Demographic characteristics and clinical manifestations of systemic sclerosis in Benghazi, Libya

Abstract

Background: Systemic sclerosis (SSc) is a rare, heterogeneous autoimmune disease characterized by skin fibrosis, vasculopathy, and internal organ involvement. This study aims to determine the frequency of clinical manifestations of SSc among Libyan patients in Benghazi. Patients and Methods: Thirty patients (28 females) attended the Rheumatology Clinic at Benghazi Medical Center between January 2016 and December 2019. They were diagnosed to have SSc according to American College of Rheumatology/European League Against Rheumatism 2013 revised classification criteria and were classified into diffuse cutaneous systemic sclerosis (dcSSc) and localized systemic sclerosis (lcSSc) disease subsets. Results: We reviewed the data of the 30 patients, including 28 (93.3%) females and 2 (6.7%) males (female: male ratio 14:1); the mean age at diagnosis was 40.5 ± 21.5 years (17–62 years). Twenty-four (80%) patients were diagnosed as dcSSc, whereas 6 (20%) were diagnosed as lcSSc. On diagnosis, patients with dcSSc were comparatively younger than those with lcSSc. The frequency of musculoskeletal manifestations and organ involvement was more frequent among patients with dcSSc (P = 0.001). All the thirty patients had rheumatoid factor and anti-nuclear antibody positivity. Anti-Scl-70 was only positive in the dcSSC subset in all patients of dcSSc only, whereas anti-centromere Ab was positive in all patients of lcSSc only. Conclusions: This is the first study to assess the clinical manifestations of SSc in the Libyan population. Our disease cohort showed similarity to what was published in other cohorts with regard to the age at the time of diagnosis, gender, and autoimmune profile.

Comprehensive description of the prevalence, serological and clinical characteristics, and visceral involvement of systemic sclerosis (scleroderma) in a large cohort from the United Arab Emirates Systemic Sclerosis Registry

“…Regarding the prevalence of the different subtypes, this study has shown that 54.5% of the patients presented with the diffuse subset, leaving only 45.5% in the lcSSc group. This finding emphasizes regional differences in populations, where Emirati patients seem to be similar to other Arab 18,19 and African American individuals (GRASP cohort), 26 exhibiting higher frequencies of the dcSSc than Caucasian 1,4,10 or Southeast Asian populations. 16,27 It could also be related to the fact that other studies included other SSc subsets and conditions, such as ssSSc, overlap syndromes, prescleroderma, and undifferentiated subsets.…”

“…1,4 Nevertheless, studies have revealed heterogeneity of the disease presentations, phenotype, and clinical variants among populations (gender, geographical, partly due to genetic and ethnic heterogeneity). 1,16,17 Reports from other Arabic countries 18,19 were mainly limited by the small sample size and heterogeneity of the included participants, which might not reflect the exact characteristics of a given population. Thus, local registries are paramount for better understanding of clinical and serological features, allowing for the implementation of patient-tailored strategies for early detection and better management and quality of care.…”