COVID-19 has negatively affected the delivery of respiratory diagnostic services across the world due to the potential risk of disease transmission during lung function testing. Community prevalence, reoccurrence of COVID-19 surges, and the emergence of different variants of the SARS-CoV-2 virus have impeded attempts to restore services. Finding consensus on how to deliver safe lung function services for both patients attending and for staff performing the tests are of paramount importance.This international statement presents the consensus opinion of 23 experts in the field of lung function and respiratory physiology balanced with evidence from the reviewed literature. It describes a robust roadmap for restoration and continuity of lung function testing services during the COVID-19 pandemic and beyond.Important strategies presented in this consensus statement relate to the patient journey when attending for lung function tests. We discuss appointment preparation, operational and environmental issues, testing room requirements including mitigation strategies for transmission risk, requirement for improved ventilation, maintaining physical distance, and use of personal protection equipment. We also provide consensus opinion on precautions relating to specific tests, filters, management of special patient groups, and alternative options to testing in hospitals.The pandemic has highlighted how vulnerable lung function services are and forces us to re-think how long term mitigation strategies can protect our services during this and any possible future pandemic. This statement aspires to address the safety concerns that exist and provide strategies to make lung function tests and the testing environment safer when tests are required.

Introduction Pulmonary hypertension (PH) is a well-recognised complication of interstitial lung diseases (ILD), which worsens prognosis and impairs exercise capacity. Echocardiography is the most widely used, non-invasive method for PH assessment. The aim of our study was to identify the factors predictive for echocardiographic signs of PH in newly recognised ILD patients. Methods Ninety-three consecutive patients (28F/65M) with different ILD were prospectively evaluated from January 2009 to March 2014. Pulmonary function testing, 6-min walk distance (6MWD), initial and sixth minute room air oxygen saturation, NT-proBNP and echocardiography were assessed in each patient. Echocardiographic PH probability was determined according to the 2009 ESC/ERS guidelines. Results In 41 patients (Group B) increased PH possibility has been diagnosed on echocardiography, in 52 patients (Group A)-low PH probability. Most pronounced differences (p ≤ 0.0005) between groups B and A concerned: age, 6MWD, room air oxygen saturation at 6 min, DLCO and TLC/DLCO index (57.6 vs 43.8 years; 478 vs 583 m; 89.1% vs 93.4%; 54.8% predicted vs 70.5% predicted and 1.86 vs 1.44; respectively). Univariate analysis showed four-fold increased probability of PH when TLC/DLCO exceeded 1.67. A scoring system incorporating age, TLC/DLCO index, 6MWD and room air oxygen saturation at 6 min provided high diagnostic utility, AUC 0.867 (95% CI 0.792-0.867). Conclusion ILD patients with TLC/DLCO index > 1.67 have a high likelihood of PH and should undergo further evaluation. The composite model of PH prediction, including age, 6-min walk test and TLC/DLCO was highly specific for recognition of PH on echocardiography.

Background: Restriction is a typical functional abnormality in interstitial lung disease (ILD) patients, but is not always present, especially in the early stage of the disease. The greater reduction of vital capacity (VC; %pred.) than total lung capacity (TLC; %pred.) is regarded as a typical pattern of lung function disturbances in ILD patients. Study Objectives: To explore the diagnostic value of spirometry in a detection restrictive pattern the relative volume loss assessed by TLC and VC in large series of patients with a diagnosis of ILD referred for lung function tests was evaluated. Methods: Retrospective, cross-sectional analysis of pulmonary function data was done. The sampleconsisted of 1,173 patients with the diagnosis of different interstitial lung diseases investigated during a period of 5 years. Only patients without airway obstruction (normal FEV1%VC) were included. In all cases spirometry and whole body plethysmography were performed by experienced staff using MasterLab – ‘Jaeger’ equipment according to ERS standards. Reference values according to ERS guidelines were applied. Results: The mean value of TLC expressed as %pred. was significantly (p < 0.001) lower than VC in all patients (93.7 ± 18.6 vs. 98.0 ± 21.4%pred.). The frequency of abnormal (lowered) TLC results was also higher than lowered VC (22.8 vs. 17.8%). Sensitivity of VC reached 69.3% and the positive predictive value was 88.5% in detecting volume restriction as compared to TLC measurement. Conclusion: The relative loss of TLC was greater than VC in our large group of patients. Measurement of TLC should be part of functional assessment of ILD patients, irrespective of whether they present or do not present a restrictive pattern in spirometry.

Chronic lung diseases are one of the most frequent causes of pulmonary hypertension (PH). The diagnostic challenge is to differentiate PH due to chronic lung disease from pulmonary arterial hypertension (PAH) with coexisting chronic lung disease. Moreover, the dominating cause of PH may change over time, requiring the implementation of new diagnostic procedures and new treatment modalities. We present a 68-year-old female, initially diagnosed with PH in the course of interstitial lung disease, with restrictive impairment of lung function. Therapy with immunosuppressive drugs resulted in significant clinical, radiological and functional improvement. However, five years later, arthritis symptoms developed, with PH worsening, despite stable lung disease. The patient was diagnosed with PAH in the course of rheumatoid arthritis. The introduction of sildenafil resulted in marked clinical and hemodynamic responses. Long-term survival (eleven years from PH onset and five years from PAH confirmation) has been achieved, and the patient remains in good functional condition. As the differential diagnosis of PH in patients with lung diseases is complex, the cooperation of pulmonologists and cardiologists is mandatory to obtain therapeutic success.

