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Purpose of Review <p>Autoimmune pancreatitis (AIP) refers to a group of diseases characterized by chronic immune-mediated inflammation of the pancreas. In the past decade, there has been tremendous progress in the understanding of these forms of AIP, and as a result, the landscape of treatment and monitoring of AIP has evolved rapidly. Here, we summarize the pathophysiology and clinical presentation of Types 1 and 2 AIP and review the management of AIP based on recent literature.&#xa0;</p> Recent Findings <p>Type 1 (lymphoplasmacytic) AIP is the pancreatic manifestation of IgG4-related disease (IgG4-RD), a systemic disease that most often presents with multiorgan involvement. Type 2 (idiopathic duct centric) AIP typically presents in younger individuals and is strongly associated with inflammatory bowel disease (IBD). Both may present with symptoms of pancreatitis or radiologic findings such as pancreatic enlargement or mass lesions. AIP frequently leads to pancreatic damage, including atrophy, exocrine insufficiency, or endocrine insufficiency (pancreatogenic diabetes mellitus), and all patients should be monitored longitudinally for the development of these complications. In contrast to Type 1 AIP, which typically has a chronic, relapsing course, the vast majority of cases of Type 2 AIP do not relapse. Glucocorticoids are universally effective in both, but the chronic and relapsing nature of Type 1 AIP necessitates the use glucocorticoid-sparing therapies. While observational data have supported the historical use of conventional immunosuppressive agents such as azathioprine, mycophenolate mofetil, and leflunomide in Type 2 AIP, recent studies including the phase 3 MITIGATE trial of inebilizumab in IgG4-RD have confirmed that B cell depletion is profoundly effective in the disease. Accordingly, B cell depletion should now be used as the first line treatment in most patients with Type 1 AIP/IgG4-RD. Multiple ongoing clinical trials are investigating the efficacy of other treatment targets in Type 1 AIP/IgG4-RD. As Type 2 AIP is most often monophasic, most patients do not require glucocorticoid-sparing medications; however, in relapsing cases associated with IBD, IBD-targeted medications may improve pancreatic inflammation as well.</p> Summary <p>In recent years, our understanding of the clinical presentation, pathophysiology, and treatment of AIP has grown immensely. While effective treatments exist, further research is needed to identify additional effective treatment options and clarify the optimal manner by which B cell depletion should be used to balance disease control and risk of immunosuppression.</p>

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Evolution of Treatment for Autoimmune Pancreatitis: Recent Advancements

  • 期刊论文

  • 发表日期：

    2025年6月17日

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  • Guy Katz,
  • Federica Bonaso,
  • Cory A. Perugino,
  • Yasmin G. Hernandez-Barco

摘要

Purpose of Review

Autoimmune pancreatitis (AIP) refers to a group of diseases characterized by chronic immune-mediated inflammation of the pancreas. In the past decade, there has been tremendous progress in the understanding of these forms of AIP, and as a result, the landscape of treatment and monitoring of AIP has evolved rapidly. Here, we summarize the pathophysiology and clinical presentation of Types 1 and 2 AIP and review the management of AIP based on recent literature. 

Recent Findings

Type 1 (lymphoplasmacytic) AIP is the pancreatic manifestation of IgG4-related disease (IgG4-RD), a systemic disease that most often presents with multiorgan involvement. Type 2 (idiopathic duct centric) AIP typically presents in younger individuals and is strongly associated with inflammatory bowel disease (IBD). Both may present with symptoms of pancreatitis or radiologic findings such as pancreatic enlargement or mass lesions. AIP frequently leads to pancreatic damage, including atrophy, exocrine insufficiency, or endocrine insufficiency (pancreatogenic diabetes mellitus), and all patients should be monitored longitudinally for the development of these complications. In contrast to Type 1 AIP, which typically has a chronic, relapsing course, the vast majority of cases of Type 2 AIP do not relapse. Glucocorticoids are universally effective in both, but the chronic and relapsing nature of Type 1 AIP necessitates the use glucocorticoid-sparing therapies. While observational data have supported the historical use of conventional immunosuppressive agents such as azathioprine, mycophenolate mofetil, and leflunomide in Type 2 AIP, recent studies including the phase 3 MITIGATE trial of inebilizumab in IgG4-RD have confirmed that B cell depletion is profoundly effective in the disease. Accordingly, B cell depletion should now be used as the first line treatment in most patients with Type 1 AIP/IgG4-RD. Multiple ongoing clinical trials are investigating the efficacy of other treatment targets in Type 1 AIP/IgG4-RD. As Type 2 AIP is most often monophasic, most patients do not require glucocorticoid-sparing medications; however, in relapsing cases associated with IBD, IBD-targeted medications may improve pancreatic inflammation as well.

Summary

In recent years, our understanding of the clinical presentation, pathophysiology, and treatment of AIP has grown immensely. While effective treatments exist, further research is needed to identify additional effective treatment options and clarify the optimal manner by which B cell depletion should be used to balance disease control and risk of immunosuppression.