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Hypersensitivity pneumonitis (HP) is an immune-mediated interstitial lung disease (ILD) relating to specific occupational, environmental or medication exposures. Disease behaviour is influenced by the nature of exposure and the host response, with varying degrees of lung inflammation and fibrosis seen within individuals. The differentiation of HP from other ILDs is important due to distinct causes, pathophysiology, prognosis and management implications. This Thoracic Society of Australia and New Zealand (TSANZ) position statement aims to provide an up-to-date summary of the evidence for clinicians relating to the diagnosis and management of HP in adults, in the Australian and New Zealand context. This document highlights recent relevant findings and gaps in the literature for which further research is required., (© 2024 The Author(s). Respirology published by John Wiley & Sons Australia, Ltd on behalf of Asian Pacific Society of Respirology.)

Background: There is increasing interest in the use of home-based monitoring in people with chronic lung diseases to improve access to care, support patient self-management, and facilitate the collection of information for clinical care and research. However, integration of home-based monitoring into clinical and research settings requires careful consideration of test performance and other attributes. There is no published guidance from professional respiratory societies to advance the science of home-based monitoring for chronic lung disease. Methods: An international multidisciplinary panel of 32 clinicians, researchers, patients, and caregivers developed a multidimensional framework for the evaluation of home-based monitoring in chronic lung disease developed through consensus using a modified Delphi survey. We also present an example of how the framework could be used to evaluate home-based monitoring using spirometry and pulse oximetry in adults with asthma, bronchiectasis/cystic fibrosis (CF), chronic obstructive pulmonary disease (COPD), and interstitial lung disease (ILD). Results: The PANACEA framework includes seven domains (test P erformance, disease m ANA gement, C ost, patient E xperience, clinician E xperience, researcher E xperience, and A ccess) to assess the degree to which home-based monitoring assessments meet the conditions for clinical and research use in chronic lung disease. Knowledge gaps and recommendations for future research of home spirometry and pulse oximetry in asthma, bronchiectasis/CF, COPD, and ILD were identified. Conclusion: The development of the PANACEA framework allows standardized evaluation of home-based monitoring in chronic lung diseases to support clinical application and future research.

Background: Progressive pulmonary fibrosis (PFF) is of substantial interest for novel pharmacotherapy discovery, but little is known about clinical trial eligibility criteria. We evaluated eligibility criteria of PPF randomized controlled trials (RCTs), their representativeness in registry patients, and forced vital capacity (FVC) changes and mortality according to trial eligibility., Methods: A systematic search was used to identify completed and in-progress phase II and III PPF RCTs. Common clinical trial eligibility criteria used in ≥60% of previous PPF RCTs were identified. The most common criteria for PPF used in RCTs ("trial-PPF criteria") and the clinical practice guideline definition of PPF ("guideline-PPF criteria") were both applied to patients enrolled in a prospective multicenter Canadian registry. Common trial eligibility criteria were tested for their frequency and association with health outcomes in registry patients who met trial-PPF and guideline-PPF criteria., Results: Ten different definitions of PPF were used in 16 RCTs. At the time of meeting PPF definitions, 50% of 864 patients with trial-PPF and 44% of 408 patients with guideline-PPF met the common trial eligibility criteria. For both definitions, trial-eligible patients had more rapid 1-year FVC decline but better transplant-free survival than trial-ineligible patients. Patients with unclassifiable interstitial lung disease (ILD) had higher proportion of trial exclusion compared to those with connective tissue disease-associated ILD and fibrotic hypersensitivity pneumonitis. Annual FVC decline (trial-PPF: -67 to -21 mL; guideline-PPF: -116 to -41 mL) and 1-year transplant-free survival (trial-PPF: 90.5 to 97.5%; guideline-PPF: 87 to 96.2%) varied in trial-eligible patients across ILD subtypes., Conclusions: Existing RCTs use a variety of definitions for PPF with eligibility criteria that have limited representativeness. FVC decline and transplant-free survival vary according to trial eligibility and ILD subtypes., (Copyright ©The authors 2024. For reproduction rights and permissions contact permissions@ersnet.org.)

Background: Lung function testing remains a cornerstone in the assessment and management of interstitial lung disease (ILD) patients. The clinical implications of the Global Lung function Initiative (GLI) reference equations and the updated interpretation strategies remain uncertain., Methods: Adult patients with ILD with baseline forced vital capacity (FVC) were included from the Australasian ILD registry and the National Healthcare Group ILD registry, Singapore.The European Coal and Steel Community and Miller reference equations were compared with the GLI reference equations to assess (a) differences in lung function percent predicted values; (b) ILD risk prediction models and (c) eligibility for ILD clinical trial enrolment., Results: Among 2219 patients with ILD, 1712 (77.2%) were white individuals. Idiopathic pulmonary fibrosis (IPF), connective tissue disease-associated ILD and unclassifiable ILD predominated.Median FVC was 2.60 (2.01-3.36) L, forced expiratory volume in 1 s was 2.09 (1.67-2.66) L and diffusing capacity of the lungs for carbon monoxide (DLCO) was 13.60 (10.16-17.60) mL/min/mm Hg. When applying the GLI reference equations, the mean FVC percentage predicted was 8.8% lower (87.7% vs 78.9%, p<0.01) while the mean DLCO percentage predicted was 4.9% higher (58.5% vs 63.4%, p<0.01). There was a decrease in 19 IPF and 119 non-IPF patients who qualified for the nintedanib clinical trials when the GLI reference equations were applied. Risk prediction models performed similarly in predicting mortality using both reference equations., Conclusion: Applying the GLI reference equations in patients with ILD leads to higher DLCO percentage predicted values and smaller lung volume percentage predicted values. While applying the GLI reference equations did not impact on prognostication, fewer patients met the clinical trial criteria for antifibrotic agents., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Rationale: Cough is a key symptom in patients with fibrotic interstitial lung disease (ILD). Objectives: This study evaluated the prevalence, longitudinal change, associations, and prognostic significance of cough severity in patients with fibrotic ILD. Methods: We included consecutive patients with idiopathic pulmonary fibrosis (IPF) and non-IPF fibrotic ILD who completed the 100-mm Cough Severity Visual Analog Scale from the prospective multicenter Canadian Registry for Pulmonary Fibrosis. Baseline cough severity and associations with patient demographics and clinical factors were determined. Relationships between baseline cough severity and health outcomes were evaluated. Measurements and Main Results: Patients with IPF ( n  = 1,061) had higher median baseline cough severity than those with non-IPF fibrotic ILD ( n  = 2,825) (24 vs. 20 mm; P  < 0.001), with worse cough associated with gastroesophageal reflux disease for both cohorts. Worse cough severity was independently associated with worse health-related quality of life at baseline, larger annualized decline in Dl CO , development of disease progression, and reduced transplant-free survival in both IPF and non-IPF fibrotic ILD cohorts. The IPF cohort (2.2 mm; 95% confidence interval, 1.6-2.9 mm) had larger annualized increments in cough severity than the non-IPF fibrotic ILD cohort (1.1 mm; 95% confidence interval, 0.8-1.4 mm; P  = 0.004). There was no difference in worsening cough over time comparing those receiving and not receiving ILD-targeted therapy or with and without lung function decline. Conclusions: Cough is common in patients with IPF and non-IPF fibrotic ILD, with increasing cough severity over time irrespective of ILD-targeted therapy. Patient-reported cough severity has prognostic implications on health-related quality of life, disease progression, and survival in fibrotic ILD.

Background: Pulmonary hypertension (PH) is a key complication in interstitial lung disease (ILD), with recent therapeutic advances., Research Question: What are the diagnostic evaluation, epidemiologic features, associated factors, prognostic significance, and outcome measures in interventional trials for PH in patients with ILD in the current literature?, Study Design and Methods: The Ovid MEDLINE, Embase, and CENTRAL databases were searched for original research evaluating PH in participants with ILD of any cause. The definition of PH was based on the investigators' criteria., Results: Three hundred two studies were included, with varying diagnostic evaluations used to define PH. Commonly used diagnostic tests were right heart catheterization (56%) and transthoracic echocardiography (50%). The pooled prevalence for PH in general populations with ILD was 36% (95% CI, 30%-42%) using right heart catheterization and 34% (95% CI, 29%-38%) using transthoracic echocardiography. Lower diffusion capacity of the lungs for carbon monoxide, worse oxygenation status, reduced exercise capacity, increased pulmonary artery to aorta ratio and pulmonary artery diameter, and elevated serum brain natriuretic peptide consistently were associated with the presence of PH in at least 60% of reported studies. The presence of PH was associated with increased symptom burden and worse prognosis. Outcome measures in interventional trials of PH in ILD focused on changes in pulmonary vascular hemodynamics and 6-min walk distance., Interpretation: PH is a common complication in ILD with significant health impacts. A standardized definition with prospective evaluation of risk-stratified assessments for PH using identified associated risk factors is warranted. Our findings provide an evidence base for validation as surrogate end points in future PH interventional trials in ILD., Trial Registry: International Prospective Register of Systematic Reviews; No.: CRD42021255394; URL: https://www.crd.york.ac.uk/prospero/., Competing Interests: Financial/Nonfinancial Disclosures The authors have reported to CHEST the following: Y. H. K. reports fellowship support from an NHMRC Investigator Grant, Austin Medical Research Foundation, and Royal Australasian College of Physicians, and grants from Boehringer Ingelheim during the conduct of the study. C. J. R. reports grants from Boehringer Ingelheim during the conduct of the study, grants and personal fees from Boehringer Ingelheim, grants and personal fees from Hoffmann-La Roche, personal fees from Veracyte, personal fees from Pliant Therapeutics, personal fees from Astra Zeneca, and personal fees from Cipla Ltd. outside the submitted work. None declared (H. L. A., M. S., R. K. C., H. H. T., A. H.)., (Copyright © 2024 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.)

