IgG4-related disease. Clinical manifestation differential diagnosis and recent International Diagnostic Criteria for IgG4-related disease

Vnitr Lek. 2022 Fall;68(E-5):4-19. doi: 10.36290/vnl.2022.070.


Immunoglobulin G4- related disease (IgG4-RD) is a rare systemic fibro-inflammatory disorder. Autoimmune pancreatitis is the most frequent manifestation of IgG4-RD. However, IgG4-RD can affect any organ such as salivary glands, orbits, retroperitoneum, prostate and many others. Recent research enabled a clear clinical and histopathological description of IgG4-RD and in 2019 four Clinical phenotypes of IgG4-related disease were described. Diagnosis is based on morphological examination with typical findings of lymphoplasmocellular inflammation, storiform fibrosis and obliterative phlebitis in IgG4-RD biopsies and the tissue invading plasma cells largely produce IgG4. Elevated serum IgG4 levels are found in many but not all patients. New diagnostic criteria for IgG4-RD have been published recently in 2019 and 2021. This review summarizes current knowledge on pathophysiology, clinical manifestations, diagnosis and differential diagnosis of IgG4-RD from the point of view 2022 and in next article brings overview of the IgG4-RD therapy.

Keywords: IgG4 immunoglobulin subclass; IgG4 related disease.

Publication types

  • Review

MeSH terms

  • Autoimmune Diseases* / diagnosis
  • Diagnosis, Differential
  • Fibrosis
  • Humans
  • Immunoglobulin G
  • Immunoglobulin G4-Related Disease* / diagnosis
  • Immunoglobulin G4-Related Disease* / pathology
  • Inflammation
  • Male
  • Rare Diseases / diagnosis


  • Immunoglobulin G