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2015 ; 6
(ä): 272
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Pathogenesis of Systemic Sclerosis
#MMPMID26106387
Pattanaik D
; Brown M
; Postlethwaite BC
; Postlethwaite AE
Front Immunol
2015[]; 6
(ä): 272
PMID26106387
show ga
Systemic scleroderma (SSc) is one of the most complex systemic autoimmune
diseases. It targets the vasculature, connective tissue-producing cells (namely
fibroblasts/myofibroblasts), and components of the innate and adaptive immune
systems. Clinical and pathologic manifestations of SSc are the result of: (1)
innate/adaptive immune system abnormalities leading to production of
autoantibodies and cell-mediated autoimmunity, (2) microvascular endothelial
cell/small vessel fibroproliferative vasculopathy, and (3) fibroblast dysfunction
generating excessive accumulation of collagen and other matrix components in skin
and internal organs. All three of these processes interact and affect each other.
The disease is heterogeneous in its clinical presentation that likely reflects
different genetic or triggering factor (i.e., infection or environmental toxin)
influences on the immune system, vasculature, and connective tissue cells. The
roles played by other ubiquitous molecular entities (such as lysophospholipids,
endocannabinoids, and their diverse receptors and vitamin D) in influencing the
immune system, vasculature, and connective tissue cells are just beginning to be
realized and studied and may provide insights into new therapeutic approaches to
treat SSc.