title: Systemic sclerosis and silica exposure: a rare association in a large Brazilian cohort.
abstract: The objective of this study is to describe the characteristics of patients with Erasmus syndrome (ES) in a large SSc Brazilian cohort. Nine hundred and forty-seven SSc patients attended at the Scleroderma Outpatient Clinic at two academic medical centers in Brazil and classified as SSc according to the ACR/EULAR criteria were retrospectively studied. Information on demographics, clinical, and laboratory features was obtained by chart review. ES patients had their HLA class II characterized by PCR-SSO method as available. Among the 947 SSc patients studied, nine (0.9 %) had ES. These ES patients were predominantly male (78 %) and smokers (68 %) and presented diffuse SSc (67 %). Mean time of occupational exposure to silica was 13.7 years, with mean age at onset of 47 years. Previous history of tuberculosis was referred by 33 % of the ES patients. All the ES patients presented Raynaud's phenomenon, esophageal involvement, and interstitial lung disease (ILD). Antinuclear antibodies were present in all the ES patients, while anti-topoisomerase I was positive in 44 % and no patient had anticentromere antibody. Three different HLA-DQB alleles (0506, 0305, and 0303) were observed. Compared to non-ES cases, patients with ES were associated with male gender (p < 0.001), diffuse SSc (p < 0.05), ILD (p < 0.05), positive anti-topoisomerase I antibodies (p < 0.05), and death (p < 0.05). Multivariate analysis did not confirm that silicosis is an independent risk factor for SSc. To conclude, ES was rare in this large SSc cohort, although associated with a bad prognosis.
authors.affiliation: Lusíada Foundation School of Medicine, Santos, Brazil.
authors.forename: Luiza F
authors.initials: LF
authors.lastname: Rocha
authors.affiliation: Division of Rheumatology, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil.
authors.forename: Ana Paula
authors.initials: AP
authors.lastname: Luppino Assad
authors.affiliation: Division of Rheumatology, Northwestern University, Chicago, IL, USA.
authors.forename: Roberta G
authors.initials: RG
authors.lastname: Marangoni
authors.affiliation: Unit of Rheumatology, University of Campinas, Campinas, Brazil.
authors.forename: Ana Paula Toledo
authors.initials: AP
authors.lastname: Del Rio
authors.affiliation: Unit of Rheumatology, University of Campinas, Campinas, Brazil.
authors.forename: João Francisco
authors.initials: JF
authors.lastname: Marques-Neto
authors.affiliation: Division of Rheumatology, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil. pdsampaiobarros@uol.com.br.|Disciplina de Reumatologia, Universidade de São Paulo, Avenida Dr. Arnaldo 455, sala 3142, Cerqueira César, São Paulo, SP, CEP 01246-903, Brazil. pdsampaiobarros@uol.com.br.
authors.forename: Percival D
authors.initials: PD
authors.lastname: Sampaio-Barros
mesh_terms: D000328:Adult; D001938:Brazil; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D016273:Occupational Exposure / Q000009:adverse effects*; D012189:Retrospective Studies; D012307:Risk Factors; D012595:Scleroderma, Systemic / Q000209:etiology*; D012822:Silicon Dioxide / Q000633:toxicity*; D012829:Silicosis / Q000209:etiology*
chemical_list: D012822:Silicon Dioxide
keywords: Erasmus syndrome; Occupational exposure; Silicosis; Systemic sclerosis