Recent advances in diagnosis, treatment, and prognosis of IgG4-related autoimmune pancreatitis: a narrative review

Immunoglobulin G4 (IgG4)-related autoimmune pancreatitis (AIP), also known as “type 1 AIP,” is a rare, chronic, and fibroinflammatory disease manifested as obstructive jaundice and enlargement of the pancreas, usually accompanied by extra-pancreatic organ involvement. The understanding of IgG4-relat...

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Published inJournal of Pancreatology Vol. 5; no. 4; pp. 202 - 209
Main Authors Xin He, Boyuan Sun, Yuxue Nie, Wen Zhang
Format Journal Article
LanguageEnglish
Published Wolters Kluwer Health/LWW 01.12.2022
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Abstract Immunoglobulin G4 (IgG4)-related autoimmune pancreatitis (AIP), also known as “type 1 AIP,” is a rare, chronic, and fibroinflammatory disease manifested as obstructive jaundice and enlargement of the pancreas, usually accompanied by extra-pancreatic organ involvement. The understanding of IgG4-related AIP is gradually deepening. In this review, we summarized the basic concepts, common clinical manifestations, and new progress of the disease including diagnostic, therapeutic strategies, and prognosis mainly based on published case reports, cohort studies, meta-analyses, and guidelines in the past 5 years. Issues such as diagnostic markers, risk factors for relapse, and more effective treatment still need to be further studied.
AbstractList Immunoglobulin G4 (IgG4)-related autoimmune pancreatitis (AIP), also known as “type 1 AIP,” is a rare, chronic, and fibroinflammatory disease manifested as obstructive jaundice and enlargement of the pancreas, usually accompanied by extra-pancreatic organ involvement. The understanding of IgG4-related AIP is gradually deepening. In this review, we summarized the basic concepts, common clinical manifestations, and new progress of the disease including diagnostic, therapeutic strategies, and prognosis mainly based on published case reports, cohort studies, meta-analyses, and guidelines in the past 5 years. Issues such as diagnostic markers, risk factors for relapse, and more effective treatment still need to be further studied.
Author Wen Zhang
Yuxue Nie
Xin He
Boyuan Sun
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  organization: a Peking Union Medical College, M.D. Program, Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing, China
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  fullname: Boyuan Sun
  organization: a Peking Union Medical College, M.D. Program, Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing, China
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  fullname: Yuxue Nie
  organization: b Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases, State Key Laboratory of Complex Severe and Rare Diseases, Beijing, China
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  fullname: Wen Zhang
  organization: b Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases, State Key Laboratory of Complex Severe and Rare Diseases, Beijing, China
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