Systemic sclerosis spectrum from the Egyptian College of Rheumatology: a nationwide observational study

Background Systemic sclerosis (SSc) is a rare, heterogeneous autoimmune disease characterised by vasculopathy, immune activation and progressive fibrosis, resulting in multiorgan involvement and significant morbidity and mortality. Aim of the work This nationwide multicentre cross-sectional study aimed to evaluate the demographic, clinical, serological and therapeutic profiles of Egyptian SSc patients, enhancing understanding of disease patterns, comorbidities and management strategies. Methods A total of 1080 patients fulfilling the 2013 American College of Rheumatology/European League Against Rheumatism classification criteria for SSc were recruited from rheumatology centres across 18 Egyptian governorates. Data on demographics, clinical features, autoantibody profiles and treatments were collected and analysed. Results The mean patient age was 40.5±11.5 years, with a pronounced female predominance (88.4%, F: M ratio 7.6:1). Limited cutaneous SSc (lcSSc) and diffuse cutaneous SSc were equally represented, while overlap syndromes occurred in 6.7%, most frequently with rheumatoid arthritis, myositis and systemic lupus erythematosus. Juvenile-onset SSc was identified in 5%, predominantly affecting females. Pulmonary and gastrointestinal involvement were common, including interstitial lung disease and gastro-oesophageal reflux disease. Pulmonary hypertension was more prevalent in lcSSc. Autoantibody analysis showed antinuclear antibodies positivity in 57.9%, RF in 6.1%, anti-topoisomerase I (anti-Scl-70) antibody in 28.1%, anticentromere antibody (ACA) in 24.8% and double positivity for anti-Scl-70 and ACA in 6.5%, with notable gender-based differences. Overlap syndrome patients showed higher comorbidity rates and more frequent therapeutic use. Regional variations in disease patterns were also observed. Conclusions This nationwide study provides a comprehensive profile of SSc in Egypt, highlighting a predominance of female patients with disease onset in the fourth decade of life. We observed significant gender-based differences in clinical manifestations and autoantibody profiles, with prevalent organ involvement including pulmonary and gastrointestinal complications. These findings underscore the importance of early recognition, tailored management strategies, and ongoing research to improve patient outcomes in SSc.