MTX as a single agent in the treatment of sarcoidosis has proved to be a safe and effective steroid alternative. Selected patients with chronic pulmonary sarcoidosis experience definite PFT improvements after MTX treatment. There is need to search for predictors of MTX treatment effectiveness.

Introduction: Decreased diffusing capacity of the lung for carbon monoxide (DLCO) is observed in some idiopathic pulmonary arterial hypertension (IPAH) patients, but its clinical significance is uncertain. We aimed to assess clinical correlates and prognostic significance of low DLCO in IPAH patients. Material and methods:In the group of 65 IPAH patients the cut off value for low DLCO was set up based on histogram as < 55% of predicted value. Demographic data, exercise capacity, lung function tests, hemodynamic parameters and survival of the patients were compared depending on DLCO value. Results: Low DLCO was found in 18% of the patients, and it was associated with male sex, older age, worse functional status and exercise capacity, and higher prevalence of coronary artery disease. Low DLCO carried a 4-fold increase of death risk in 5-year perspective. Conclusions: Low DLCO was a marker of worse functional capacity and increased risk of death in studied IPAH patients.

Background: Hypersensitivity pneumonitis (HP) is the third, according to frequency, interstitial lung disease, with the estimated incidence rate of 1-2/100,000. In HP patients, the extensive inflammatory lesions encompassing both small airways and lung parenchyma, as well as subsequent development of lung fibrosis, may result in respiratory insufficiency and secondary pulmonary hypertension (PH). The aim of the present retrospective study was to assess the prevalence of echocardiographic signs of PH and its' clinical predictors, in newly recognized HP patients. Methods: Consecutive HP patients, recognized in single pulmonary unit between 2005 and 2017, in whom echocardiography was performed at diagnosis, entered the present study. HP diagnosis was verified in every patient according to current diagnostic recommendations. The results of high resolution computed tomography of the chest (HRCT) were re-evaluated by two independent radiologists, blinded to clinical data. Echocardiographic signs of PH were defined as pulmonary artery systolic pressure (PASP) exceeding 36 mmHg. Regression analysis was applied to calculate PH risk, and receiver operator characteristic curves (ROC) were plotted to investigate diagnostic utility of various parameters in PH prediction. Results: PASP exceeding 36 mmHg was noted in 26 out of 70 patients (37%)-with equal frequency among patients with fibrotic and non-fibrotic HP. Significant predictors of PH on echocardiography were: partial oxygen tension in arterialized capillary blood (PaO2) <69 mmHg, lung transfer capacity for carbon monoxide (TLCO) <42% of predicted, six minutes walking test (6MWT) distance <455 meters, and 6MWT desaturation rate >8%. In case of TLCO <42% of predicted, probability of PH on echocardiography was increased by five-fold, in case of 6MWT desaturation rate >8%-by four fold. Conclusions:The best predictors of PASP >36 mmHg on echocardiography in HP patients at diagnosis were: TLCO <42% and 6MWT desaturation rate >8%. Neither the presence of lung fibrosis on HRCT, nor the duration of the disease or patients age, were helpful in PH prediction.

Hypersensitivity pneumonitis (HP) is one of the interstitial lung diseases with clearly established diagnostic criteria. Nevertheless, pharmacologic treatment recommendations are still lacking. Most specialists use steroids as first-line drugs, sometimes combined with an immunosuppressive agent. Aim: The aim of the present retrospective study was to establish predictive factors for treatment success and survival advantage in HP patients. Methods: We analyzed the short-term treatment outcome and overall survival in consecutive HP patients treated with prednisone alone or combined with azathioprine. Results: The study group consisted of 93 HP patients, 54 (58%) with fibrotic HP and 39 (42%) with non-fibrotic HP. Mean (± SD) VCmax % pred. and TL,co % pred. before treatment initiation were 81.5 (±20.8)% and 48.3 (±15.7)%, respectively. Mean relative VCmax and TL,co change after 3–6 months of therapy were 9.5 (±18.8)% and 21.4 (±35.2)%, respectively. The short-term treatment outcomes were improvement in 49 (53%) patients, stabilization in 16 (17%) patients, and progression in 28 (30%) patients. Among those with fibrotic HP, improvement was noted in 19 (35%) cases. Significant positive treatment outcome predictors were fever after antigen exposure, lymphocyte count in broncho-alveolar lavage fluid (BALF) exceeding 54%, RV/TLC > 120% pred., and ill-defined centrilobular nodules in high-resolution computed tomography (HRCT). An increased eosinophil count in BALF and fibrosis in HRCT were significant negative treatment outcome predictors. The presence of fibrosis in HRCT remained significant in a multivariate analysis. A positive response to treatment, as well as preserved baseline VCmax (% pred.) and TLC (% pred.), predicted longer survival, while fibrosis in HRCT was related to a worse prognosis. Conclusion: Immunomodulatory treatment may be effective in a significant proportion of patients with HP, including those with fibrotic changes in HRCT. Therefore, future trials are urgently needed to establish the role of immunosuppressive treatment in fibrotic HP.