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Background <p>Type 1 autoimmune pancreatitis (AIP) is a unique form of pancreatitis characterized by IgG4-related inflammation and corticosteroid (CS) responsiveness, but long-term outcomes remain unclear. We evaluated 10-year relapse, malignancy, metabolic/nutritional changes, and survival.</p> Methods <p>We retrospectively analyzed 83 CS-treated patients with type 1 AIP followed for ≥ 10&#xa0;years or until death, assessing: relapse and risk factors; standardized incidence ratios (SIRs) and risk factors for malignancies; 10-year changes in HbA1c and serum albumin; CS-related adverse events; and overall survival (OS).</p> Results <p>Cumulative relapse rates at 1, 3, 5, 7, and 10&#xa0;years were 11.0%, 26.9%, 38.3%, 50.0%, and 56.9%. CS discontinuation independently predicted relapse, whereas serum IgG4 normal at diagnosis or normalized after CS was protective. Overall malignancy risk was not increased (SIR 1.00; 95%CI 0.59–1.42), while pancreatic cancer (PC) showed a numerically higher SIR (1.98 overall; 2.96 ≥ 5&#xa0;years postdiagnosis). In 72 patients with 10-year laboratory follow-up, HbA1c improved at 3&#xa0;years but tended to worsen at 10&#xa0;years, and serum albumin declined by 10&#xa0;years (mean −&#xa0;0.27&#xa0;g/dL), exceeding age-related norms. Preexisting diabetes attenuated albumin decline. Ten-year survival was 85.5%, and CS maintenance was associated with better OS.</p> Conclusions <p>Type 1 AIP shows progressive relapse and metabolic deterioration over a decade, while CS maintenance and IgG4 normalization confer long-term protection. Although the overall risk of malignancy was not increased, the incidence of PC during late follow-up was slightly higher. These findings highlight the need for continuous monitoring for relapse, PC, and nutritional decline in AIP management.</p>

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A decade of clinical course and prognostic determinants in type 1 autoimmune pancreatitis: insights from a 10-year longitudinal multicenter retrospective study

  • 期刊论文

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  • 发表日期：

    2026年5月25日

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  • Masaki Miyazawa,
  • Hajime Takatori,
  • Masaki Nishitani,
  • Takuya Komura,
  • Kiichiro Kaji,
  • Masaaki Yano,
  • Kazuya Kitamura,
  • Ryotaro Nakai,
  • Hiroki Matsukawa,
  • Naoki Takemura,
  • Masahiro Yanagi,
  • Kazuki Nagai,
  • Hidenori Kido,
  • Noboru Takata,
  • Tomoyuki Hayashi,
  • Akihiro Seki,
  • Kouki Nio,
  • Shinya Yamada,
  • Noriho Iida,
  • Tetsuro Shimakami,
  • Taro Yamashita

摘要

Background

Type 1 autoimmune pancreatitis (AIP) is a unique form of pancreatitis characterized by IgG4-related inflammation and corticosteroid (CS) responsiveness, but long-term outcomes remain unclear. We evaluated 10-year relapse, malignancy, metabolic/nutritional changes, and survival.

Methods

We retrospectively analyzed 83 CS-treated patients with type 1 AIP followed for ≥ 10 years or until death, assessing: relapse and risk factors; standardized incidence ratios (SIRs) and risk factors for malignancies; 10-year changes in HbA1c and serum albumin; CS-related adverse events; and overall survival (OS).

Results

Cumulative relapse rates at 1, 3, 5, 7, and 10 years were 11.0%, 26.9%, 38.3%, 50.0%, and 56.9%. CS discontinuation independently predicted relapse, whereas serum IgG4 normal at diagnosis or normalized after CS was protective. Overall malignancy risk was not increased (SIR 1.00; 95%CI 0.59–1.42), while pancreatic cancer (PC) showed a numerically higher SIR (1.98 overall; 2.96 ≥ 5 years postdiagnosis). In 72 patients with 10-year laboratory follow-up, HbA1c improved at 3 years but tended to worsen at 10 years, and serum albumin declined by 10 years (mean − 0.27 g/dL), exceeding age-related norms. Preexisting diabetes attenuated albumin decline. Ten-year survival was 85.5%, and CS maintenance was associated with better OS.

Conclusions

Type 1 AIP shows progressive relapse and metabolic deterioration over a decade, while CS maintenance and IgG4 normalization confer long-term protection. Although the overall risk of malignancy was not increased, the incidence of PC during late follow-up was slightly higher. These findings highlight the need for continuous monitoring for relapse, PC, and nutritional decline in AIP management.