Background/Objectives: Conventional oxygen therapy (COT) is the cornerstone of management for hypoxaemia associated with acute respiratory failure (ARF) in wards. COT implementation guidance is provided in local health guidance documents (LHGDs). This study aimed to identify ward-delivered adult COT implementation LHGDs in Australian health services and assess their content and accuracy. Methods: A scoping review was conducted on 1 May 2022 and updated on 19 December 2023 to identify public health services COT LHGDs. Data were extracted and analysed regarding COT initiation, monitoring, maintenance and weaning, and management of clinical deterioration. Results: Thirty-seven included LHGDs, and eleven referenced the Australian COT guidelines. A definition in the LHGDs for hypoxaemia is that any oxygen saturation (SpO 2 ) or arterial blood gas (ABG) is rare. None required ABG prior to COT initiation. Twenty-nine provided target SpO 2 aims for initiation and maintenance. Fifteen did not specify the criteria for clinical review. Nine LHGDs provided guidance on weaning. Conclusions: There was considerable variation in the structure and content of COT LHGDs in Australian health services. Variations and limited guideline concordance of LHGDs may impact the quality and safety of health care. Considerations for future research include the development and implementation of standardised core LHGD recommendations for COT, as well as conducting a national oxygen audit to better measure and benchmark the safety and quality of care.

Background: Accurate spirometry interpretation is critical in the diagnosis and management of COPD. With increasing efforts for a unified approach by the Global Lung Function Initiative (GLI), this study evaluated the application of race-specific 2012 GLI and race-neutral 2022 GLI reference equations compared with Choi's reference equations, which is derived and widely used in South Korea, for spirometry interpretation in Northeast Asian patients with COPD., Research Question: What are the effects of applying race-specific 2012 GLI, race-neutral 2022 GLI, and Choi's reference equations on the diagnosis, severity grade, and clinical outcome associations of COPD?, Study Design and Methods: Serial spirometry data from the Korea COPD Subgroup Study (KOCOSS) consisting of 3,477 patients were used for reanalysis using 2012 GLI, 2022 GLI, and Choi's reference equations. The COPD diagnosis and severity categorization, associations with disease manifestations and health outcomes, and longitudinal trajectories of lung function were determined., Results: Although there was strong concordance in COPD diagnosis comparing 2012 GLI, and 2022 GLI reference equations with Choi's reference equations, a notable portion of patients were reclassified to milder disease severity (17.0% and 23.4% for 2012 GLI and 2022 GLI reference equations, respectively). Relationships between FEV 1 % predicted values calculated using 2012 GLI, 2022 GLI, and Choi's equations with clinical outcomes including dyspnea severity, exercise capacity, health-related quality of life, and frequency of exacerbations remain consistently significant. Similar annual decline rates of FEV 1 and FVC % predicted were observed among the reference equations used, except for slower annual decline rate of FEV 1 in Choi's equation compared with 2022 GLI race-neutral equation., Interpretation: Application of GLI reference equations for spirometry interpretation in Northeast Asian patients with COPD has potential implications on disease severity grade for clinical management and trial participation, and maintains consistent significant relationships with key disease outcomes., Competing Interests: Financial/Nonfinancial Disclosures None declared., (Copyright © 2024 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.)

Background: Identification of hypoxaemia and hypercapnia is essential for the diagnosis and treatment of acute respiratory failure. While arterial blood gas (ABG) analysis is standard for PO 2 and PCO 2 measurement, venous blood gas (VBG) analysis is increasingly used as an alternative. Previous systematic reviews established that VBG reporting of PO 2 and PCO 2 is less accurate, but the impacts on clinical management and patient outcomes are unknown., Aims: This study aimed to systematically review available evidence of the clinical impacts of using ABGs or VBGs and examine the arteriovenous difference in blood gas parameters., Methods: A comprehensive search of the MEDLINE, Embase and Cochrane Library databases since inception was conducted. Included studies were prospective or cross-sectional studies comparing peripheral ABG to peripheral VBG in adult non-critical care inpatients presenting with respiratory symptoms., Results: Of 15 119 articles screened, 15 were included. No studies were found that examined clinical impacts resulting from using VBG compared to ABG. Included studies focused on the agreement between ABG and VBG measurements of pH, PO 2 , PCO 2 and HCO 3 - . Due to the heterogeneity of the included studies, qualitative evidence synthesis was performed. While the arteriovenous difference in pH and HCO 3 - was generally predictable, the difference in PO 2 and PCO 2 was more significant and less predictable., Conclusions: Our study reinforces the notion that VBG is not comparable to ABG for physiological measurements. However, a key revelation from our research is the significant lack of data regarding the clinical implications of using VBG instead of ABG, a common scenario in clinical practice. This highlights a critical knowledge gap., (© 2024 The Author(s). Internal Medicine Journal published by John Wiley & Sons Australia, Ltd on behalf of Royal Australasian College of Physicians.)

This Thoracic Society of Australia and New Zealand Guideline on the provision of home oxygen therapy in adults updates a previous Guideline from 2015. The Guideline is based upon a systematic review and meta-analysis of literature to September 2022 and the strength of recommendations is based on GRADE methodology. Long-term oxygen therapy (LTOT) is recommended for its mortality benefit for patients with COPD and other chronic respiratory diseases who have consistent evidence of significant hypoxaemia at rest (PaO2 ≤ 55 mm Hg or PaO2 ≤59 mm Hg in the presence of hypoxaemic sequalae) while in a stable state. Evidence does not support the use of LTOT for patients with COPD who have moderate hypoxaemia or isolated nocturnal hypoxaemia. In the absence of hypoxaemia, there is no evidence that oxygen provides greater palliation of breathlessness than air. Evidence does not support the use of supplemental oxygen therapy during pulmonary rehabilitation in those with COPD and exertional desaturation but normal resting arterial blood gases. Both positive and negative effects of LTOT have been described, including on quality of life. Education about how and when to use oxygen therapy in order to maximize its benefits, including the use of different delivery devices, expectations and limitations of therapy and information about hazards and risks associated with its use are key when embarking upon this treatment., (© 2024 The Author(s). Respirology published by John Wiley & Sons Australia, Ltd on behalf of Asian Pacific Society of Respirology.)

Background: Transbronchial lung cryobiopsy (TBLC) is an alternative to surgical lung biopsy for histopathological evaluation of unclassifiable interstitial lung disease (ILD) or ILD diagnosed with low confidence. This meta-analysis synthesised current literature regarding cryobiopsy diagnostic performance and safety, focusing on procedural and sampling techniques., Methods: Medline and Embase were searched on 11 April 2022. Studies included adults with unclassifiable ILD, reporting diagnostic yield, complications and methodological techniques of TBLC. Meta-analyses were performed for diagnostic yield, pneumothorax and bleeding. Subgroup analyses and meta-regression assessed methodological variables. PROSPERO registration: CRD42022312386., Results: 70 studies were included with 6183 participants. Diagnostic yield of TBLC was 81% (95% CI 79-83%, I 2 =97%), with better yield being observed with general anaesthesia (p=0.007), ILD multidisciplinary meeting prior to cryobiopsy (p=0.02), 2.4 mm cryoprobe (p=0.04), higher mean forced vital capacity (p=0.046) and higher mean diffusing capacity for carbon monoxide (p=0.023). Pneumothorax rate was 5% (95% CI 4-5%, I 2 =91%), with higher rates associated with a 2.4 mm cryoprobe (p<0.00001), routine post-procedure imaging (p<0.00001), multiple lobe sampling (p<0.0001), reduced mean diffusing capacity for carbon monoxide (p=0.028) and general anaesthesia (p=0.05). Moderate-to-severe bleeding rate was 12% (11-14%, I 2 =95%) and higher rates were associated with a 2.4 mm cryoprobe (p=0.001) and bleeding score selection (p=0.04)., Interpretation: Patient characteristics and modifiable factors, including procedural methods and anaesthetic techniques, impacted diagnostic yield and safety outcomes of TBLC in people with unclassifiable ILD and contributed to heterogeneity of clinical outcomes. These variables should be considered for individualised clinical decision making and guideline development and warrant routine reporting in future research., Competing Interests: Conflict of interest: J.A. Lachowicz reports grants from University of Melbourne, and payment or honoraria for lectures, presentations, manuscript writing or educational events from AstraZeneca. N.E. Smallwood reports grants from NHMRC, MRFF, Cancer Council Australia, Fisher & Paykel Healthcare (FPH), Windermere Foundation, Lung Foundation Australia and Lord Mayor's Foundation Melbourne, consultancy fees from The Limbic and Orchard Consulting, payment or honoraria for lectures, presentations, manuscript writing or educational events from GlaxoSmithKline, Boehringer Ingelheim, AstraZeneca, FPH and Health Ed, support for attending meetings from Chiesi and Boehringer Ingelheim, leadership roles with the Thoracic Society of Australia and New Zealand (Board Director and past state president), Victorian Doctors’ Program (Board Director), European Respiratory Society (Co-chair guidelines committee), and receipt of equipment, materials, drugs, medical writing, gifts or other services from FPH. J.D. Prasad, P. Patel and C. Voutier have nothing to disclose. Y.H. Khor reports grants from NHMRC, MRFF, Air Liquide Healthcare, Austin Medical Research Foundation, Lung Foundation Australia/Thoracic Society of Australia and New Zealand and RACP, and leadership roles with the Thoracic Society of Australia and New Zealand (Board director, Special Interest Group Convenor), American Thoracic Society (Clinical Problems Assembly Program Committee; guideline methodologist) and European Respiratory Society (Associate editor for European Respiratory Journal). D.P. Steinfort reports grants from the Australian National Health and Medical Research Council (Leadership Investigator Grant (#2008317))., (Copyright ©The authors 2024.)

