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@article{1670607,
author = {Brodszki, Nicholas and FrazerandAbel, Ashley and Grumach, Anete S. and Kirschfink, Anete S. and Litzman, Jiří and Perez, Elena and Seppanen, Mikko R. J. and Sullivan, Kathleen E. and Jolles, Stephen},
article_location = {New York},
article_number = {4},
doi = {http://dx.doi.org/10.1007/s10875-020-00754-1},
keywords = {Complement; complement deficiencies; classical pathway; alternative pathway; mannan-binding lectin},
language = {eng},
issn = {0271-9142},
journal = {Journal of Clinical Immunology},
title = {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},
url = {https://link.springer.com/content/pdf/10.1007/s10875-020-00754-1.pdf},
volume = {40},
year = {2020}
}
TY - JOUR
ID - 1670607
AU - Brodszki, Nicholas - Frazer-Abel, Ashley - Grumach, Anete S. - Kirschfink, Anete S. - Litzman, Jiří - Perez, Elena - Seppanen, Mikko R. J. - Sullivan, Kathleen E. - Jolles, Stephen
PY - 2020
TI - 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
JF - Journal of Clinical Immunology
VL - 40
IS - 4
SP - 576-591
EP - 576-591
PB - Springer
SN - 02719142
KW - Complement
KW - complement deficiencies
KW - classical pathway
KW - alternative pathway
KW - mannan-binding lectin
UR - https://link.springer.com/content/pdf/10.1007/s10875-020-00754-1.pdf
L2 - https://link.springer.com/content/pdf/10.1007/s10875-020-00754-1.pdf
N2 - 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 similar to 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.
ER -
BRODSZKI, Nicholas, Ashley FRAZER-ABEL, Anete S. GRUMACH, Anete S. KIRSCHFINK, Jiří LITZMAN, Elena PEREZ, Mikko R. J. SEPPANEN, Kathleen E. SULLIVAN and Stephen JOLLES. 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. \textit{Journal of Clinical Immunology}. New York: Springer, 2020, vol.~40, No~4, p.~576-591. ISSN~0271-9142. Available from: https://dx.doi.org/10.1007/s10875-020-00754-1.
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