ABSTRACT
The pathogenesis of systemic sclerosis (SSc) is extremely complex and heterogeneous. Extensive research on vasculature, immune response, cytokine profile and fibroblasts has enabled understanding of major components of pathogenesis of SSc. Several population-based studies have established the association of ethnicity with development and severity of SSc. Genetic factors not only influence susceptibility, but also predispose an individual to develop different clinical phenotypes of SSc. The role of epigenetic mechanisms is being explored to understand the pathogenesis of SSc. The genome wide association studies identified strong association between single nucleotide polymorphisms in major histocompatibility complex loci and SSc indicating contribution of antigen-driven adaptive immunity in the pathogenesis of SSc. Inflammation is a critical event that is involved in the early phase of pathogenesis, progressive vascular damage and fibrosis. Chemokines are involved in early stage of pathogenesis of SSc.
