Vnitr Lek 2022, 68(5):E20-E26 | DOI: 10.36290/vnl.2022.070

IgG4-related disease. Clinical manifestation differential diagnosis and recent International Diagnostic Criteria for IgG4-related disease

Zdeněk Adam1, David Zeman2, Aleš Čermák3, Milan Dastych4, Martina Doubková5, Theodor Horváth6, Šárka Skorkovská7, Zuzana Adamová8, Zdeněk Řehák9, Renata Koukalová9, Luděk Pour1, Martin Štork1, Marta Krejčí1, Viera Sandecká1, Sabina Ševčíková10, Zdeněk Král1
1 Interní hematologická a onkologická klinika LF MU a FN Brno
2 Ústav laboratorní medicíny - Oddělení klinické biochemie, FN Brno
3 Urologická klinika LF MU a FN Brno
4 Interní gastroenterologická klinika LF MU a FN Brno
5 Klinika nemocí plicních a tuberkulózy LF MU a FN Brno
6 Chirurgická klinika LF MU a FN Brno
7 Oční klinika LF MU a FN Brno
8 Chirurgické oddělení nemocnice Frýdek Místek a Chirurgické oddělení nemocnice Vsetín
9 Oddělení nukleární medicíny, Masarykův onkologický ústav Brno
10 Ústav patologické fyziologie LF MU, Brno

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.

Accepted: August 5, 2022; Published: August 22, 2022  Show citation

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Adam Z, Zeman D, Čermák A, Dastych M, Doubková M, Horváth T, et al.. IgG4-related disease. Clinical manifestation differential diagnosis and recent International Diagnostic Criteria for IgG4-related disease. Vnitr Lek. 2022;68(5):E20-26. doi: 10.36290/vnl.2022.070.
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