الفهرس 
Review of LiteratureOnly 14 pages are availabe for public view
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Abstract
Systemic 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).The relevant radiological, laboratory and immunological investigations.
Our results revealed that the mean age at time of diagnosis was 32.66 ± 13.08 with the disease durations range from 1 to 40 years with a median of five years. Male to female ratio of 1: 5.2 and 20% of patients were smokers. 62% of patients were nonworking, 22% were governmental employees, 4% were bank accountant, 4% were students, 4% were ex-sergeants, one patient (2%) was ex-officer and another one patient (2%) was a carpenter.
Skin tightness was present in all patients, the mRSS ranges from 4 to 45 with a mean of 17.48 ± 10.44. GIT, musculoskeletal, and neuropsychiatric were detected 90% of patents. ILD and PAH were detected in 50% and 18% of patients respectively.
ANA was detected in 98%, RF was detected 4%, antitopo I was detected in 36% and ACA was detected in 8% of patients. 96% of patient had abnormal NFC.
from the 50 SSC patients, 64% of patients had LCSSC and 36% of patients had DCSSC. Puffy fingers, arthralgia and early pattern of NFC were more significantly increased in LCSSC; while digital tip ulcers, pitting scars, ILD, mRSS, antitopo I and late pattern of NFC were more significantly increased in DCSSC.
Dysphagia, dementia, mRSS, lower FEV1%, lower FVC%, higher FEV1/FVC, anti topo I and late pattern of NFC
were more significantly increased in Patients with ILD compared to those without ILD.
Older age, renal affection, dysphagia, Sicca compex, lower FEV1% and late pattern of NFC were more significantly increased in Patients with PAH compared to those without PAH.
Female sex, longer disease duration, cardiovascular affection, lower FEV1% and lower FVC% were more significantly increased in Patients with late pattern of NFC compared to other patterns
There were statistical significant negative correlations between mRSS and both of FEV1% and FVC%. There was also a statistical significant positive correlation between mRSS and FEV1/FVC.
There was significant positive correlation between anti topoisomerase I titers ant FEV1% in patients with topoisomrase I seropositivity.