IgG4-related disease. Clinical manifestation differential diagnosis and recent International Diagnostic Criteria for IgG4-related disease
Authors:
Zdeněk Adam 1; David Zeman 2; Aleš Čermák 3; Milan Dastych 4; Martina Doubková 5; Theodor Horváth 6; Šárka Skorkovská 7; Zuzana Adamová 8; Zdeněk Řehák 9; Renata Koukalová 9; Luděk Pour 1; Martin Štork 1; Marta Krejčí 1; Viera Sandecká 1; Sabina Ševčíková 10; Zdeněk Král 1
Authors‘ workplace:
Interní hematologická a onkologická klinika LF MU a FN Brno
1; Ústav laboratorní medicíny – Oddělení klinické biochemie, FN Brno
2; Urologická klinika LF MU a FN Brno
3; Interní gastroenterologická klinika LF MU a FN Brno
4; Klinika nemocí plicních a tuberkulózy LF MU a FN Brno
5; Chirurgická klinika LF MU a FN Brno
6; Oční klinika LF MU a FN Brno
7; Chirurgické oddělení nemocnice Frýdek Místek a Chirurgické oddělení nemocnice Vsetín
8; Oddělení nukleární medicíny, Masarykův onkologický ústav Brno
9; Ústav patologické fyziologie LF MU, Brno
10
Published in:
Vnitř Lék 2022; 68(E-5): 4-19
Category:
Review Articles
doi:
https://doi.org/10.36290/vnl.2022.070
Overview
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 related disease – IgG4 immunoglobulin subclass
Sources
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