Complement deficiencies and human diseases

Rosain, Jérémie; Ngo, Stéphanie; Bordereau, Pauline; Poulain, Nelly; Roncelin, Stéphane; Martins, Paula Vieira; Dragon-Durey, Marie-Agnès; Frémeaux‐Bacchi, Véronique

doi:10.1684/abc.2014.0961

Annales De Biologie Clinique

2014

DOI: 10.1684/abc.2014.0961

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Complement deficiencies and human diseases

Jérémie Rosain

Stéphanie Ngo

Pauline Bordereau

et al.

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Cited by 4 publications

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“…LAD Table adapted from Grumach & Kirschfink[1]; additional data were obtained from Rosain et al[50]; Skattum et al[51]; Degn et al[13];…”

mentioning

confidence: 99%

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

Frazer‐Abel

Grumach³

et al. 2020

J Clin Immunol

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This guideline aims to describe the complement system and the functions of the constituent pathways, with particular focus on primary immunodeficiencies (PIDs) and their diagnosis and management. The complement system is a crucial part of the innate immune system, with multiple membrane-bound and soluble components. There are three distinct enzymatic cascade pathways within 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 are protean and include increased susceptibility to infection, autoimmune diseases (e.g., systemic lupus erythematosus), age-related macular degeneration, renal disorders (e.g., atypical hemolytic uremic syndrome) and angioedema. Modern complement analysis allows an in-depth insight into the functional and molecular basis of nearly all complement deficiencies. However, therapeutic options remain relatively limited for the majority of complement deficiencies with the exception of hereditary angioedema and inhibition of an overactivated complement system in regulation defects. Current management strategies for complement disorders associated with infection include education, family testing, vaccinations, antibiotics and emergency planning.

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“…LAD Table adapted from Grumach & Kirschfink[1]; additional data were obtained from Rosain et al[50]; Skattum et al[51]; Degn et al[13];…”

mentioning

confidence: 99%

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

Frazer‐Abel

Grumach³

et al. 2020

J Clin Immunol

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Les vacances du Facteur H

Bulteau

Kolopp‐Sarda

2017

Revue Francophone des Laboratoires

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Diagnostic biologique des déficits immunitaires primitifs

Miot

Poli

Beauvillain

et al. 2018

Revue Francophone des Laboratoires

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Le diagnostic précoce des déficits immunitaires primitifs (DIP) est un élément clé pour une prise en charge adéquate des patients, afin de limiter la survenue de complications. Les manifestations cliniques et biologiques des DIP sont variées, incluant les infections récurrentes, mais aussi des manifestations oncologiques ou auto immunes. Des examens biologiques simples, tels que l'hémogramme, le dosage pondéral des immunoglobulines et les sérologies post-vaccinales, peuvent permettre d'orienter le diagnostic étiologique. Des analyses plus spécialisées, comme le phénotypage lymphocytaire ou le dosage des sous classes d'IgG peuvent être réalisées en seconde intention. L'ensemble de ces examens nécessitent une interprétation rigoureuse en tenant notamment compte de l'âge du patient et du spectre infectieux.

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Complement deficiencies and human diseases

Cited by 4 publications

References 0 publications

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

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

Les vacances du Facteur H

Diagnostic biologique des déficits immunitaires primitifs

Contact Info

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