Disease characteristics of systemic sclerosis among Egyptian patients

Introduction: Scleroderma, or systemic sclerosis (SSc), is a chronic connective tissue disease that has been classified as one of the autoimmune rheumatic diseases. The usual hallmarks of SSc are autoimmunity, inflammation, widespread small-vessel vasculopathy affecting multiple vascular beds, and progressive interstitial and vascular fibrosis in the skin and internal organs. Aim of the work: The aim of the study was to determine the disease characteristics and frequency of different clinical manifestations among Egyptian patients. Patients and methods: Seventy-five patients with SSc, all fulfilling the criteria of the American College of Rheumatology for classification of scleroderma, were selected for this study. They were being followed up in Cairo University Internal Medicine department. The patients’ data were collected by a review of their medical records. We compared the frequency of symptoms in scleroderma patients with both diffuse cutaneous and limited cutaneous sclerosis (dcSSc and lcSSc). Results: Fourteen patients out of 75 (18.7%) had dcSSc and 61/75 (81.3%) had lcSSc. We found that within the limited subtype 11/61 (18%) were male and 50/61 (82%) were female, with a male to female ratio of 1: 4.6. Within the diffuse subtype, 3/14 (21.4%) were male and 11/14 (78.6%) were female, with a male to female ratio of 1: 3.7. Raynaud’s phenomenon was the first presenting manifestation (in 77.3%), followed by arthritis (in 12%) and skin tightness (in 9.3%). Conclusion: SSc is more common in the female population than in the male population. lcSSc is more common than dcSSc.