| Autor: |
Rocha LF; Lusíada Foundation School of Medicine, Santos, Brazil., Luppino Assad AP; Division of Rheumatology, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil., Marangoni RG; Division of Rheumatology, Northwestern University, Chicago, IL, USA., Del Rio AP; Unit of Rheumatology, University of Campinas, Campinas, Brazil., Marques-Neto JF; Unit of Rheumatology, University of Campinas, Campinas, Brazil., Sampaio-Barros PD; 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. |
| Abstrakt: |
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. |
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