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