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2015 ; 1
(2
): 91-9
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Complement in Lupus Nephritis: New Perspectives
#MMPMID27536669
Bao L
; Cunningham PN
; Quigg RJ
Kidney Dis (Basel)
2015[Sep]; 1
(2
): 91-9
PMID27536669
show ga
BACKGROUND: Systemic lupus erythematosus (SLE) is an autoimmune disorder caused
by loss of tolerance to self-antigens, the production of autoantibodies and
deposition of complement-fixing immune complexes (ICs) in injured tissues. SLE is
characterized by a wide range of clinical manifestations and targeted organs,
with lupus nephritis being one of the most serious complications. The complement
system consists of three pathways and is tightly controlled by a set of
regulatory proteins to prevent injudicious complement activation on host tissue.
The involvement of the complement system in the pathogenesis of SLE is well
accepted; yet, its exact role is still not clear. SUMMARY: Complement plays dual
roles in the pathogenesis of SLE. On the one hand, the complement system appears
to have protective features in that hereditary homozygous deficiencies of
classical pathway components, such as C1q and C4, are associated with an
increased risk for SLE. On the other hand, IC-mediated activation of complement
in affected tissues is clearly evident in both experimental and human SLE along
with pathological features that are logical consequences of complement
activation. Studies in genetically altered mice have shown that lack of
complement inhibitors, such as complement factor H (CFH) or decay-accelerating
factor (DAF) accelerates the development of experimental lupus nephritis, while
treatment with recombinant protein inhibitors, such as Crry-Ig, CR2-Crry, CR2-DAF
and CR2-CFH, ameliorates the disease development. Complement-targeted drugs,
including soluble complement receptor 1 (TP10), C1 esterase inhibitor and a
monoclonal anti-C5 antibody (eculizumab), have been shown to inhibit complement
safely, and are now being investigated in a variety of clinical conditions. KEY
MESSAGES: SLE is an autoimmune disorder which targets multiple systems.
Complement is centrally involved and plays dual roles in the pathogenesis of SLE.
Studies from experimental lupus models and clinical trials support the use of
complement-targeted therapy in the treatment of SLE.
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