Characteristics of Progressive Systemic Sclerosis in a Cohort of Egyptian Patients

Abstract

ystemic sclerosis (SSC) is a multisystem disease that is characterised by three main immunopathologcal conditions; endothelial dysfunction with resultant a small-vessel vasculopathy, fibroblast dysfunction resulting in excessive collagen production and fibrosis, and immunological abnormalities. SSC is classified according to the extent of skin involvement into, limited cutaneous systemic sclerosis (LCSSC) and diffuse cutaneous systemic sclerosis (DCSSC). Associated Organ-specific and non-organ specific impairments lead to limitations in physical, work and social activities, ultimately influencing health-related quality of life. The aim of this work was to estimate the frequency of epidemiological, clinical and laboratory characteristics of progressive systemic sclerosis in a cohort of Egyptian patients. The study included 50 SSC patients. These patients were subjected to the following protocol:- 1) Detailed history taking, clinical and rheumatological examination. 2) Measuring the dermal skin thickness by the modified Rodnan skin score (mRSS). 3) Nail fold capillaroscpy (NFC).