2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension Developed by the task force for the diagnosis and treatment of pulmonary hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS).

Background: Drug-induced interstitial lung disease (DIILD) occurs as a result of numerous agents, but the risk often only becomes apparent after the marketing authorisation of such agents. Methods: In this PRISMA-compliant systematic review, we aimed to evaluate and synthesise the current literature on DIILD. Results: Following a quality assessment, 156 full-text papers describing more than 6000 DIILD cases were included in the review. However, the majority of the papers were of low or very low quality in relation to the review question (78%). Thus, it was not possible to perform a meta-analysis, and descriptive review was undertaken instead. DIILD incidence rates varied between 4.1 and 12.4 cases/million/year. DIILD accounted for 3–5% of prevalent ILD cases. Cancer drugs, followed by rheumatology drugs, amiodarone and antibiotics, were the most common causes of DIILD. The radiopathological phenotype of DIILD varied between and within agents, and no typical radiological pattern specific to DIILD was identified. Mortality rates of over 50% were reported in some studies. Severity at presentation was the most reliable predictor of mortality. Glucocorticoids (GCs) were commonly used to treat DIILD, but no prospective studies examined their effect on outcome. Conclusions: Overall high-quality evidence in DIILD is lacking, and the current review will inform larger prospective studies to investigate the diagnosis and management of DIILD.

The phenotype and outcome of severe pulmonary hypertension in chronic obstructive pulmonary disease (COPD) is described in small numbers, and predictors of survival are unknown. Data was retrieved for 101 consecutive, treatment-naïve cases of pulmonary hypertension in COPD.Mean¡SD follow-up was 2.3¡1.9 years. 59 patients with COPD and severe pulmonary hypertension, defined by catheter mean pulmonary artery pressure o40 mmHg, had significantly lower carbon monoxide diffusion, less severe airflow obstruction but not significantly different emphysema scores on computed tomography compared to 42 patients with mild-moderate pulmonary hypertension. 1-and 3-year survival for severe pulmonary hypertension, at 70% and 33%, respectively, was inferior to 83% and 55%, respectively, for mild-moderate pulmonary hypertension. Mixed venous oxygen saturation, carbon monoxide diffusion, World Health Organization functional class and age, but not severity of airflow obstruction, were independent predictors of outcome. Compassionate treatment with targeted therapies in 43 patients with severe pulmonary hypertension was not associated with a survival benefit, although improvement in functional class and/or fall in pulmonary vascular resistance .20% following treatment identified patients with improved survival.Standard prognostic markers in COPD have limited value in patients with pulmonary hypertension. This study identifies variables that predict outcome in this phenotype. Despite poor prognosis, our data suggest that further evaluation of targeted therapies is warranted.

Purpose: To use a combination of helium-3 (3-He) magnetic resonance imaging (MRI) and proton single-shot fast spin echo (SSFSE) to compare ventilated lung volumes in groups of "healthy" smokers, smokers diagnosed with moderate chronic obstructive pulmonary disease (COPD), and never-smokers. Materials and Methods:All study participants were assessed with spirometry prior to imaging. 3-He images were collected during an arrested breath hold, after inhaling a mixture of 200 mL of hyperpolarized 3-He/800 mL of N 2 . Proton SSFSE images were acquired after inhaling 1 liter of room air. The ventilated volume for each study participant was calculated from the 3-He images, and a ratio was calculated to give a percentage ventilated lung volume.Results: Never-smokers exhibited a 90% mean ventilated volume. The mean ventilated lung volumes for healthy smokers and smokers diagnosed with COPD were 75.2% and 67.6%, respectively. No correlation with spirometry was demonstrated for either of the smoking groups. Conclusion:Combined 3-He/Proton SSFSE MRI of the lungs is a noninvasive method, using nonionizing radiation, which demonstrates ventilated airspaces and enables the calculation of ventilated lung volumes. This method appears to be sensitive to early obstructive changes in the lungs of smokers. HYPERPOLARIZED HELIUM-3 (3-He) magnetic resonance imaging (MRI) is an emerging technique, which has been shown to produce high-resolution images of ventilated human airspaces (1,2). Proton MRI of the lungs has long been regarded to be of limited use in lung imaging due to cardiac motion artifacts, low proton density, and large magnetic susceptibility gradients associated with lung tissue. However, with ongoing development of fast imaging sequences, proton MRI of the lungs is now enjoying somewhat of a renaissance (3,4). In this work single-shot fast spin echo (SSFSE) breathhold images of the lungs were used to calculate a thoracic volume (5), and 3-He ventilation images were used to calculate a ventilated volume (6 -8) for each study participant. A ratio of ventilated volume to thoracic volume was calculated to give a percentage ventilated lung volume. MATERIALS AND METHODSThe local research ethics committee gave approval, and written informed consent was obtained from each study participant. The lungs of 13 volunteers (5 male, 8 female; mean age ϭ 51; range ϭ 40 -62) and 5 patients with chronic obstructive pulmonary disease (COPD) (2 male, 3 female; mean age ϭ 53; range ϭ 47-61) were imaged using proton SSFSE MRI and 3-He MRI in the coronal plane. All participants were assessed with spirometry prior to imaging and assigned to three groups: eight healthy never-smokers, five "healthy" smokers with a smoking history of Ͼ10 pack years, and five smokers with moderate COPD as demonstrated by spirometry and clinical history. Thus, COPD was defined as a subject who is symptomatic (chronic cough and shortness of breath), and the spirometric indices are 30% Ͻ forced expiratory volume in one second (FEV1) Ͻ 80% of the predicted value in comb...