Prevalence and clinical manifestations of Erasmus syndrome in systemic sclerosis: a cross-sectional study

• Main Authors: Soraia Azevedo, Joana Sousa-Neves, Daniela Santos-Faria, Joana Leite Silva, Joana Ramos Rodrigues, Daniela Peixoto, José Tavares-Costa, Sérgio Alcino, Carmo Afonso, Filipa Teixeira
• Format: Article
• Language: English
• Published: Sociedade Portuguesa de Reumatologia 2020-07-01
• Series: Acta Reumatológica Portuguesa
• Subjects: systemic sclerosis; disease parameters; silicosis; occupational disease; erasmus syndrome
• Online Access: http://www.actareumatologica.pt/files/article/1317_prevalence_and_clinical_manifestations_of_erasmus_syndrome_in_systemic_sclerosis_a_cross_sectional_study_file.pdf

Introduction: Erasmus syndrome (ErS) is a rare entity in which Systemic Sclerosis (SSc) develops following exposure to silica, with or without associated silicosis. The objectives of this study were: 1) to evaluate the prevalence of ErS in our SSc cohort; 2) to characterize the cases; 3) to evaluate the clinical and laboratory characteristics of SSc in patients with (Ers) or without silica exposure. Methods: Cross-sectional and analytical study. Sociodemographic, clinical and laboratory data were collected from all patients with SSc diagnosed in our department according to ACR / EULAR criteria. Data on professional activity and possible exposure to silica were obtained by phone interview. Results: Among 48 patients with SSc, the prevalence of ErS was 16.7% (8/48). All cases identified were male, corresponding to 72.7% of men with SSc followed at our department. There was a statistically significant association between ErS and male gender (p