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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 place of work: 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 the journal: Vnitř Lék 2022; 68(E-5): 4-19
Category: Review Articles
doi: https://doi.org/10.36290/vnl.2022.070

Summary

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


Zdroje

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