Background and Objective: There is currently no self-management package designed to meet the needs of people with pulmonary fibrosis (PF). This study evaluated the feasibility and acceptability of a PF-specific self-management package., Methods: Adults with PF were randomly allocated (1:1) to either receive the self-management package with healthcare professional (HCP) support or standardised PF information. Primary outcomes were feasibility and acceptability of the intervention. Secondary outcomes included health-related quality of life, self-efficacy, breathlessness, daily steps, use of PF-related treatments, and healthcare utilisation. Participants' experiences of using the package were explored using qualitative interviews., Results: Thirty participants were included. Recruitment rate was 91% and 100% of those recruited were randomised. Eighty-seven percent of participants who received the package read ≥1 module and set a goal. Secondary outcomes were feasible to collect with high assessment completion rates (87%). Most participants reported the package was easy to use and enhanced knowledge, but suggested some improvements, while HCP support was highly valued., Conclusion: A PF-specific self-management package was feasible to deliver and requires further testing in a trial powered to detect changes in clinical outcomes., Innovation: This is the first self-management package designed specifically for people with PF, informed by patient experience and expert consensus., Competing Interests: The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: Yet H. Khor reports a relationship with 10.13039/501100000925National Health and Medical Research Council that includes: funding grants. Yet H. Khor reports a relationship with Medical Research Future Fund that includes: funding grants. Yet H. Khor reports a relationship with Air Liquide Healthcare that includes: non-financial support. Yet H. Khor reports a relationship with 10.13039/501100006630Lung Foundation Australia that includes: funding grants. Yet H. Khor reports a relationship with 10.13039/501100001247Thoracic Society of Australia and New Zealand that includes: funding grants. Yet H. Khor reports a relationship with The Royal Australasian College of Physicians that includes: funding grants. Co-author is a board director (Special Interest Group Convenor) at the Thoracic Society of Australia and New Zealand; a guideline methodologist (Clinical Problems Assembly Program Committee) at the 10.13039/100001465American Thoracic Society; and an associate editor for the European Respiratory Journal, European Respiratory Society - Y.H.K. If there are other authors, they declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (© 2024 The Authors.)

Background: Fibrotic interstitial lung diseases (fILDs) are a heterogeneous group of lung diseases associated with significant morbidity and mortality. Despite a large increase in the number of clinical trials in the last 10 years, current regulatory-approved management approaches are limited to two therapies that prevent the progression of fibrosis. The drug development pipeline is long and there is an urgent need to accelerate this process. This manuscript introduces the concept and design of an innovative research approach to drug development in fILD: a global Randomised Embedded Multifactorial Adaptive Platform in fILD (REMAP-ILD)., Methods: Description of the REMAP-ILD concept and design: the specific terminology, design characteristics (multifactorial, adaptive features, statistical approach), target population, interventions, outcomes, mission and values, and organisational structure., Results: The target population will be adult patients with fILD, and the primary outcome will be a disease progression model incorporating forced vital capacity and mortality over 12 months. Responsive adaptive randomisation, prespecified thresholds for success and futility will be used to assess the effectiveness and safety of interventions. REMAP-ILD embraces the core values of diversity, equity, and inclusion for patients and researchers, and prioritises an open-science approach to data sharing and dissemination of results., Conclusion: By using an innovative and efficient adaptive multi-interventional trial platform design, we aim to accelerate and improve care for patients with fILD. Through worldwide collaboration, novel analytical methodology and pragmatic trial delivery, REMAP-ILD aims to overcome major limitations associated with conventional randomised controlled trial approaches to rapidly improve the care of people living with fILD., Competing Interests: Competing interests: LK-D—research grants from Boehringer Ingelheim and Bristol-Myers-Squibb, research grant from the Brazilian Ministry of Health (PROADI-SUS), non-financial research support from Fisher & Paykel; personal fees from Sarava and Boehringer Ingelheim. TK—personal fees from Boehringer Ingelheim, United Therapeutics, Puretech and Veracyte. CJR—research grant from Boehringer Ingelheim; personal fees from Boehringer Ingelheim, Pliant Therapeutics, AstraZeneca, Trevi Therapeutics, Hoffmann La Roche and Cipla. PR-O—research grants from Boehringer Ingelheim, Hoffmann La Roche, CSL Behring, FibroGen, Vicore Pharma, Gilead, Galecto and Chiesi; personal fees from Boehringer Ingelheim, Hoffmann La Roche, Cipla, Tecnofarma, Respiratory Effectiveness Group and The Limbic. NC—personal fees from Boehringer Ingelheim, Liminal Biosciences, Vicor Pharma, Bridge Biotherapeutics and Transcrip. MF-C—research grants from CSL Behring, Boehringer Ingelheim and Roche; personal fees from Boehringer Ingelheim, MSD, AstraZeneca and GSK. A-MH-V—research grants from Boehringer Ingelheim and Janssen; personal fees from ARXX, Boehringer Ingelheim, Janssen, Medscape, Roche, Genentech, Bayer, Lilly and Merck Sharp & Dohme. KAJ—research grants from University Hospital Foundation and Three Lakes Foundation; personal fees from Boehringer Ingelheim, Pliant, Thyron, Brainomix and Hoffman La Roche. YHK—research grants from NHMRC, MRFF, Air Liquide Healthcare, Austin Medical Research Foundation, Lung Foundation Australia/Thoracic Society of Australia and New Zealand, and RACP. SBM—research grants from Three Lakes Foundation, NIH/NHLBI, Merck, Boehringer Ingelheim, Pliant Therapeutics, American Thoracic Society, and National Scleroderma Foundation; personal fees from Wolters Kluwer, Roche, DevPro Biopharma, Gilead, Accendatech, Cowen and APIE Therapeutics. LP—research grants from Janssen and Ferrer; personal fees from Janssen, Ferrer, United Therapeutics, MSD and Liquidia. HP—research grants from Boehringer Ingelheim, AstraZeneca and Siemens; personal fees from Boehringer Ingelheim, Sanofi, Janssen, MSD, AstraZeneca and Chiesi. MM-M—research grants from Boehringer Ingelheim and Roche; personal fees from Boehringer Ingelheim, Ferrer and Chiesi. JST—research grant from Boehringer Ingelheim; personal fees from Boehringer Ingelheim and Aflofarm. SR—personal fees from Cipla and Boehringer Ingelheim. JJ—research grants from Gilead, Microsoft research and GSK; personal fees from Boehringer Ingelheim, Roche, GSK and Takeda. DR—research grants from NIHR; personal fees from OMass Therapeutics, Sosei Heptares and GSK. LGS—personal fees from Daiichi Sankyo and Chiesi. MK—research grants from Boehringer Ingelheim and Roche; personal fees from Nichtraucherhelden/Sanero, Boehringer Ingelheim and Roche. NK—research grants from Veracyte, Boehringer Ingelheim, BMS and non-financial support from AstraZeneca; scientific founder at Thyron; personal fees from Boehringer Ingelheim, Pliant, AstraZeneca, RohBar, Veracyte, Augmanity, CSL Behring, Splisense, Galapagos, Fibrogen, GSK, Merck and Thyron; and reports Equity in Pliant and Thyron. RL—senior consultant for Berry Consultants. WA—personal fees from Boehringer Ingelheim. GJ—research grants from AstraZeneca, Biogen, Galecto, GSK, Nordic Biosciences, RedX and Pliant; personal fees from Apollo Therapeutics, AstraZeneca, Brainomix, Bristol-Myers-Squibb, Chiesi, Cohbar, Daewoong, Veracyte, GSK, Resolution Therapeutics, Pliant, Hoffmann La Roche, PatientMPower, Pinsent Masons, Galapagos and Vicore Pharma. BGB, IB-V, BW, TJ, MQ and VC have no conflicts to declare., (© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: High-flow nasal oxygen therapy (HFNO) is used in diverse hospital settings to treat patients with acute respiratory failure (ARF). This systematic review aims to summarise the evidence regarding any benefits HFNO therapy has compared with conventional oxygen therapy (COT) for patients with ARF., Methods: Three databases (Embase, Medline and CENTRAL) were searched on 22 March 2023 for studies evaluating HFNO compared with COT for the treatment of ARF, with the primary outcome being hospital mortality and secondary outcomes including (but not limited to) escalation to invasive mechanical ventilation (IMV) or non-invasive ventilation (NIV). Risk of bias was assessed using the Cochrane risk-of-bias tool (randomised controlled trials (RCTs)), ROBINS-I (non-randomised trials) or Newcastle-Ottawa Scale (observational studies). RCTs and observational studies were pooled together for primary analyses, and secondary analyses used RCT data only. Treatment effects were pooled using the random effects model., Results: 63 studies (26 RCTs, 13 cross-over and 24 observational studies) were included, with 10 230 participants. There was no significant difference in the primary outcome of hospital mortality (risk ratio, RR 1.08, 95% CI 0.93 to 1.26; p=0.29; 17 studies, n=5887) between HFNO and COT for all causes ARF. However, compared with COT, HFNO significantly reduced the overall need for escalation to IMV (RR 0.85, 95% CI 0.76 to 0.95 p=0.003; 39 studies, n=8932); and overall need for escalation to NIV (RR 0.70, 95% CI 0.50 to 0.98; p=0.04; 16 studies, n=3076). In subgroup analyses, when considering patients by illness types, those with acute-on-chronic respiratory failure who received HFNO compared with COT had a significant reduction in-hospital mortality (RR 0.58, 95% CI 0.37 to 0.91; p=0.02)., Discussion: HFNO was superior to COT in reducing the need for escalation to both IMV and NIV but had no impact on the primary outcome of hospital mortality. These findings support recommendations that HFNO may be considered as first-line therapy for ARF., Prospero Registration Number: CRD42021264837., Competing Interests: Competing interests: YHK reports in-kind trial support from Air Liquide Healthcare, outside this project. NS reports receiving grant funding from Fisher & Paykel Healthcare for an investigator-initiated research study outside this project. The remaining authors have nothing to disclose., (© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Introduction: Home oxygen therapy is one of the few interventions that can improve survival in patients with chronic obstructive pulmonary disease (COPD) when administered appropriately, although it may cause side effects and be an unnecessary burden for some patients., Areas Covered: This narrative review summarizes the current literature on the assessment of hypoxemia, different types of home oxygen therapy, potential beneficial and adverse effects, and emerging research on home oxygen therapy in COPD. A literature search was performed using MEDLINE and EMBASE up to January 2024, with additional articles being identified through clinical guidelines., Expert Opinion: Hypoxemia is common in patients with more severe COPD. Long-term oxygen therapy is established to prolong survival in patients with chronic severe resting hypoxemia. Conversely, in the absence of chronic severe resting hypoxemia, home oxygen therapy has an unclear or conflicting evidence base, including for palliation of breathlessness, and is generally not recommended. However, beneficial effects in some patients cannot be precluded. Evidence is emerging on the optimal daily duration of oxygen use, the role of high-flow and auto-titrated oxygen therapy, improved informed decision-making, and telemonitoring. Further research is needed to validate novel oxygen delivery systems and monitoring tools and establish long-term effects of ambulatory oxygen therapy in COPD.

