Current Rheumatology Reports

, 15:297

The Immune Pathogenesis of Scleroderma: Context Is Everything

SCLERODERMA (J VARGA, SECTION EDITOR)

DOI: 10.1007/s11926-012-0297-8

Cite this article as:
Greenblatt, M.B. & Aliprantis, A.O. Curr Rheumatol Rep (2013) 15: 297. doi:10.1007/s11926-012-0297-8
Part of the following topical collections:
  1. Topical Collection on Scleroderma

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

SclerodermaSystemic sclerosisAnimal modelsInterleukin-13Interleukin-33Toll-like receptor 3InterferonImmunePathogenesis

Copyright information

© Springer Science+Business Media New York 2012

Authors and Affiliations

  • Matthew B. Greenblatt
    • 1
    • 3
  • Antonios O. Aliprantis
    • 2
    • 4
  1. 1.Department of PathologyBrigham and Women’s Hospital and Harvard Medical SchoolBostonUSA
  2. 2.Department of Medicine, Division of Rheumatology, Allergy and ImmunologyBrigham and Women’s Hospital and Harvard Medical SchoolBostonUSA
  3. 3.Department of PathologyBrigham and Women’s HospitalBostonUSA
  4. 4.Department of Medicine, Division of Rheumatology, Allergy and ImmunologyBostonUSA