Background: Systemic Sclerosis (SSc) is a chronic connective tissue disease with multi-organ involvement characterized by immune activation, inflammation, widespread small-vessel vasculopathy, progressive interstitial and vascular fibrosis in the skin and internal organs. The aim of our study was to present different clinical manifestations and the relevant radiological and laboratory investigations in El-Sharkia patients with SSc.
Methods: This is a cross-sectional study carried out in Rheumatology and Rehabilitation department, Faculty of Medicine, Zagazig University Hospitals on 60 SSc patients. These patients were subjected to history taking, clinical and rheumatological examination, measuring the dermal skin thickness by the modified Rodnan skin score (mRSS), other clinical data and investigations collected from patients' files from follow up unit.
Results: Our results revealed that the mean age was (44.50±11.17) with disease durations range from 2 to 17 years with a median of 7 years, patients were 54 females (90%) and 6 males (10%). Skin tightness, puffy fingers and raynaud phenomenon were present in all patients; mRSS (ranges 4-45). Antinuclear antibodies were positive in all patients.
Conclusion: This study has shown that all our El-Sharkia SSc patients had Anti-nuclear antibodies (ANA) positive, puffy fingers, skin tightness, raynaud phenomenon and Worsening skin score. Also, limited cutaneous systemic sclerosis (lcSSc) was more common than diffuse cutaneous systemic sclerosis (dcSSc) in our SSc patients. Abnormal pulmonary function tests, interstitial lung disease (ILD), renal and cardiac involvements are more common in dcSSc than lcSSc. Extensive clinical investigations should be considered for early diagnosis and follow up of El-Sharkia SSc patients.