Acute hypoxemic respiratory failure (ARF) is a common cause for hospital admission. High-flow nasal oxygen (HFNO) is increasingly used as a first-line treatment for patients with ARF, including in medical wards. Clinical guidance is crucial when providing HFNO, and health services use local health guidance documents (LHGDs) to achieve this. It is unknown what hospital LHGDs recommend regarding ward administration of HFNO. This study examined Australian hospitals' LHGDs regarding ward-based HFNO administration to determine content that may affect safe delivery. A scoping review was undertaken on 2 May 2022 and updated on 29 January 2024 to identify public hospitals' LHGDs regarding delivery of HFNO to adults with ARF in medical wards in two Australian states. Data were extracted and analysed regarding HFNO initiation, monitoring, maintenance and weaning, and management of clinical deterioration. Of the twenty-six included LHGDs, five documents referenced Australian Oxygen Guidelines. Twenty LHGDs did not define a threshold level of hypoxaemia where HFNO use was recommended over conventional oxygen therapy. Thirteen did not provide target oxygen saturation ranges whilst utilising HFNO. Recommendations varied regarding maximal levels of inspired oxygen and flow rates in the medical ward. Eight LHGDs did not specify any system to identify and manage deteriorating patients. Five LHGDs did not provide guidance for weaning patients from HFNO. There was substantial variation in the LHGDs regarding HFNO care for adult patients with ARF in Australian hospitals. These findings have implications for the delivery of high-quality, safe clinical care in hospitals.

Background: Acute exacerbations of idiopathic pulmonary fibrosis (AE-IPF) increases mortality risk, but which factors increase mortality is unknown. We aimed to perform a prognostic review of factors associated with mortality in patients with IPF., Study Design: and methods: We searched MEDLINE, EMBASE, and CINAHL for studies that reported on the association between any prognostic factor and AE-IPF. We assessed risk of bias using the QUIPS tool. We conduced pairwise meta-analyses using REML heterogeneity estimator, and GRADE approach to assess the certainty of the evidence., Results: We included 35 studies in our analysis. We found that long-term supplemental oxygen at baseline (aHR 2.52 [95 % CI 1.68 to 3.80]; moderate certainty) and a diagnosis of IPF compared to non-IPF ILD (aHR 2.19 [95 % CI 1.22 to 3.92]; moderate certainty) is associated with a higher risk of death in patients with AE-IPF. A diffuse pattern on high resolution computed tomography (HRCT) compared to a non-diffuse pattern (aHR 2.61 [95 % CI 1.32 to 2.90]; moderate certainty) is associated with a higher risk of death in patients with AE-IPF. We found that using corticosteroids prior to hospital admission (aHR 2.19 [95 % CI 1.26 to 3.82]; moderate certainty) and those with increased neutrophils (by % increase) in bronchoalveolar lavage (BAL) during the exacerbation is associated with a higher risk of death (aHR 1.02 [1.01 to 1.04]; moderate certainty)., Interpretation: Our results have implications for healthcare providers in making treatment decisions and prognosticating the clinical trajectory of patients, for researchers to design future interventions to improve patient trajectory, and for guideline developers in making decisions about resource allocation., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 Elsevier Ltd. All rights reserved.)

Background: People with interstitial lung disease (ILD) were deemed more vulnerable to the SARS-CoV-2 virus and isolated as a means of reducing risk of infection. This study examined the impact of the pandemic on daily life, psychological wellbeing and access to healthcare and identified approaches undertaken to remain safe., Methods: Four specialist clinics in tertiary centres in Australia (Victoria: two sites; New South Wales: one site; Western Australia: one site) recruited patients with ILD during an 8-week period from March 2021. Semi-structured telephone interviews were conducted with transcripts analysed using principles of grounded theory., Results: Ninety participants were interviewed between April and December 2021. Participants were predominantly female, former smokers with an average age of 66 years. IPF and connective tissue-ILD being the most common subtypes. Five main themes were identified: vulnerability reduced social interaction and isolation, access to healthcare services and support, staying active, emotional and psychological impact. Self-management strategies included staying active both physically and mentally., Discussion: Self-management was key to managing the impact of the pandemic. In combination with advances in technology, implementation of strategies for monitoring wellbeing and support for self-management provides an opportunity to leverage the lessons learnt to ensure a more individualised model of care for people with ILD., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Rationale: Hypoxemia in fibrotic interstitial lung disease (ILD) indicates disease progression and is of prognostic significance. The onset of hypoxemia signifies disease progression and predicts mortality in fibrotic ILD. Accurately predicting new-onset exertional and resting hypoxemia prompts appropriate patient discussion and timely consideration of home oxygen. Objectives: We derived and externally validated a risk prediction tool for both new-onset exertional and new-onset resting hypoxemia. Methods: This study used ILD registries from Canada for the derivation cohort and from Australia and the United States for the validation cohort. New-onset exertional and resting hypoxemia were defined as nadir oxyhemoglobin saturation < 88% during 6-minute-walk tests, resting oxyhemoglobin saturation < 88%, or the initiation of ambulatory or continuous oxygen. Candidate predictors included patient demographics, ILD subtypes, and pulmonary function. Time-varying Cox regression was used to identify the top-performing prediction model according to Akaike information criterion and clinical usability. Model performance was assessed using Harrell's C-index and goodness-of-fit (GoF) likelihood ratio test. A categorized risk prediction tool was developed. Results: The best-performing prediction model for both new-onset exertional and new-onset resting hypoxemia included age, body mass index, a diagnosis of idiopathic pulmonary fibrosis, and percent predicted forced vital capacity and diffusing capacity of carbon monoxide. The risk prediction tool exhibited good performance for exertional hypoxemia (C-index, 0.70; GoF, P  = 0.85) and resting hypoxemia (C-index, 0.77; GoF, P  = 0.27) in the derivation cohort, with similar performance in the validation cohort except calibration for resting hypoxemia (GoF, P  = 0.001). Conclusions: This clinically applicable risk prediction tool predicted new-onset exertional and resting hypoxemia at 6 months in the derivation cohort and a diverse validation cohort. Suboptimal GoF in the validation cohort likely reflected overestimation of hypoxemia risk and indicated that the model is not flawed because of underestimation of hypoxemia.

Background: The most effective method for encouraging self-management in individuals with pulmonary fibrosis (PF) is unclear. This review aimed to identify common self-management components, the outcome measures used and the impact of these components in PF., Methods: A scoping review was conducted according to the Joanna Briggs Institute Manual for Evidence Synthesis using Medline, Embase, PsychInfo, CINAHL and the Cochrane Central Register of Controlled Trials. Eligible studies included those with educational, behavioural or support components aimed at facilitating self-management among adults with PF and employed quantitative and/or qualitative methods., Results: 87 studies were included. Common self-management components included education (78%), managing physical symptoms (66%) and enhancing psychosocial wellbeing (54%). Components were predominantly delivered in a pulmonary rehabilitation setting (71%). No studies tested a PF-specific self-management package. Common outcome measures were 6-min walk distance (60%), St George's Respiratory Questionnaire (37%) and the Medical Research Council Dyspnoea scale (34%). Clinically significant improvements in these outcomes were seen in ≥50% of randomised controlled trials. Qualitative data highlighted the importance of healthcare professional and peer support and increased confidence in managing PF., Conclusion: Self-management components are commonly incorporated into pulmonary rehabilitation programmes rather than being offered as standalone packages. Future research should focus on testing PF-specific self-management packages and employ standardised outcome assessments that include self-efficacy and health-related behaviours., Competing Interests: Conflict of interest: J.Y.T. Lee has nothing to disclose. G. Tikellis has nothing to disclose. L. Dowman has nothing to disclose. A.W. Jones reports funding from a Lung Foundation Australia Boehringer Ingelheim Fellowship and unpaid committee positions with Thoracic Society of Australia and New Zealand. M. Hoffman has nothing to disclose. C.R. Mellerick has nothing to disclose. C. Malaguti has nothing to disclose. Y.H. Khor reports grants from National Health and Medical Research Council, Medical Research Future Fund, Lung Foundation Australia, and Austin Medical Research Foundation, and nonfinancial support from Air Liquide Healthcare during the conduct of the study, outside the submitted work. A.E. Holland reports grants from National Health and Medical Research Council and Medical Research Future Fund; nonfinancial support from Air Liquide Healthcare during the conduct of the study, outside the submitted work; and unpaid positions with Thoracic Society of Australia and New Zealand and Lung Foundation Australia., (Copyright ©The authors 2023.)

