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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
N. Brodszki, A. Frazer-Abel, AS. Grumach, M. Kirschfink, J. Litzman, E. Perez, MRJ. Seppänen, KE. Sullivan, S. Jolles
Language English Country Netherlands
Document type Journal Article, Research Support, Non-U.S. Gov't
NLK
ProQuest Central
from 1997-01-01
Medline Complete (EBSCOhost)
from 2010-01-01 to 1 year ago
Health & Medicine (ProQuest)
from 1997-01-01
Public Health Database (ProQuest)
from 1997-01-01
Springer Nature OA/Free Journals
from 1981-01-01
- MeSH
- Autoimmune Diseases diagnosis genetics therapy MeSH
- Genetic Testing MeSH
- Infections MeSH
- Complement System Proteins genetics MeSH
- Humans MeSH
- Mutation genetics MeSH
- Disease Susceptibility MeSH
- Primary Immunodeficiency Diseases diagnosis genetics therapy MeSH
- Practice Guidelines as Topic MeSH
- Societies, Medical MeSH
- Patient Education as Topic MeSH
- Inflammation diagnosis genetics therapy MeSH
- Check Tag
- Humans MeSH
- Publication type
- Journal Article MeSH
- Research Support, Non-U.S. Gov't MeSH
- Geographicals
- Europe MeSH
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.
Allergy Associates of the Palm Beaches North Palm Beach FL USA
Department of Pediatrics Children's Hospital Skåne University Hospital Lund Sweden
Division of Allergy and Immunology The Children's Hospital of Philadelphia Philadelphia PA USA
Immunodeficiency Centre for Wales Cardiff University and University Hospital of Wales Cardiff UK
Institute of Immunology University of Heidelberg Heidelberg Germany
References provided by Crossref.org
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