SCLERODERMA (J VARGA, SECTION EDITOR)

Current Rheumatology Reports

, 15:297

First online:

The Immune Pathogenesis of Scleroderma: Context Is Everything

  • Matthew B. GreenblattAffiliated withDepartment of Pathology, Brigham and Women’s Hospital and Harvard Medical SchoolDepartment of Pathology, Brigham and Women’s Hospital
  • , Antonios O. AliprantisAffiliated withDepartment of Medicine, Division of Rheumatology, Allergy and Immunology, Brigham and Women’s Hospital and Harvard Medical SchoolDepartment of Medicine, Division of Rheumatology, Allergy and Immunology Email author 

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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.

Keywords

Scleroderma Systemic sclerosis Animal models Interleukin-13 Interleukin-33 Toll-like receptor 3 Interferon Immune Pathogenesis