Systemic Lupus Erythematosus and Deficiencies of Early Components of the
Complement Classical Pathway
#MMPMID26941740
Macedo AC
; Isaac L
Front Immunol
2016[]; 7
(?): 55
PMID26941740
show ga
The complement system plays an important role in the innate and acquired immune
response against pathogens. It consists of more than 30 proteins found in soluble
form or attached to cell membranes. Most complement proteins circulate in
inactive forms and can be sequentially activated by the classical, alternative,
or lectin pathways. Biological functions, such as opsonization, removal of
apoptotic cells, adjuvant function, activation of B lymphocytes, degranulation of
mast cells and basophils, and solubilization and clearance of immune complex and
cell lysis, are dependent on complement activation. Although the activation of
the complement system is important to avoid infections, it also can contribute to
the inflammatory response triggered by immune complex deposition in tissues in
autoimmune diseases. Paradoxically, the deficiency of early complement proteins
from the classical pathway (CP) is strongly associated with development of
systemic lupus erythematous (SLE) - mainly C1q deficiency (93%) and C4 deficiency
(75%). The aim of this review is to focus on the deficiencies of early components
of the CP (C1q, C1r, C1s, C4, and C2) proteins in SLE patients.
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