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

Breville, Gautier; Zamberg, Ido; Sadallah, Salima; Stephan, Caroline; Ponte, Belen; Seebach, Jorg Dieter

doi:10.3389/fimmu.2020.604759

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Title		Case Report: Severe Complement-Mediated Thrombotic Microangiopathy in IgG4-Related Disease Secondary to Anti-Factor H IgG4 Autoantibodies
Authors		Breville, Gautier Zamberg, Ido Sadallah, Salima Stephan, Caroline Ponte, Belen Seebach, Jorg Dieter
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 disease — SARS CoV2 — Anti-factor H auto-antibodies — Antibodies — Atypical hemolytic uremic syndrome — Complement factor H — Complement factor H-related protein — Thrombotic microangiopathy
Identifiers		DOI: 10.3389/fimmu.2020.604759 PMID: 33643292
Full text		Article (Published version) (795 Kb) - Free access Other version: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7905310/
Structures		Faculté de médecine / Section de médecine clinique / Département de médecine Faculté de médecine / Section de médecine clinique / Département d'anesthésiologie, pharmacologie, soins intensifs et urgences Faculté de médecine / Section de médecine clinique / Département des neurosciences cliniques
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