Onemocnění asociované s imunoglobulinem IgG4. Klinické příznaky, diferenciální diagnostika a recentní mezinárodní diagnostická kritéria
[IgG4-related disease. Clinical manifestation differential diagnosis and recent International Diagnostic Criteria for IgG4-related disease]
Language Czech Country Czech Republic Media print
Document type Review, Journal Article
PubMed
36283812
DOI
10.36290/vnl.2022.070
PII: 132192
- Keywords
- IgG4 immunoglobulin subclass, IgG4 related disease,
- MeSH
- Autoimmune Diseases * diagnosis MeSH
- Diagnosis, Differential MeSH
- Fibrosis MeSH
- Immunoglobulin G4-Related Disease * diagnosis pathology MeSH
- Immunoglobulin G MeSH
- Humans MeSH
- Rare Diseases diagnosis MeSH
- Inflammation MeSH
- Check Tag
- Humans MeSH
- Male MeSH
- Publication type
- Journal Article MeSH
- Review MeSH
- Names of Substances
- Immunoglobulin G MeSH
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.
References provided by Crossref.org