Introduction: Psoriatic arthritis is a chronic, autoimmune inflammatory arthritis that occurs with psoriasis and has profound impact on patients' physical and psychological well-being. This study aims to determine the prevalence and risk factors associated with psoriatic arthritis among patients with psoriasis., Methods: A single-center, cross-sectional study was conducted over a 12-month period at the Dermatology Clinic, Hospital Pulau Pinang, Malaysia involving all consecutive psoriasis patients. CASPAR (ClASsification of Psoriatic ARthritis) criteria were used to diagnose psoriatic arthritis., Results: A total of 360 patients with psoriasis were recruited, of whom 107 (29.7%) had psoriatic arthritis. Psoriatic arthritis patients had equal gender distribution and the mean age of arthritis onset was 40.7 ± 12.8 years. Psoriasis preceded arthritis in 81.3% of patients (n = 87) with a mean latency interval of 10.5 years. Polyarthropathy was the predominant subtype affecting 46.8% (n = 50) of patients, followed by oligoarthropathy (22.4%, n = 24), axial joint disease (5.6%, n = 6), predominant distal interphalangeal joint disease (2.8%, n = 3), and mixed subtype (22.4%, n = 24). Enthesitis and dactylitis occurred in 12.1% (n = 13) and 20.6% (n = 22) of arthritis patients, respectively, and deformity was present in 37.4% (n = 40). Psoriatic arthritis was significantly associated with being an ever smoker (adjusted odds ratio [aOR] 0.41; 95% confidence interval [CI] 0.18-0.91, p = .029), genital psoriasis (aOR 2.25; 95% CI 1.17-4.33, p = .015), and increased erythrocyte sedimentation rate (ESR) (aOR 1.02; 95% CI 1.01-1.04, p = .005) and C-reactive protein [CRP] (aOR 1.04; 95% CI 1.00-1.08, p = .040)., Conclusion: Our study showed a high prevalence of psoriatic arthritis among the psoriasis cohort. Genital involvement, and increased ESR and CRP were associated with psoriatic arthritis among patients with psoriasis., (© 2023 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.)

Background: Pulmonary hypertension (PH) is an important complication of interstitial lung disease (ILD), as its development confers a poor prognosis. There are no specific recommendations for methods of assessment for PH in ILD populations., Aims: To determine current assessment practices for PH in an Australian ILD centre., Methods: In the Austin Health ILD database, 162 consecutive patients with idiopathic pulmonary fibrosis or connective tissue disease-associated ILD were identified and retrospectively evaluated for methods of PH assessment with transthoracic echocardiography (TTE), serum N-terminal pro-brain natriuretic peptide (NT-proBNP) and right heart catheterisation (RHC) in relation to patient demographic and physiological parameters., Results: The median follow-up was 30 (14.4-56.4) months. At baseline, vital capacity was 80.0 ± 18.4% predicted, and diffusing capacity for carbon monoxide was 59.6 ± 15.2% predicted. Evaluation for PH was performed in 147 (90.7%) patients, among whom 105 (64.8%) had TTE performed at least once. At the initial TTE, 33.7% patients had high probability of PH, defined as RVSP >40 mmHg + RAp and/or right ventricular dysfunction. At the time of the most recent TTE, these criteria were met in 45 (52.3%) patients. Elevated serum NT-proBNP levels during the first year were observed in 47 (38.8%) patients. Only 14 (8.6%) patients had RHC., Conclusion: Our institutional PH assessment practice in ILD demonstrates a substantial prevalence of probable PH at baseline. As new therapies emerge for the treatment of PH in ILD, well-defined screening practices are important in this population for early identification and optimal management., (© 2022 The Authors. Internal Medicine Journal published by John Wiley & Sons Australia, Ltd on behalf of Royal Australasian College of Physicians.)

Interstitial lung disease (ILD) is a diverse group of inflammatory and fibrotic lung conditions causing significant morbidity and mortality. A multitude of factors beyond the lungs influence symptoms, health-related quality of life, disease progression and survival in patients with ILD. Despite an increasing emphasis on multidisciplinary management in ILD, the absence of a framework for assessment and delivery of comprehensive patient care poses challenges in clinical practice. The treatable traits approach is a precision medicine care model that operates on the premise of individualised multidimensional assessment for distinct traits that can be targeted by specific interventions. The potential utility of this approach has been described in airway diseases, but has not been adequately considered in ILD. Given the similar disease heterogeneity and complexity between ILD and airway diseases, we explore the concept and potential application of the treatable traits approach in ILD. A framework of aetiological, pulmonary, extrapulmonary and behavioural and lifestyle treatable traits relevant to clinical care and outcomes for patients with ILD is proposed. We further describe key research directions to evaluate the application of the treatable traits approach towards advancing patient care and health outcomes in ILD., Competing Interests: Conflict of interest: Y.H. Khor reports grants from NHMRC (investigator grant), during the conduct of the study; nonfinancial support from Air Liquide Healthcare, outside the submitted work. V. Cottin reports an unrestricted grant from Boehringer Ingelheim (paid to institution), consulting fees from AstraZeneca, Boehringer Ingelheim, Celgene/BMS, CSL Behring, Ferrer/United Therapeutics, GSK, Pliant, Pure Tech, RedX, Roche, Sanofi and Shionogi, fees for lectures and consulting from Boehringer Ingelheim, Ferrer/United Therapeutics and Roche, support for attending meetings from Boehringer Ingelheim and Roche, participation on a data and safety monitoring board for Galapagos and Galecto, and participation on a an adjudication committee for Fibrogen, outside the submitted work. A.E. Holland has nothing to disclose. Y. Inoue reports grants from Japanese Ministry of Health, Labour, and Welfare, grants from Japan Agency for Medical Desearch and Development, advisory board participation and lecture fees from Boehringer Ingelheim, Inc., lecture fees from Shionogi & Co Ltd, Kyorin Pharmaceutical Co Ltd, GSK and Novartis, and advisory board participation for Roche/Promedior, Galapagos, Taiho pharma, CSL Behring and Vicore Pharma, outside the submitted work. V.M. McDonald reports personal fees from GlaxoSmithKline, AstraZeneca and Arterial Group, grants from Ramaciotti Foundation Grant, MRFF, JHH Charitable Trust and NHMRC, outside the submitted work. J. Oldham reports grants from National Institutes of Health and Boehringer Ingelheim, during the conduct of the study; personal fees from Boehringer Ingelheim, Roche/Genentech, Lupin Pharmaceuticals and Gatehouse Bio, and data monitoring committee participation for Novartis, Endeavor BioMedicines and Genenetch, outside the submitted work. E.A. Renzoni reports a research grant from Boehringer Ingelheim (paid to institution), payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from Boeringher Ingelheim, Roche and Chiesi, support for attending meetings and/or travel from Boehringer Ingelheim, and advisory board participation for Boeringher Ingelheim and Roche, outside the submitted work. A.M. Russell reports lecture fees from Boehringer Ingelheim, Hoffman La Roche, Irish Lung Fibrosis Association, Respiratory Professional Care and the Interstitial Lung Disease Interdisciplinary Network, educational resource development for Boehringer Ingelheim, and consultancy for Hoffman La Roche, Irish Lung Fibrosis Association, Respiratory Professional Care and the Interstitial Lung Disease Interdisciplinary Network, outside the submitted work. M.E. Strek reports grants or contracts from Boehringer Ingelheim and Pulmonary Fibrosis Foundation, payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from Boehringer Ingelheim and American College of Chest Physicians, and participation on Fibrogen adjudication committee, outside the submitted work; and is part of the Chair Scientific Review Committee of the Pulmonary Fibrosis Foundation. C.J. Ryerson reports grants and personal fees from Boehringer Ingelheim and Hoffmann-La Roche, personal fees from Veracyte, Pliant Therapeutics, AstraZeneca, Trevi Therapeutics and Cipla Ltd, and grants from VIDA diagnostics, outside the submitted work., (Copyright ©The authors 2023. For reproduction rights and permissions contact permissions@ersnet.org.)

Purpose of Review: Telerehabilitation is an alternative delivery model for pulmonary rehabilitation, an evidence-based nonpharmacological intervention, in people with chronic pulmonary disease. This review synthesizes current evidence regarding the telerehabilitation model for pulmonary rehabilitation with an emphasis on its potential and implementation challenges, as well as the clinical experiences from the COVID-19 pandemic., Recent Findings: Different models of telerehabilitation for delivering pulmonary rehabilitation exist. Current studies comparing telerehabilitation to centre-based pulmonary rehabilitation primarily focus on the evaluation in people with stable chronic obstructive pulmonary disease, which demonstrated equivalent improvements in exercise capacity, health-related quality of life and symptoms with improved programme completion rates. Although telerehabilitation may improve access to pulmonary rehabilitation by addressing travel burden, improving schedule flexibility and geographic disparity, there are challenges of ensuring satisfaction of healthcare interactions and delivering core components of initial patient assessment and exercise prescription remotely., Summary: Further evidence is needed on the role of telerehabilitation in various chronic pulmonary diseases, as well as the effectiveness of different modalities in delivering telerehabilitation programmes. Economic and implementation evaluation of currently available and emerging models of telerehabilitation in delivering pulmonary rehabilitation are needed to ensure sustainable adoption into clinical management for people with chronic pulmonary disease., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Rationale: Chronic cough is a common symptom in patients with interstitial lung disease (ILD), negatively contributing to health-related quality of life. Despite this, there is limited information and understanding on the experience of this group of patients with chronic cough. This study aimed to explore the symptom experiences for chronic cough in patients with ILD to identify its characteristics and impacts., Methods: A qualitative study using semi-structured telephone interviews was undertaken in 16 adults with a diagnosis of ILD of any type and severity. Patients were recruited from a quaternary referral centre in Melbourne, Australia. Interviews were transcribed verbatim and coded by two researchers using thematic analysis., Results: Patients (age range: 39-87 years, forced vital capacity: 53-107% predicted and diffusing capacity of the lung for carbon monoxide: 28-89% predicted) experienced a spectrum of cough severity and characteristics, including both dry and productive coughs. The impact of chronic cough included physical symptoms, social and emotional difficulties, and interference with work and vocational participation. Management strategies used to relieve cough included mucolytics, opiates, throat lozenges, warm drinks, pacing, breath control, relaxation exercises, movement, continuous positive airways pressure and supplemental oxygen. Patients expressed a need for further information and education regarding chronic cough, including its triggers and management., Conclusions: This study highlights the experience and significance of chronic cough in patients with ILD. The nature and severity of chronic cough in patients with ILD appears to be more heterogeneous than previously described, with physical, social and emotional impacts contributing to symptom burden., Competing Interests: Conflict of interest: N.S.L. Goh reports personal fees from Boehringer Ingelheim and AstraZeneca, and nonfinancial support from Air Liquide outside the submitted work. The other authors report no relevant disclosures., (Copyright ©The authors 2023.)

