Pancreas
United European Gastroenterol J. 2024;12(3):319–25
Clinical features and long-term outcomes of patients with type 2 autoimmune pancreatitis
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 the authors’ 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: 88 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%) had 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 inflammatory bowel disease 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 extrapancreatic malignancies (5%) were diagnosed, none pancreatic. One patient died in a car accident.
Conclusions: Type 2 autoimmune pancreatitis has benign long-term clinical outcomes. Mortality and cancer rates are low and no specific follow-up is needed after radiological remission.
DOI: 10.1002/ueg2.12504