The aim of this study was to identify risk factors associated with mortality in patients with systemic sclerosis (SSc), particularly those with interstitial lung disease (ILD), over a long-term follow-up in a large Brazilian SSc cohort. Methods:We conducted a medical records review study of 380 scleroderma patients from 1982 to 2019. Systemic sclerosis ILD was considered in those with evidence of ILD on chest high-resolution computed tomography (HRCT). Causes of death were determined.Results: Among the 380 SSc patients, SSc-ILD on chest HRCT was observed in 227 patients (59.7%). Seventy-two patients (18.9%) died during a mean follow-up of 7.2 years since the SSc diagnosis; among them, 57 (79.2%) had SSc-ILD, compared with 15 (20.8%) without SSc-ILD ( p < 0.001). Of the 72 deaths, 51.4% were considered related to SSc, and ILD was the leading cause of death. The overall survival rates at 5, 10, and 15 years were 87.9%, 81.5%, and 74.9%, respectively. Kaplan-Meier analysis showed a significantly worse prognosis among patients with SSc-ILD than among those without ILD ( p < 0.001). Among patients with SSc-ILD, disease duration of less than 4 years ( p < 0.001), forced vital capacity <80% at baseline ( p = 0.017), and pulmonary systolic arterial pressure ≥40 mm Hg on echocardiography ( p < 0.001) were significantly associated with mortality by multivariate analysis. Conclusions:In Brazilian SSc patients, the presence of ILD was associated with a worse prognosis. The higher mortality among SSc-ILD patients, especially those with a shorter disease duration and forced vital capacity <80%, highlights the need for early screening and closer monitoring before irreversible lung function deterioration occurs.

This prospective study aimed to compare vascular parameters (endothelin-1 [ET-1] blood levels, laser Doppler imaging [LDI] of distal phalanxes, and nailfold capillaroscopy) between open-angle glaucoma patients with low- and high-tension optic disc hemorrhages (LTDH and HTDH, respectively). The 33 enrolled patients (mean age, 62.3 ± 13 years) were classified as LTDH or HTDH if they presented at the time of DH detection an intraocular pressure (IOP) < 16 mmHg or ≥ 16 mmHg, respectively. Demographic and ophthalmological data, ET-1 concentrations, LDI (before and 1, 10, and 20 min after cold stimulation), and nailfold capillaroscopy findings were evaluated. The ET-1 blood level was 65% higher in the LTDH (2.27 ± 1.46 pg/ml) than in the HTDH (1.37 ± 0.57 pg/ml; p = 0.03) group. Moreover, there was a statistically significant negative correlation between ET-1 blood concentration and IOP at the time of DH detection (r = −0.45, p = 0.02). Blood flow measurements 10 and 20 min after cold stimulation were lower in the LTDH group than in the HTDH group (p < 0.01). Patients developing DH with lower IOPs have higher ET-1 blood levels and more peripheral vascular dysfunction as estimated by LDI than those with higher IOPs. These findings suggest that distinct underlying mechanisms may be involved in patients developing DH within different IOP ranges.

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Purpose: To compare vascular parameters, assessed by endothelin-1 (ET-1) blood levels, Laser Doppler Imaging (LDI) of distal phalanx, and nailfold capillaroscopy (NC), between open-angle glaucoma (OAG) patients with low (LTDH) and high-tension optic disc hemorrhages (HTDH). Methods: In this prospective study, patients were classified as LTDH if presenting an IOP<16 mmHg and HTDH if an IOP³16 mmHg at the time of DH detection. Demographic and ophthalmological data, ET-1 concentration, LDI (before and after 1, 10 and 20 minutes after cold stimulation) and NC findings were evaluated. Results: Thirty-tree patients were included (mean age, 62.3±13 years). Regarding ET-1 blood level, it was 65% higher in the LTDH group (2.27±1.46 pg/ml) compared to the HTDH (1.37±0.57 pg/ml; p=0.03). In addition, there was a significant negative correlation between ET-1 blood concentration and IOP at the time of DH detection (r=-0.45; p=0.02). Blood flow measurement in LTDH patients were lower than those observed in patients with HTDH 10 and 20 minutes after cold stimulus (p<0.01). Conclusion: Patients developing DH with lower IOPs have higher ET-1 blood levels and more peripheral vascular dysfunction (than those with higher IOPs), as estimated by LDI. These findings suggest that distinct underling mechanisms maybe involved in patients developing DH with different IOP ranges.

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