Background: Little is known about generalisability of randomised controlled trials (RCTs) for idiopathic pulmonary fibrosis (IPF). We evaluated eligibility criteria for phase III IPF RCTs to determine their representativeness in clinical registries, and calculated forced vital capacity (FVC) changes according to eligibility criteria., Methods: Common eligibility criteria used in >60% of IPF RCTs were identified from a literature search and applied to patients with IPF from prospective Australian and Canadian registries. Additional pre-specified criteria of 6-min walk distance (6MWD) and different measures of preceding disease progression were also evaluated. Joint longitudinal-survival modelling was used to compare FVC decline according to eligibility for individual and composite criteria., Results: Out of 990 patients with IPF, 527 (53%) met all common RCT eligibility criteria at the first clinic visit, including 343 with definite IPF and 184 with radiological probable usual interstitial pneumonia pattern without histological confirmation ( i.e. provisional IPF). The percentages of eligible patients for landmark RCTs of nintedanib and pirfenidone were 19-50%. Adding 6MWD ≥150 m and different measures of preceding disease progression to the composite common criteria reduced the percentages of patients meeting eligibility to 52% (n=516) and 4-18% (n=12-61), respectively. Patients meeting the composite common criteria had less-rapid 1-year FVC decline than those who did not (-90 versus -103 mL, p=0.01). Definite IPF generally had more-rapid 1-year FVC decline compared to provisional IPF., Conclusions: Eligibility criteria of previous IPF RCTs have limited generalisability to clinical IPF populations, with FVC decline differing between eligible and ineligible populations., Competing Interests: Conflict of interest: Y.H. Khor reports fellowship support from NHMRC Investigator Grant, and support for the CARE-PF registry (with no influence on this study) from Boehringer Ingelheim, during the conduct of the study. K.A. Johannson reports personal fees, non-financial support and other from Boehringer Ingelheim, personal fees from Hoffman-La Roche Ltd, Pliant Therapeutics and Thyron, grants from University Hospital Foundation, Pulmonary Fibrosis Society of Calgary and University of Calgary Cumming School of Medicine, and personal fees and non-financial support from Three Lakes Foundation, outside the submitted work. V. Marcoux reports grants from Boehringer Ingelheim, during the conduct of the study; grants from University of British Columbia and Boehringer Ingelheim, outside the submitted work. J.H. Fisher reports grants from Boehringer Ingelheim, during the conduct of the study; grants from Canadian Pulmonary Fibrosis Foundation and University of Toronto, and personal fees from Boehringer Ingelheim and AstraZeneca, outside the submitted work. D. Assayag reports grants from Boehringer Ingelheim Canada, and lecture honoraria from Boehringer Ingelheim and Hoffman La Roche, outside the submitted work. H. Manganas reports support for the present manuscript from University of British Columbia; grants from Boehringer Ingelheim Canada, Hoffmann La Roche, Galapagos and BMS, honoraria for educational events from Boehringer Ingelheim Canada, and advisory board membership for Boehringer Ingelheim Canada, outside the submitted work. M. Kolb reports grants from Boehringer Ingelheim, Pieris and Roche, consulting fees from Boehringer Ingelheim, Roche, Horizon, Cipla, Abbvie, Bellerophon, Algernon, CSL Behring, United Therapeutics and LabCorp, lecture honoraria from Roche, Novartis and Boehringer Ingelheim, payment for expert testimony from Roche, advisory board participation with United Therapeutics and LabCorp, and reports an editorial allowance from ERJ, outside the submitted work. C.J. Ryerson reports grants from Boehringer Ingelheim, during the conduct of the study; grants and personal fees from Boehringer Ingelheim and Hoffmann-La Roche, and personal fees from Veracyte, Pliant Therapeutics, AstraZeneca and Cipla Ltd, outside the submitted work. All other authors have nothing to disclose., (Copyright ©The authors 2023. For reproduction rights and permissions contact permissions@ersnet.org.)

Introduction: The COVID-19 pandemic resulted in a rapid transformation of health services. This study aimed to understand the experiences of healthcare by people with interstitial lung disease (ILD), to inform future service delivery., Methods: Four specialist clinics in tertiary centres in Australia (Victoria:2 sites; New South Wales: 1 site; Western Australia: 1 site) recruited patients with ILD during an 8-week period from March 2021. Participants completed a COVID-specific questionnaire focused on health-related experiences during 2020., Results: Ninety nine (65% of 153) participants completed the questionnaire. 47% had idiopathic pulmonary fibrosis or connective tissue disease-associated ILD, 62% were female and the average age was 66 years. Whilst 56% rated their overall health in 2020 as the same as months prior, 38% indicated a worsening in health attributed to reduced physical activity and fear of contracting the virus. Access to healthcare professionals was 'good' in 61%, and 'fair-to-poor' for 37% due to missed respiratory assessments, with telehealth (mainly telephone) being perceived as less effective. 89% had contact with respiratory physicians, 68% with general practitioners, predominantly via telephone, with few video consultations. High satisfaction with care was reported by 78%, with lower satisfaction attributed to delays in assessments, disruption to usual services such as pulmonary rehabilitation, and dissatisfaction with telehealth., Conclusion: People with ILD were generally satisfied with their care during 2020, however reduced access to healthcare professionals was challenging for those experiencing a deterioration in health. Telehealth was largely well received but did not always meet the needs of people with ILD particularly when unwell., (© 2023. The Author(s).)

Obstructive sleep apnea (OSA) is a significant healthcare burden affecting approximately one billion people worldwide. The prevalence of OSA is rising with the ongoing obesity epidemic, a key risk factor for its development. While in-laboratory polysomnography (PSG) is the gold standard for diagnosing OSA, it has significant drawbacks that prevent widespread use. Portable devices with different levels of monitoring are available to allow remote assessment for OSA. To better inform clinical practice and research, this comprehensive systematic review evaluated diagnostic performances, study cost and patients' experience of different levels of portable sleep studies (type 2, 3, and 4), as well as wearable devices and non-contact systems, in adults. Despite varying study designs and devices used, portable diagnostic tests are found to be sufficient for initial screening of patients at risk of OSA. Future studies are needed to evaluate cost effectiveness with the incorporation of portable diagnostic tests into the diagnostic pathway for OSA, as well as their application in patients with chronic respiratory diseases and other comorbidities that may affect test performance., Competing Interests: Declaration of competing interest None., (Copyright © 2023 Elsevier Ltd. All rights reserved.)

Background: Functional capacity, as measured by the 6-min walk test (6MWT), is often reduced in fibrotic interstitial lung disease (ILD). This study evaluated longitudinal changes and the prognostic significance of 6MWT parameters, and explored change in oxygenation status as a physiological criterion to define disease progression in patients with fibrotic ILD., Research Questions: What are the trajectories and prognostic value of 6MWT parameters in patients with fibrotic ILD?, Study Design and Methods: Using prospective registries in Australia and Canada, patients with idiopathic pulmonary fibrosis (IPF) and non-IPF fibrotic ILD were stratified by the presence of criteria for progressive pulmonary fibrosis (PPF). The cumulative incidence of exertional and resting hypoxemia and changes in 6-min walk distance (6MWD) and composite indices (distance-saturation product and distance-saturation-oxygen product) were determined, with prognostic significance evaluated at the time of meeting criteria for PPF. New-onset exertional or resting hypoxemia was evaluated as another potential criterion for PPF., Results: Patients with IPF/PPF (n = 126) and non-IPF/PPF (n = 227) had a similar cumulative incidence of exertional hypoxemia and annualized decline in 6MWD and composite indices, which varied across each PPF criterion. Patients with IPF/non-PPF (n = 231) and non-IPF/non-PPF (n = 531) had a significantly lower incidence of hypoxemia than those with IPF/PPF, with an annualized increase in 6MWD and composite indices in the non-IPF/non-PPF group. Exertional or resting hypoxemia at the time of meeting criteria for PPF was independently associated with reduced transplant-free survival in IPF and non-IPF, adjusting for patient demographics and lung function. Adding new-onset exertional or resting hypoxemia as a physiological criterion reduced the median time to development of PPF from 11.2 to 6.7 months in IPF and from 11.7 to 5.6 months in non-IPF in patients who eventually met both definitions (P < .001 for both)., Interpretation: Patients with IPF/PPF and non-IPF/PPF have comparable deterioration in functional capacity. Oxygenation status provides prognostic information in PPF and may assist in defining disease progression in fibrotic ILD., (Copyright © 2022 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.)

