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European Society for Immunodeficiencies (ESID) and European Reference Network on Rare Primary Immunodeficiency, Autoinflammatory and Autoimmune Diseases (ERN RITA) complement guideline: deficiencies, diagnosis, and management

Brodszki, Nicholas, Frazer-Abel, Ashley, Grumach, Anete S., Kirschfink, Michael, Litzman, Jiri, Perez, Elena, Seppänen, Mikko R. J., Sullivan, Kathleen E. and Jolles, Stephen 2020. European Society for Immunodeficiencies (ESID) and European Reference Network on Rare Primary Immunodeficiency, Autoinflammatory and Autoimmune Diseases (ERN RITA) complement guideline: deficiencies, diagnosis, and management. Journal of Clinical Immunology 40 , pp. 576-591. 10.1007/s10875-020-00754-1

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Abstract

This guideline aims to describe the complement system and the functions of the constituent pathways, with particular focus onprimary immunodeficiencies (PIDs) and their diagnosis and management. The complement system is a crucial part of the innateimmune system, with multiple membrane-bound and soluble components. There are three distinct enzymatic cascade pathwayswithin the complement system, the classical, alternative and lectin pathways, which converge with the cleavage of central C3.Complement deficiencies account for ~5% of PIDs. The clinical consequences of inherited defects in the complement system areprotean and include increased susceptibility to infection, autoimmune diseases (e.g., systemic lupus erythematosus), age-relatedmacular degeneration, renal disorders (e.g., atypical hemolytic uremic syndrome) and angioedema. Modern complement analysisallows an in-depth insight into the functional and molecular basis of nearly all complement deficiencies. However, therapeuticoptions remain relatively limited for the majority of complement deficiencies with the exception of hereditary angioedema andinhibition of an overactivated complement system in regulation defects. Current management strategies for complement disor-ders associated with infection include education, family testing, vaccinations, antibiotics and emergency plannin

Item Type: Article
Date Type: Publication
Status: Published
Schools: Medicine
Publisher: Springer (part of Springer Nature): Springer Open Choice Hybrid Journals
ISSN: 0271-9142
Date of First Compliant Deposit: 21 February 2020
Date of Acceptance: 20 January 2020
Last Modified: 08 Jun 2020 12:34
URI: http://orca.cf.ac.uk/id/eprint/129854

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