Format

Send to

Choose Destination
Cytokine. 2011 Aug;55(2):157-64. doi: 10.1016/j.cyto.2011.04.001. Epub 2011 May 4.

Cytokine profiles in localized scleroderma and relationship to clinical features.

Author information

1
Division of Rheumatology, Children's Hospital of Pittsburgh of the University of Pittsburgh Medical Center, University of Pittsburgh, School of Medicine, Pittsburgh, PA 15224, USA. katherine.kurzinski@chp.edu

Abstract

Localized scleroderma (LS) is a disfiguring autoimmune disease of the skin and underlying tissue that mainly affects the pediatric population. Inflammation of the tissue leads to fibrosis and atrophy, causing physical and psychological disability that can continue throughout childhood into adulthood. Available therapies for LS have had variable effects and are associated with morbidity themselves. A better understanding of the pathophysiology of LS, especially during the active inflammatory phase, would lead to more directed and efficacious therapies. As in systemic sclerosis (SSc), the other form of scleroderma, T-helper (Th) cells and their associated cytokines have been suggested to contribute significantly to the pathophysiology of LS supported by the presence of cytokines from these lineages in the sera and tissue of LS patients. It is postulated that the imbalance between Th1/Th2/Th17 cell subsets drives inflammation in the early stages of disease (Th1 and Th17 predominant) and fibrosis in the later stages of scleroderma (Th2 predominant). We review the available experimental data regarding cytokines in LS and compare them to available clinical disease severity and activity features. This provides the platform to launch further investigations into the role of select cytokines in the pathogenesis of LS and to provide directed therapeutic options in the future.

PMID:
21536453
PMCID:
PMC3632442
DOI:
10.1016/j.cyto.2011.04.001
[Indexed for MEDLINE]
Free PMC Article

Supplemental Content

Full text links

Icon for Elsevier Science Icon for PubMed Central
Loading ...
Support Center