TY - JOUR
T1 - A review of the alternative pathway of complement and its relation to HELLP syndrome
T2 - is it time to consider HELLP syndrome a disease of the alternative pathway
AU - Vaught, Arthur J.
AU - Braunstein, Evan
AU - Chaturvedi, Shruti
AU - Blakemore, Karin
AU - Brodsky, Robert A.
N1 - Funding Information:
The time for research and manuscript has been funded by NIH [RO1 HL133113], the Johns Hopkins Robert E. Meyerhoff Professorship, and the BIRCWH NIH [1K12HD085845-01].
Publisher Copyright:
© 2020 Informa UK Limited, trading as Taylor & Francis Group.
PY - 2022
Y1 - 2022
N2 - Complement is a part of the innate immune system with a critical role in host defense. Although essential for survival, when dysregulated or excessively triggered complement activation can cause tissue damage and drive inflammatory and immune disorders. The alternative pathway of complement (APC) is especially important for survival against infection and can be triggered by a variety of settings: infection, trauma, surgery, or pregnancy. This excessive drive of complement manifest distinctive hemolytic diseases like atypical hemolytic uremic syndrome (aHUS) and paroxysmal nocturnal hemoglobinuria (PNH). These diseases share phenotypic similarities to HELLP syndrome: a hypertensive disorder of pregnancy with hemolysis, elevated liver enzymes, and low platelets. In this manuscript, there will be a brief review of complement activation and a description of important regulator proteins. The review will further discuss pregnancy as a major trigger of the alternative pathway, and how diseases of the APC are treated during pregnancy. Finally, the similarities between HELLP syndrome and diseases of the APC will be examined.
AB - Complement is a part of the innate immune system with a critical role in host defense. Although essential for survival, when dysregulated or excessively triggered complement activation can cause tissue damage and drive inflammatory and immune disorders. The alternative pathway of complement (APC) is especially important for survival against infection and can be triggered by a variety of settings: infection, trauma, surgery, or pregnancy. This excessive drive of complement manifest distinctive hemolytic diseases like atypical hemolytic uremic syndrome (aHUS) and paroxysmal nocturnal hemoglobinuria (PNH). These diseases share phenotypic similarities to HELLP syndrome: a hypertensive disorder of pregnancy with hemolysis, elevated liver enzymes, and low platelets. In this manuscript, there will be a brief review of complement activation and a description of important regulator proteins. The review will further discuss pregnancy as a major trigger of the alternative pathway, and how diseases of the APC are treated during pregnancy. Finally, the similarities between HELLP syndrome and diseases of the APC will be examined.
KW - Alternative pathway of complement
KW - HELLP syndrome
KW - preeclampsia
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U2 - 10.1080/14767058.2020.1755650
DO - 10.1080/14767058.2020.1755650
M3 - Review article
C2 - 32338085
AN - SCOPUS:85084270051
SN - 1476-7058
VL - 35
SP - 1392
EP - 1400
JO - Journal of Maternal-Fetal and Neonatal Medicine
JF - Journal of Maternal-Fetal and Neonatal Medicine
IS - 7
ER -