Systemic sclerosis in Egyptian population with emphasis on clinical and nailfold capillaroscopic features

To describe various demographic, clinical, laboratory, and capillaroscopic features of Egyptian patients with systemic sclerosis (SSc) and to explore the relation between various capillaroscopic features and internal organ involvement as well as other disease parameters. In this cross-sectional multi-centric prospective analysis, two hundred twenty-two adult patients with SSc were recruited. Data regarding general and rheumatological examination including the modified Rodnan skin score (MRSS), internal organ involvement and related imaging and laboratory investigations were collected. Both activity and severity indices were measured. Nail fold capillary microscopy (NFC) was performed for 144 patients. Out of 222 patients; 139 (62.6%) had limited type while 83 (37.4%) had diffuse type, middle aged females were predominant (91%). Peripheral vascular, pulmonary, gastrointestinal and general manifestations were the most frequently documented affection with high activity, severity profiles and bad prognostic features in more than half of the patients. MRSS was above 20 in 122 patients (55%), and 126 patients (56.8%) had pulmonary hypertension (SPAP) while 78.4% had ILD which showed a weak correlation to age and disease duration (r = 0.135, r = - 0.152 respectively). Significant weak correlations were found between disease duration and SPAP (r = 0.134), Medseger's severity score (r = 0.189), peripheral vascular, skin, and GIT affection severity (r = 0.210, 0.135, and 0.196 respectively). Significant difference was observed between different NFC patterns and antibodies, severity scale, some disease manifestations i.e.: general, gastrointestinal, myopathy, and digital ulceration while no significance was found between NFC patterns and the type of SSc. This study provided a comprehensive clinical and laboratory characterization of a large cohort of Egyptian patients with SSc which reflects a more severe disease with unfavorable prognostic aspects compared to the available world-wide reports. Nail fold capillaroscopy showed many associations to different aspects of the disease especially in late and active capillaroscopic features, if introduced early, it may improve the disease outcome.