The widespread use of smartphones and the internet has enabled self-monitoring and more hybrid-care models. The COVID-19 pandemic has further accelerated remote monitoring, including in the heterogenous and often vulnerable group of patients with interstitial lung diseases (ILDs). Home monitoring in ILD has the potential to improve access to specialist care, reduce the burden on health-care systems, improve quality of life for patients, identify acute and chronic disease worsening, guide treatment decisions, and simplify clinical trials. Home spirometry has been used in ILD for several years and studies with other devices (such as pulse oximeters, activity trackers, and cough monitors) have emerged. At the same time, challenges have surfaced, including technical, analytical, and implementational issues. In this Series paper, we provide an overview of experiences with home monitoring in ILD, address the challenges and limitations for both care and research, and provide future perspectives. VIDEO ABSTRACT., Competing Interests: Declaration of interests MSW reports grants from Boehringer Ingelheim, Hoffman la Roche, The Netherlands Organisation for Health Research and Development, The Dutch Lung Foundation, and The Dutch Pulmonary Fibrosis Foundation; consulting fees from Boehringer Ingelheim, Hoffman la Roche, Galapagos, Bristol Myers Squibb (BMS), Galecto, Respivant, Nerretherapeutics, PureTech Health, Kinevant Sciences, Molecure, Horizontherapeutics, and CSL Behring; speaker fees from Boehringer Ingelheim, Hoffman la Roche, and Novartis; support for attending meetings from Boehringer Ingelheim, Hoffman la Roche, and Galapagos; and DSMB fees from Savara and Galapagos. All grant and fees were paid to her institution. CCM reports grants from Boehringer Ingelheim and AstraZeneca; and speakers fees from Boehringer Ingelheim and Hoffman-la Roche. All grants and fees were paid to her institution. KAJ reports grants from Three Lakes Foundation, Chest Foundation, University of Calgary, and University Hospital Foundation; consulting fees from Boehringer Ingelheim, Hoffman-La Roche, Pliant, and Three Lakes Foundation; and speaker fees from Boehringer Ingelheim and Hoffman-La Roche. PDJ reports grants from the Pulmonary Foundation Scholar Award, Chest/ATS Foundation COVID19 diversity grant, VCU DOIM Pilot award, and NIH Fogarty Fellowship; and speaker fees from the Virginia Association of Community Psychiatric Nurses. YHK reports grants from the National Health and Medical Research Council Investigator Grant, Centre of Research Excellence in Pulmonary Fibrosis PACT Grant-in-Aid and CREATE Grant-in-Aid, Austin Medical Research Foundation Research Grant, Royal Australasian College of Physicians Research Establishment Fellowship and Robert and Elizabeth Albert Travel Grant, and Lung Foundation Australia-Lizotte Family Award for Idiopathic Pulmonary Fibrosis Research. YK reports speaker fees from Asahi Kasei Pharma, Shionogi, Boehringer Ingelheim, AstraZeneca, Eisai, KYORIN Pharmaceutical, Mitsubishi Tanabe Pharma, and Novartis Pharma; and advisory board fees from Shionogi, Boehringer Ingelheim, Taiho Pharmaceutical, Chugai Pharmaceutical, Janssen Pharmaceutical, and Healios. GCT reports consulting fees from Boehringer Ingelheim and Knight pharmaceuticals; and speaker fees from Boehringer Ingelheim, Tutear, Raffo, AstraZeneca and Knight pharmaceuticals. BEV reports travel support from Boehringer Ingelheim, Raffo, Roche, Bago, and Tutear; and advisory board fees from Boehringer Ingelheim, and Tutear. RNvZ-S reports consulting fees from Aspen/GlaxoSmithKline (GSK), Novartis, and Boehringer Ingelheim; speaker fees from Novartis, Pfizer, Johnson & Johnson, MSD, AstraZeneca, Hoffman la Roche, Philips, and Cipla. MK reports grants from Boehringer Ingelheim and Hoffman-La Roche; consulting fees from Boehringer Ingelheim, Hoffman-La Roche, and Galapagos; and speaker fees from Boehringer Ingelheim and Hoffman-La Roche. TMM reports consulting fees from Boehringer Ingelheim, Roche/Genentech, AstraZeneca, Bayer, Blade Therapeutics, BMS, Galapagos, Galecto, GSK, IQVIA, Pliant, Respivant, Theravance, and Veracyte; and speaker fees from Boehringer Ingelheim and Roche/Genentech. SKR and PvdW declare no competing interests., (Copyright © 2023 Elsevier Ltd. All rights reserved.)

Rationale: Self-management is considered as an important part of disease management for people with pulmonary fibrosis (PF), but there is a lack of consensus regarding what components should be included. This study aimed to attain consensus from experts in PF and people living with the disease on the essential components and format of a PF self-management package., Methods: A two-round Delphi process was conducted. In each round, a panel of experts completed an online survey to rate a range of components, formats and delivery methods, followed by an online patient focus group to integrate patient perspectives. Consensus was defined a priori ., Results: 45 experts participated in Round 1 and 51 in Round 2. Both focus groups included six people with PF. 12 components were considered essential for self-management in PF: 1) understanding treatment options; 2) understanding and accessing clinical trials; 3) managing medications; 4) role of oxygen therapy; 5) role and importance of pulmonary rehabilitation and regular physical activity; 6) managing shortness of breath; 7) managing fatigue; 8) managing mood; 9) managing comorbidities; 10) smoking cessation advice and support; 11) accessing community support; and 12) how to communicate with others when living with PF. Both groups agreed that self-management in PF required individualisation, goal setting and feedback., Conclusion: This study identified 12 essential components and highlighted individualisation, goal setting and feedback in self-management of PF. The findings provide a basis for the development of PF self-management interventions., Competing Interests: Conflict of interest: J.Y.T. Lee has no relevant conflict of interest to disclose. Conflict of interest: G. Tikellis has no relevant conflict of interest to disclose. Conflict of interest: Y.H. Khor reports fellowship support from an NHMRC Investigator Grant and grants from Boehringer Ingelheim, during the conduct of the study. Conflict of interest: A.E. Holland has no relevant conflict of interest to disclose., (Copyright ©The authors 2022.)

Recent clinical practice guidelines have addressed the diagnosis of idiopathic pulmonary fibrosis (IPF) and fibrotic hypersensitivity pneumonitis (fHP). These disease-specific guidelines were developed independently, without clear direction on how to apply their respective recommendations concurrently within a single patient, where discrimination between these two fibrotic interstitial lung diseases represents a frequent diagnostic challenge. The objective of this review, created by an international group of experts, was to suggest a pragmatic approach on how to apply existing guidelines to distinguish IPF and fHP. Key clinical, radiologic, and pathologic features described in previous guidelines are integrated in a set of diagnostic algorithms, which then are placed in the broader context of multidisciplinary discussion to guide the generation of a consensus diagnosis. Although these algorithms necessarily reflect some uncertainty wherever strong evidence is lacking, they provide insight into the current approach favored by experts in the field based on currently available knowledge. The authors further identify priorities for future research to clarify ongoing uncertainties in the diagnosis of fibrotic interstitial lung diseases., (Copyright © 2022 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.)

Rationale: In 2018, a systematic review evaluating transbronchial lung cryobiopsy (TBLC) in patients with interstitial lung disease (ILD) was performed to inform American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Asociación Latinoamericana del Tórax clinical practice guidelines on the diagnosis of idiopathic pulmonary fibrosis. Objectives: To perform a new systematic review to inform updated guidelines. Methods: Medline, Excerpta Medica Database, and the Cochrane Central Register of Controlled Trials (CCTR) were searched through June 2020. Studies that enrolled patients with ILD and reported the diagnostic yield or complication rates of TBLC were selected for inclusion. Data was extracted and then pooled across studies via meta-analysis. The quality of the evidence was appraised using the grading of recommendations, assessment, development, and evaluation approach. Results: Histopathologic diagnostic yield (number of procedures that yielded a histopathologic diagnosis divided by the total number of procedures performed) of TBLC was 80% (95% confidence interval [CI], 76-83%) in patients with ILD. TBLC was complicated by bleeding and pneumothorax in 30% (95% CI, 20-41%) and 8% (95% CI, 6-11%) of patients, respectively. Procedure-related mortality, severe bleeding, prolonged air leak, acute exacerbation, respiratory failure, and respiratory infection were rare. The quality of the evidence was very low owing to the uncontrolled study designs, lack of consecutive enrollment, and inconsistent results. Conclusions: Very low-quality evidence indicated that TBLC has a diagnostic yield of approximately 80% in patients with ILD, with manageable complications.

Background: Pulse oximeters are often used at home by patients with chronic respiratory diseases and more recently for remote monitoring of patients with COVID-19. There are no published data outside a supervised telemedicine setting regarding patients' experiences with these devices. Our objective was to explore patients' usage patterns and perceptions of using pulse oximetry at home., Methods: Patients with chronic respiratory disease who had a pulse oximeter at home were recruited to complete a structured survey., Results: Thirty participants with a range of chronic respiratory diseases (mean age 71 y, 16 females) were recruited. Most participants (83%) used home oxygen therapy. Pulse oximeters were bought online (46.7%), at a pharmacy (40%), at a medical equipment store (6.7%), through a clinic (3.3%), or from an oxygen supplier (3.3%). Use was self-initiated in 56.7% of cases and was based on a health care-related recommendation in 26.7% of cases. Sixty percent of participants used the oximeter daily, with 90% expressing confidence in interpreting their oximeter readings primarily due to education from health care professionals and in-patient experiences. Almost all participants adjusted their activity levels or management based upon oximeter readings. Most participants reported that using a pulse oximeter at home was helpful in judging their physical limitations and provided reassurance and confidence in their disease management., Conclusions: Subjects appeared confident in their use of home pulse oximetry. Health professionals should identify patients who use pulse oximeters for monitoring and ensure that they are able to interpret readings correctly and, if appropriate, adjust management safely., Competing Interests: Dr Khor discloses relationships with Air Liquide Healthcare, Boehringer Ingelheim, and Roche. Dr McDonald discloses a relationship with Air Liquide Healthcare. The remaining authors have disclosed no conflicts of interest., (Copyright © 2022 by Daedalus Enterprises.)

