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Title

Case Report: Severe Complement-Mediated Thrombotic Microangiopathy in IgG4-Related Disease Secondary to Anti-Factor H IgG4 Autoantibodies

Authors
Sadallah, Salima
Published in Frontiers in immunology. 2021, vol. 11, 604759
Abstract Objective: To first describe and estimate the potential pathogenic role of Ig4 autoantibodies in complement-mediated thrombotic microangiopathy (TMA) in a patient with IgG4-related disease (IgG4-RD). Methods: This study is a case report presenting a retrospective review of the patient's medical chart. Plasma complement C3 and C4 levels, immunoglobulin isotypes and subclasses were determined by nephelometry, the complement pathways' activity (CH50, AP50, MBL) using WIESLAB® Complement System assays. Human complement factor H levels, anti-complement factor H auto-antibodies were analyzed by ELISA, using HRP-labeled secondary antibodies specific for human IgG, IgG4, and IgA, respectively. Genetic analyses were performed by exome sequencing of 14 gens implicated in complement disorders, as well as multiplex ligation-dependent probe amplification looking specifically for CFH, CFHR1-2-3, and 5.
Keywords IgG4-related diseaseSARS CoV2Anti-factor H auto-antibodiesAntibodiesAtypical hemolytic uremic syndromeComplement factor HComplement factor H-related proteinThrombotic microangiopathy
Identifiers
PMID: 33643292
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Research group Laboratoire d'immunologie translationnelle (856)
Citation
(ISO format)
BREVILLE, Gautier et al. Case Report: Severe Complement-Mediated Thrombotic Microangiopathy in IgG4-Related Disease Secondary to Anti-Factor H IgG4 Autoantibodies. In: Frontiers in Immunology, 2021, vol. 11, p. 604759. doi: 10.3389/fimmu.2020.604759 https://archive-ouverte.unige.ch/unige:155532

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Deposited on : 2021-10-20

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