RESUMEN
Imaging plays a pivotal role in systemic sclerosis for both diagnosis management of pulmonary complications, and high-resolution computed tomography (HRCT) is the most sensitive technique for the evaluation of systemic sclerosis-associated interstitial lung disease (SSc-ILD). Indeed, several studies have demonstrated that HRCT helps radiologists and clinicians to make a correct diagnosis on the basis of recognised typical patterns for SSc-ILD. Most SSc patients affected by ILD have a non-specific interstitial pneumonia pattern (NISP) on HRCT scan, whilst a minority of cases fulfil the criteria for usual interstitial pneumonia (UIP). Moreover, several recent studies have demonstrated that lung ultrasound (LUS) is an emergent tool in SSc diagnosis and follow-up, although its role is still to be confirmed. Therefore, this article aims at evaluating the role of LUS in SSc screening, aimed at limiting the use of CT to selected cases.
RESUMEN
During bronchoscopy hypoxemia is commonly found and oxygen supply can be delivered by interfaces fed with high gas flows. Recently, the high-flow nasal cannula (HFNC) has been introduced for oxygen therapy in adults, but they have not been used so far during bronchoscopy in adults. Forty-five patients were randomly assigned to 3 groups receiving oxygen: 40 L/min through a Venturi mask (V40, N = 15), nasal cannula (N40, N = 15), and 60 L/min through a nasal cannula (N60, N = 15) during bronchoscopy. Gas exchange and circulatory variables were sampled before (FiO(2) = 0.21), at the end of bronchoscopy (FiO(2) = 0.5), and thereafter (V40, FiO(2) = 0.35). In 8 healthy volunteers oxygen was randomly delivered according to V40, N40, and N60 settings, and airway pressure was measured. At the end of bronchoscopy, N60 presented higher PaO(2), PaO(2)/FiO(2), and SpO(2) than V40 and N40 that did not differ between them. In the volunteers (N60) median airway pressure amounted to 3.6 cmH(2)O. Under a flow rate of 40 L/min both the Venturi mask and HFNC behaved similarly, but nasal cannula associated with a 60 L/min flow produced the better results, thus indicating its use in mild respiratory dysfunctions.
RESUMEN
BACKGROUND: Chronic thromboembolic pulmonary hypertension (CTEPH) is a progressive disease leading to worsening functional status and reduced survival for those patients who cannot undergo pulmonary endarterectomy (PEA). Pharmacotherapy with novel drugs for pulmonary hypertension may be useful in treating patients who are poor candidates for surgery, but there are still few clinical data on medical therapy for CTEPH. The aim of this prospective open-label, multicenter, study is to compare the efficacy of 1-year bosentan treatment to standard drugs in nonoperated patients with CTEPH. PATIENTS AND METHODS: Thirty-four nonoperated patients with CTEPH were enrolled. Functional assessment included 6 minute walk test (6MWT), Borg index, WHO classification, arterial blood gases and echocardiography systolic pulmonary artery pressure (sPAP). Seventeen patients received bosentan (62.5 mg b.i.d. for 4 weeks and then 125 mg b.i.d.); 17 patients were treated with standard therapy alone. RESULTS: At admission sPAP was 76.18+/-5.96 mmHg in bosentan group and 71.48+/-3.71 mmHg in controls, p(a)O(2) 64.68+/-2.25 mmHg in bosentan group, and 59.52+/-2.05 mmHg in controls, 6MWT 297.53+/-34.25 mt in bosentan group, and 268.47+/-36.54 mt in controls. After 12 months there were significant differences between the groups in the 6MWT (+57.24+/-22.21 m vs -73.13+/-21.23 m, p<0.001), dyspnoea index (Borg score 4.29+/-0.49 vs 7.06+/-0.32, p<0.001) and oxygenation (p(a)O(2) 65.93+/-3.76 mmHg vs 48.48+/-1.31 mmHg, p<0.001). The sPAP was stable after 12 months of bosentan (76.18+/-5.96 mmHg vs 71.00+/-5.41 mmHg, p=0.221) in contrast to controls (71.48+/-3.71 mmHg vs 80.44+/-4.70 mmHg, p=0.029). CONCLUSION: The data of this open-label study in nonoperated CTEPH patients suggest an improvement in functional outcomes adding Bosentan to diuretics and oral anticoagulants. No improvement was observed using only standard drugs after 1-year.