Rationale: Multimorbidity is common and leads to substantial concomitant medication burden in patients with interstitial lung disease (ILD), which may affect tolerability of ILD-targeted medications and health outcomes. Objectives: To determine the associations of concomitant medication burden with tolerability of ILD-targeted medications and survival in patients with idiopathic pulmonary fibrosis (IPF) and non-IPF ILD. Methods: Patients with IPF receiving nintedanib or pirfenidone and patients with non-IPF ILD receiving azathioprine or mycophenolate were identified from two Australian and Canadian registries. Baseline concomitant medication burden was evaluated using three measures: medication count, polypharmacy (⩾5 medications), and the medication regimen complexity index (MRCI). Medication intolerance and discontinuation were evaluated at 6 months and 1 year after initiation of ILD-targeted medications, respectively. Cox regression models and likelihood ratio tests were used to determine the prognostic significance of medication burden on transplant-free survival. Results: In 645 treated patients with IPF, 43% experienced adverse reactions leading to intolerance (defined as dose reduction, temporary dose interruption, or permanent drug discontinuation) of antifibrotic medications within 6 months of initiation, with high baseline concomitant medication burden being consistently associated with intolerance (medication count: P  = 0.005; polypharmacy: P  = 0.006; MRCI: P  = 0.004). This association was not observed for immunosuppressive medications in 1,255 treated patients with non-IPF ILD, who also had a lower intolerance (18%). Baseline concomitant medication burden was not independently associated with permanent discontinuation of antifibrotic (29%) and immunosuppressive medications (20%) at 1 year. The MRCI was the only measure of concomitant medication burden associated with transplant-free survival in both cohorts ( P  < 0.01 for both), which improved prognostication beyond common clinical factors and the ILD-GAP index ( P  < 0.001 for both). Conclusions: Concomitant medication burden is associated with intolerance of antifibrotic medications in patients with IPF. Medication regimen complexity is superior to simpler evaluation of concomitant medication burden for predicting prognosis in ILD.

Background: The American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Asociación Latinoamericana del Tórax convened to update clinical practice guidelines for interstitial lung disease (ILD). Objective: To conduct a systematic review to evaluate existing ILD literature to determine whether patients with progressive pulmonary fibrosis (PPF) should be treated with the antifibrotic pirfenidone. Data Sources: A literature search was conducted across MEDLINE, EMBASE, and Cochrane databases through December 2020 for studies using pirfenidone to treat patients with PPF. Data Extraction: Mortality, disease progression, lung function, and adverse event data were extracted. Meta-analyses were performed when possible. The Grading of Recommendations, Assessment, Development and Evaluation Working Group approach was used to assess the quality of evidence. Synthesis: Two studies met inclusion criteria. Meta-analyses revealed that changes in forced vital capacity (FVC) percent predicted (mean difference [MD], 2.3%; 95% confidence interval [CI], 0.5-4.1%), the FVC in milliliters (MD, 100.0 ml; 95% CI, 98.1-101.9 ml), and the 6-minute-walk distance in meters (MD, 25.2 m; 95% CI, 8.3-42.1 m) all favored pirfenidone over placebo. The changes in the diffusing capacity of the lung for carbon monoxide (DLCO) in millimoles per kilopascal per minute (MD, 0.40 mmol/kPa/min; 95%, CI 0.10-0.70 mmol/kPa/min) and risk of DLCO declining more than 15% (relative risk [RR], 0.27; 95% CI, 0.08-0.95) also favored pirfenidone. The risks of gastrointestinal discomfort (RR, 1.83; 95% CI, 1.29-2.60) and photosensitivity (RR, 4.88; 95% CI, 1.09-21.83) were higher with pirfenidone. The quality of the evidence was low or very low according to the Grading of Recommendations, Assessment, Development and Evaluation criteria, depending on the outcome. Conclusions: Pirfenidone use in patients with PPF is associated with a statistically significant decrease in disease progression and with protection of lung function. However, there is very low certainty in the estimated effects because of limitations in the available evidence. Primary Source of Funding: Funded by the American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Asociación Latinoamericana del Tórax.

Background: Home oxygen therapy is prescribed for patients with advanced lung disease based on the criteria established in landmark trials in subjects with COPD. In clinical practice, its use has been extrapolated to other diseases, including interstitial lung disease (ILD). Patients with COPD and ILD experience a high symptom burden and require access to specialized multidisciplinary care. We aimed to evaluate the health-related outcomes and supportive care needs of patients with COPD and ILD receiving home oxygen therapy., Methods: This was a retrospective cohort study using the oxygen database of a quaternary metropolitan teaching hospital. Patients with a diagnosis of COPD or ILD who were prescribed home oxygen therapy between January 2012-December 2018 were identified. Demographic information, results of physiologic testing, comorbidities, hospitalizations, and mortality data were collected., Results: Three hundred and eighty-four subjects were included for analysis, of whom 56% were male. The median age was 75 y. The majority (59%) had a diagnosis of COPD. Long-term oxygen therapy (LTOT) was prescribed for 187 (48.7%), with no significant demographic differences between those with COPD or ILD. Another 187 were prescribed ambulatory oxygen alone, with 55 transitioning to LTOT during the study period. Most subjects (65.4%) were referred for pulmonary rehabilitation; however, palliative care referrals were generally low (22.9%). Referrals to other medical specialties and allied health were common (82%). Transplant-free survival after commencement of LTOT was poor, with 38% of subjects surviving at 5 y. The 5-y survival of subjects with ILD after commencing on LTOT was 10% compared to 52% for those with COPD. Multivariable Cox regression analyses showed that the only predictor of survival after commencing LTOT was the principal respiratory diagnosis., Conclusions: This study found that subjects prescribed LTOT had poor transplant-free survival after initiation, which was significantly worse for those with ILD compared to those with COPD. Despite their poor overall survival, worse than many cancers, only a minority were referred for palliative care input. Referrals to pulmonary rehabilitation were also suboptimal. This patient population had complex care needs requiring multidisciplinary management. Appropriate and early referrals to palliative care and improved care coordination for this complex group of patients are key areas for improvement in clinical practice., Competing Interests: Dr Khor discloses relationships with Air Liquide Healthcare, Boehringer Ingelheim, and Roche. Dr McDonald discloses relationships with Air Liquide Healthcare and Menarini. The remaining authors have disclosed no conflicts of interest., (Copyright © 2022 by Daedalus Enterprises.)

Background and Objective: Inhalation of high concentrations of respirable crystalline silica (RCS) can lead to silicosis. RCS contains varying levels of iron, which can cause oxidative stress and stimulate ferritin production. This study evaluated iron-related and inflammatory markers in control and silicosis patients., Methods: A cohort of stone benchtop industry workers (n = 18) were radiologically classified by disease severity into simple or complicated silicosis. Peripheral blood and bronchoalveolar lavage (BAL) were collected to measure iron, ferritin, C-reactive protein, serum amyloid A and serum silicon levels. Ferritin subunit expression in BAL and transbronchial biopsies was analysed by reverse transcription quantitative PCR. Lipid accumulation in BAL macrophages was assessed by Oil Red O staining., Results: Serum iron levels were significantly elevated in patients with silicosis, with a strong positive association with serum ferritin levels. In contrast, markers of systemic inflammation were not increased in silicosis patients. Serum silicon levels were significantly elevated in complicated disease. BAL macrophages from silicosis patients were morphologically consistent with lipid-laden foamy macrophages. Ferritin light chain (FTL) mRNA expression in BAL macrophages was also significantly elevated in simple silicosis patients and correlated with systemic ferritin., Conclusion: Our findings suggest that elevated iron levels during the early phases of silicosis increase FTL expression in BAL macrophages, which drives elevated BAL and serum ferritin levels. Excess iron and ferritin were also associated with the emergence of a foamy BAL macrophage phenotype. Ferritin may represent an early disease marker for silicosis, where increased levels are independent of inflammation and may contribute to fibrotic lung remodelling., (© 2022 The Authors. Respirology published by John Wiley & Sons Australia, Ltd on behalf of Asian Pacific Society of Respirology.)

Background: The American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Asociación Latinoamericana del Tórax convened to update clinical practice guidelines for interstitial lung disease (ILD). Objective: To conduct a systematic review to evaluate existing ILD literature to determine whether patients with progressive pulmonary fibrosis (PPF) should be treated with the antifibrotic nintedanib. Data Sources: A literature search was conducted across MEDLINE, EMBASE, and Cochrane databases through December 2020 for studies using nintedanib to treat patients with PPF. Data Extraction: Mortality, disease progression, and adverse event data were extracted, and meta-analyses performed when possible. The Grading of Recommendations, Assessment, Development and Evaluation (GRADE) Working Group approach was used to assess the quality of evidence. Synthesis: Two relevant studies were selected. The annual decline in forced vital capacity was less in the nintedanib arm in the overall study population (mean difference [MD], 107 ml/yr; 95% confidence interval [CI], 65.4 to 148.5 ml/yr) and in the subgroups with usual interstitial pneumonia (UIP) pattern of pulmonary fibrosis (MD, 128.2 ml/yr; 95% CI, 70.8 to 185.6 ml/yr), non-UIP patterns of pulmonary fibrosis (MD, 75.3 ml/yr; 95% CI, 15.5 to 135.0 ml/yr), fibrotic connective tissue disease-related ILD (MD, 106.2 ml/yr; 95% CI, 10.6 to 201.9 ml/yr), fibrotic idiopathic nonspecific interstitial pneumonia (MD, 141.7 ml/yr; 95% CI, 46.0 to 237.4 ml/yr), and fibrotic occupational ILD (MD, 252.8 ml/yr; 95% CI, 79.2 to 426.5 ml/yr), but not fibrotic hypersensitivity pneumonitis (MD, 72.9 ml/yr; 95% CI, -8.9 to 154.7 ml/yr), fibrotic sarcoidosis (MD, -20.5 ml/yr; 95% CI, -337.1 to 296.1 ml/yr), or unclassified fibrotic ILD (MD, 68.5 ml/yr; 95% CI, -31.3 to 168.4 ml/yr) when compared with placebo. Gastrointestinal side effects were common. Quality of evidence for the outcomes ranged from very low to moderate GRADE. Conclusions: Nintedanib use in patients with PPF is associated with a statistically significant decrease in disease progression but increase in gastrointestinal side effects regardless of the radiographic pattern of pulmonary fibrosis. However, limitations in the available evidence lead to low certainty in these effect estimates and make definitive conclusions about the differential effects by subtype of ILD difficult to determine. Primary Source of Funding: Funded by the American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Asociación Latinoamericana del Tórax.