Correction to: Chronic Granulomatous Disease in a Brazilian Patient Mimetizing Sarcoidosis.

The original version of abstract PO-162 "Chronic Granulomatous Disease in a Brazilian Patient Mimetizing Sarcoidosis" incorrectly listed the name of the second author as Micheli Barsioti. The correct spelling of the author's name is Michele Baziotti Man.

Systemic sclerosis and silica exposure: a rare association in a large Brazilian cohort.

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.

Cross-cultural adaptation and validation of the Brazilian version of the Scleroderma Health Assessment Questionnaire (SHAQ).

The Scleroderma Health Assessment Questionnaire (SHAQ) is a feasible multisystem specific tool that has been extensively used as an additional assessment for systemic sclerosis (SSc). The aim of this study is to cross-culturally adapt and validate the Brazilian version of the SHAQ. Construct validity was assessed based on the correlations between SHAQ and both the Medical Outcomes Survey Short Form 36 version 2 (SF-36v2™) and the Health Assessment Questionnaire Disability Index (HAQ-DI). The correlation between the SHAQ and disease severity was assessed by Spearman's correlation coefficient. The reproducibility of the SHAQ was evaluated by the intraclass correlation coefficient (ICC). Among the 151 consecutive outpatients evaluated, 59 % had limited SSc subtype. The overall disease severity visual analog scale (VAS) of the SHAQ was statistically significantly correlated to HAQ-DI, pain VAS, and the SF-36v2™ physical component summary score (r = 0.595, r = 0.612, and r = -0.582, respectively; p < 0.001). Further analysis of all SF-36v2™ components revealed statistically significant correlations between overall disease severity VAS and bodily pain (r = -0.621, p < 0.001), vitality (r = -0.544, p < 0.001), physical function (r = -0.510, p < 0.001), and role limitation-physical dimensions (r = -0.505, p < 0.001). Moreover, digestive, pulmonary, and overall disease severity VASs were statistically significantly correlated to the number of organs involved (r = 0.178, p = 0.029; r = 0.214, p = 0.008; r = 0.282, p < 0.001). We also demonstrated high reproducibility for SHAQ (ICC = 0.757, 95 % confidence interval = 0.636-0.842). The Brazilian version of the SHAQ demonstrated both construct and discriminant validities as well as good reproducibility.

Survival, causes of death, and prognostic factors in systemic sclerosis: analysis of 947 Brazilian patients.

OBJECTIVE: To analyze survival, prognostic factors, and causes of death in a large cohort of patients with systemic sclerosis (SSc). METHODS: From 1991 to 2010, 947 patients with SSc were treated at 2 referral university centers in Brazil. Causes of death were considered SSc-related and non-SSc-related. Multiple logistic regression analysis was used to identify prognostic factors. Survival at 5 and 10 years was estimated using the Kaplan-Meier method. RESULTS: One hundred sixty-eight patients died during the followup. Among the 110 deaths considered related to SSc, there was predominance of lung (48.1%) and heart (24.5%) involvement. Most of the 58 deaths not related to SSc were caused by infection, cardiovascular or cerebrovascular disease, and cancer. Male sex, modified Rodnan skin score (mRSS) > 20, osteoarticular involvement, lung involvement, and renal crisis were the main prognostic factors associated to death. Overall survival rate was 90% for 5 years and 84% for 10 years. Patients presented worse prognosis if they had diffuse SSc (85% vs 92% at 5 yrs, respectively, and 77% vs 87% at 10 yrs, compared to limited SSc), male sex (77% vs 90% at 5 yrs and 64% vs 86% at 10 yrs, compared to female sex), and mRSS > 20 (83% vs 90% at 5 yrs and 66% vs 86% at 10 yrs, compared to mRSS < 20). CONCLUSION: Survival was worse in male patients with diffuse SSc, and lung and heart involvement represented the main causes of death in this South American series of patients with SSc.