IgG4-related digestive diseases: diagnosis and treatment

Front Immunol. 2023 Oct 5:14:1278332. doi: 10.3389/fimmu.2023.1278332. eCollection 2023.

Abstract

IgG4-related digestive diseases encompass a group of chronic inflammatory disorders characterized by autoimmune reactions and fibrosis affecting multiple digestive organs. These diseases are identified by elevated serum levels of IgG4 and the presence of IgG4-positive plasma cell infiltration in the affected sites, along with storiform fibrosis, obliterative phlebitis, and eosinophilic infiltration. Although extensive research has been conducted, a comprehensive understanding of these conditions remains elusive. Current clinical diagnosis often relies on the application of integrated diagnostic criteria for IgG4-related diseases, combined with specific organ involvement criteria. Distinguishing them from malignancies poses considerable challenges. Moreover, further investigations are required to elucidate the underlying pathogenic mechanisms and explore potential therapeutic interventions. This review provides a systematic classification of IgG4-related digestive diseases while discussing their diagnostic strategies, clinical presentations, and treatment modalities. The comprehensive insights shared herein aim to guide clinicians in their practice and contribute to the advancement of knowledge in this field.

Keywords: IgG4; autoimmune reaction; diagnosis; digestive disease; treatment.

Publication types

  • Review

MeSH terms

  • Autoimmune Diseases* / diagnosis
  • Autoimmune Diseases* / therapy
  • Diagnosis, Differential
  • Fibrosis
  • Humans
  • Immunoglobulin G
  • Immunoglobulin G4-Related Disease* / diagnosis
  • Immunoglobulin G4-Related Disease* / therapy

Substances

  • Immunoglobulin G

Grants and funding

The author(s) declare that no financial support was received for the research, authorship, and/or publication of this article.