Title | The immune pathogenesis of scleroderma: context is everything. |
Publication Type | Journal Article |
Year of Publication | 2013 |
Authors | Greenblatt MB, Aliprantis AO |
Journal | Curr Rheumatol Rep |
Volume | 15 |
Issue | 1 |
Pagination | 297 |
Date Published | 2013 Jan |
ISSN | 1534-6307 |
Keywords | Animals, Autoimmune Diseases, Disease Models, Animal, Fibrosis, Genetic Predisposition to Disease, Graft vs Host Disease, Humans, Interleukin-13, Mice, Scleroderma, Systemic, Skin |
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. |
DOI | 10.1007/s11926-012-0297-8 |
Alternate Journal | Curr Rheumatol Rep |
PubMed ID | 23288576 |
Grant List | K08 AR 054859 / AR / NIAMS NIH HHS / United States |
Related Faculty:
Matthew B. Greenblatt, M.D., Ph.D.