Onemocnění asociované s imunoglobulinem IgG4. Klinické příznaky, diferenciální diagnostika a recentní mezinárodní diagnostická kritéria

Title in English IgG4-related disease. Clinical manifestation differential diagnosis and recent International Diagnostic Criteria for IgG4-related disease
Authors

ADAM Zdeněk ZEMAN David ČERMÁK Aleš DASTYCH Milan DOUBKOVÁ Martina HORVÁTH Teodor SKORKOVSKÁ Šárka ADAMOVÁ Zuzana ŘEHÁK Zdeněk KOUKALOVÁ Renata POUR Luděk ŠTORK Martin KREJČÍ Marta SANDECKÁ Viera ŠEVČÍKOVÁ Sabina KRÁL Zdeněk

Year of publication 2022
Type Article in Periodical
MU Faculty or unit

Faculty of Medicine

Citation
Web https://www.casopisvnitrnilekarstvi.cz/pdfs/vnl/2022/05/15.pdf
Doi http://dx.doi.org/10.36290/vnl.2022.070
Keywords IgG4 immunoglobulin subclass; IgG4 related disease
Description 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, retroperito-neum, 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. © 2022, SOLEN s.r.o.. All rights reserved.

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