Chronic hypersensitivity pneumonitis is a common fibrotic interstitial lung disease. The prevalence of pulmonary hypertension diagnosed by right heart catheterisation and its cardiopulmonary function findings in patients with chronic hypersensitivity pneumonitis are unknown.Consecutive symptomatic patients with chronic hypersensitivity pneumonitis were prospectively evaluated. All patients were submitted to right heart catheterisation, pulmonary function testing, a 6-min walk test, echocardiography, blood gas determination and N-terminal pro-brain natriuretic peptide analyses. Nonhypoxaemic patients also underwent incremental cardiopulmonary exercise testing. 50 patients underwent right heart catheterisation; 25 (50%) of these had pulmonary hypertension and 22 (44%) had a pre-capillary haemodynamic pattern. The patients with pre-capillary pulmonary hypertension had lower forced vital capacity (mean¡SD 50¡17% versus 69¡22% predicted, p,0.01), carbon monoxide diffusing capacity (37¡12% versus 47¡14% predicted, p,0.01), arterial oxygen tension (median (interquartile range) 59.0 (47.8-69.3) versus 73.0 (62.2-78.5) mmHg, p,0.01) and saturation after the 6-min walk test (78¡8% versus 86¡7%, p,0.01). In pre-capillary pulmonary hypertension, oxygen uptake was also lower at the anaerobic threshold (41¡11% versus 50¡8% predicted, p50.04) and at peak exercise (12.8¡1.6 versus 15.0¡2.5 mL?kg -1 ?min -1 , p50.02). Pre-capillary pulmonary hypertension is common in symptomatic chronic hypersensitivity pneumonitis and is related to interstitial lung disease severity. Additionally, pulmonary hypertension is more prevalent in hypoxaemic patients with impaired lung function and exercise capacity. @ERSpublications PH is common in chronic hypersensitivity pneumonitis and is related to interstitial lung disease severity

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Determination of potentially-reversible factors contributing to exertional dyspnea remains an unmet clinical need in chronic thromboembolic pulmonary hypertension (CTEPH). Therefore, we aimed to evaluate the influence of inspiratory muscle weakness (IMW) on exercise capacity and dyspnea during effort in patients with CTEPH. We performed a prospective cross-sectional study that included thirty-nine consecutive patients with CTEPH (48 ± 15 yrs, 61% female) confirmed by right heart catheterization that underwent an incremental cardiopulmonary exercise test, 6-minute walk test and maximum inspiratory pressure (MIP) measurement. MIP < 70%pred was found in 46% of patients. On a multiple linear regression analysis, MIP was independently associated with 6MWD and . Patients with MIP < 70% presented greater than those with MIP ≥ 70%. Additionally, they also presented stronger sensations of dyspnea throughout exercise, even when adjusted for ventilation. At rest and at different levels of exercise, mean inspiratory flow (VT/TI) was significantly higher in patients with MIP < 70%. In conclusion, IMW is associated with a rapid increase of dyspnea, higher inspiratory load and poor exercise capacity in patients with CTEPH.

Our results confirm that increased MMP9 immunoreactivity in muscle fibres is a common feature of all inflammatory myopathies and suggest that MMP2 and MMP7 cannot be implicated in the inflammatory events of FM.

Compared to patients with IPAH, patients with CTEPH present more impaired gas exchange during exercise, and PetCO abnormalities may be used to identify more clinically and hemodynamically severe cases.