated with steroids versus surgery (at 5 years 13% vs. 33%; p = 0.038) but this difference was not statistically significant at multivariable analysis. The risk of endocrine or severe exocrine insufficiency was low (5% and 25%). Four extra-pancreatic malignancies (5%) were diagnosed, none pancreatic. One patient died in a car accident.

Conclusions: Type 2 AIP has benign long-term clinical outcomes. Mortality and cancer rates are low and no specific follow-up is needed after radiological remission.

Keywords: autoimmune pancreatitis; inflammatory bowel disease; long term outcomes; pancreatic insufficiency.

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Conflict of interest statement

The authors have no conflicts of interest to declare.

Figures

FIGURE 1
FIGURE 1
The probability of relapse‐free survival in 88 type 2 autoimmune pancreatitis patients.
FIGURE 2
FIGURE 2
The probability of relapse‐free survival in 78 non‐surgically resected patients (solid line) and in 10 surgically resected patients (dashed line). The difference was statistically significant (p = 0.038) at univariable analysis.
See this image and copyright information in PMC

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