Update on pathophysiology of scleroderma with special reference to immunoinflammatory events.

Abstract:

:Scleroderma or systemic sclerosis (SSc) is a complex disease in which the vasculopathy and the activation of the immune system with production of inflammatory mediators lead to dysregulated fibroblast activation. The resulting excessive deposition of collagens and other extracellular matrix proteins ends in fibrosis and organ dysfunction. The cause is unknown, but environmental factors are thought to play a role by triggering abnormal responses in genetically susceptible hosts. The recent past has witnessed important advances in the definition of mechanisms that underlie the persistent activation in fibroblasts of genes involved in uncontrolled fibrosis, a hallmark of SSc. These include the preferential production of type 2 T cell cytokines in target organs, the presence of autoantibodies with fibroblast-activating capacities, the production of vasoconstrictive mediators that impact on fibroblast biosynthetic properties, the transforming growth factor-beta-related metabolic signature, and the presence of altered signaling pathways in fibroblasts. Furthermore, while no animal models recapitulate all the features of SSc, they have been instrumental for assessing the relevance of specific processes to the development of fibrosis. More importantly, some of the research findings are leading to therapies that target altered processes with the potential of changing the prognosis of some dismal aspects of the disease.

journal_name

Ann Med

journal_title

Annals of medicine

authors

Chizzolini C

doi

10.1080/07853890601098152

subject

Has Abstract

pub_date

2007-01-01 00:00:00

pages

42-53

issue

1

eissn

0785-3890

issn

1365-2060

pii

770703414

journal_volume

39

pub_type

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