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Curr Rheumatol Rep. 2013 Jan;15(1):297. doi: 10.1007/s11926-012-0297-8.

The immune pathogenesis of scleroderma: context is everything.

Author information

1
Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, Boston, MA 02115, USA. matt.greenblatt@gmail.com

Abstract

The fundamental mechanisms that drive the pathogenesis of systemic sclerosis (SSc) remain elusive, despite over 50 years of investigation. Here, we review recent progress in the understanding of the immunopathogenesis of SSc. In particular, we consider interleukin-13 (IL13), and its upstream and downstream pathways, as an example of an immune system-derived mediator involved in fibrotic and vascular pathology. Emerging results linking pattern-recognition receptors and interferon pathways to SSc are also stressed. We discuss genetic data linking the immune system to SSc risk and efforts to apply animal models to subsets of patients recently resolved by gene expression profiling. These developments will help build a context for better understanding of previous observations and design of the next generation of studies that may eventually lead to effective treatment.

PMID:
23288576
PMCID:
PMC3539168
DOI:
10.1007/s11926-012-0297-8
[Indexed for MEDLINE]
Free PMC Article

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