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

Czech version

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

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