Incidence, prevalence and clinical features of systemic sclerosis patients from a southern Brazilian city.

BACKGROUND

Systemic sclerosis (SSc) is a chronic and heterogeneous disorder characterized by vascular, inflammatory, and fibrotic changes in the skin and internal organs. As epidemiological studies on SSc in Brazil are few, most reports are from international literature. This study aimed to determine the incidence, prevalence, clinical and laboratory characteristics, and therapeutic management of patients diagnosed with SSc in Cascavel, a southern Brazilian city.

Methods

Cross-sectional study that collected data from all rheumatology services in the city (six private rheumatology clinics, a regional specialty center, a municipal specialty center, and an outpatient clinic at a university hospital) in 2020. After the epidemiological survey, eligible patients were invited to undergo investigation for possible orofacial manifestations.

Results

We identified 57 patients who were treated for SSc in clinics, 30 of whom lived in Cascavel, Brazil. The estimated prevalence of SSc was 9 ± 0.03 cases/100,000 population, and the incidence in 2020 was 1.2 ± 0.004 cases/100,000 population. Most patients were female, and the mean age at diagnosis was 42.5 ± 17.22 years. The most frequently reported clinical feature is Raynaud’s phenomenon, followed by skin thickening. Limited SSc was the SSc syndrome most frequently identified by physicians, and the antinuclear antibody test was positive in 86.66% of the patients. Six patients participated in the orofacial alteration investigation. Half of the patients had very low hyposalivation, and all had telangiectasias and periodontal disease.

Conclusions

Clinical and epidemiological data from Cascavel, Brazil corroborate the findings of previous studies. Orofacial compromise was common in patients with SSc. Increased awareness among healthcare teams managing these patients is necessary to evaluate and manage orofacial problems.