<p>Autoimmune pancreatitis (AIP) is a&#xa0;rare inflammatory disease of the pancreas that has gained recognition as a&#xa0;distinct entity in clinical research in recent decades. Diagnosing AIP presents a&#xa0;clinical challenge, requiring a&#xa0;comprehensive evaluation of various diagnostic modalities. In 2011, the International Consensus Diagnostic Criteria (ICDC) were introduced to provide a&#xa0;structured diagnostic approach. Additional diagnostic systems are also in use, but the ICDC remain the “gold standard”. From a&#xa0;clinical and histopathological perspective, AIP is classified into two types. Type&#xa0;1 AIP represents the pancreatic manifestation of an immunoglobulin&#xa0;G4 (IgG&#xa0;4)-related systemic disease, whereas type&#xa0;2 AIP is confined to the pancreas and is associated with ulcerative colitis. According to the ICDC, patients who do not fully meet the criteria for either entity may be diagnosed with not-otherwise-specified AIP. Differentiating AIP from pancreatic cancer can be challenging. Unlike other forms of pancreatitis, AIP typically responds well to appropriate treatment with steroids; however, disease relapse is relatively common. In recent decades, significant research efforts have been dedicated to AIP, yet substantial unanswered questions and research needs remain. In 2021, recommendations on the diagnosis and treatment of AIP were included for the first time in the German S3 guideline on pancreatitis.</p>

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Diagnostik und Therapie der Autoimmunpankreatitis

  • Julian Cardinal von Widdern,
  • Jonas Rosendahl

摘要

Autoimmune pancreatitis (AIP) is a rare inflammatory disease of the pancreas that has gained recognition as a distinct entity in clinical research in recent decades. Diagnosing AIP presents a clinical challenge, requiring a comprehensive evaluation of various diagnostic modalities. In 2011, the International Consensus Diagnostic Criteria (ICDC) were introduced to provide a structured diagnostic approach. Additional diagnostic systems are also in use, but the ICDC remain the “gold standard”. From a clinical and histopathological perspective, AIP is classified into two types. Type 1 AIP represents the pancreatic manifestation of an immunoglobulin G4 (IgG 4)-related systemic disease, whereas type 2 AIP is confined to the pancreas and is associated with ulcerative colitis. According to the ICDC, patients who do not fully meet the criteria for either entity may be diagnosed with not-otherwise-specified AIP. Differentiating AIP from pancreatic cancer can be challenging. Unlike other forms of pancreatitis, AIP typically responds well to appropriate treatment with steroids; however, disease relapse is relatively common. In recent decades, significant research efforts have been dedicated to AIP, yet substantial unanswered questions and research needs remain. In 2021, recommendations on the diagnosis and treatment of AIP were included for the first time in the German S3 guideline on pancreatitis.