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Prediction of disease course in patients with progressive pulmonary fibrosis remains challenging. The purpose of this study was to assess the prognostic value of lung fibrosis extent quantified at computed tomography (CT) using data-driven texture analysis (DTA) in a large cohort of well-characterized patients with idiopathic pulmonary fibrosis (IPF) enrolled in a national registry.This retrospective analysis included participants in the Australian IPF Registry with available CT between 2007 and 2016. CT scans were analysed using the DTA method to quantify the extent of lung fibrosis. Demographics, longitudinal pulmonary function and quantitative CT metrics were compared using descriptive statistics. Linear mixed models, and Cox analyses adjusted for age, gender, BMI, smoking history and treatment with anti-fibrotics were performed to assess the relationships between baseline DTA, pulmonary function metrics and outcomes.CT scans of 393 participants were analysed, 221 of which had available pulmonary function testing obtained within 90 days of CT. Linear mixed-effect modelling showed that baseline DTA score was significantly associated with annual rate of decline in forced vital capacity and diffusing capacity of carbon monoxide. In multivariable Cox proportional hazard models, greater extent of lung fibrosis was associated with poorer transplant-free survival (hazard ratio [HR] 1.20, p 0.0001) and progression-free survival (HR 1.14, p 0.0001).In a multi-centre observational registry of patients with IPF, the extent of fibrotic abnormality on baseline CT quantified using DTA is associated with outcomes independent of pulmonary function.

Supported by the National Health and Medical Research Council Centre of Research Excellence in Pulmonary Fibrosis (GNT1116371), and by foundation partner, Boehringer Ingelheim, and program partners, Roche and Galapagos. Rationale : The interstitial lung disease (ILD) multidisciplinary meetings (MDM), composed of pulmonologists, radiologists, and pathologists, is integral to the rendering of an accurate ILD diagnosis. However, there is significant heterogeneity in the conduct of ILD MDMs, and questions regarding their best practices remain unanswered. Objectives : To achieve consensus among ILD experts on essential components of an ILD MDM. Methods : Using a Delphi methodology, semi-structured interviews with ILD experts were used to identify key themes and features of ILD MDMs. These items informed two subsequent rounds of online questionnaires that were used to achieve consensus among a broader, international panel of ILD experts. Experts were asked to rate their level of agreement on a five-point Likert scale. An a priori threshold for consensus was set at a median score 4 or 5 with an interquartile range of 0. Results : We interviewed 15 ILD experts, and 102 ILD experts participated in the online questionnaires. Five items and two exploratory statements achieved consensus on being essential for an ILD MDM following two questionnaire rounds. There was consensus that the presence of at least one radiologist, a quiet setting with a visual projection system, a high-quality chest high-resolution computed tomography, and a standardized template summarizing collated patient data are essential components of an ILD MDM. Experts also agreed that it would be useful for ILD MDMs to undergo an annual benchmarking process and a validation process by fulfilling a minimum number of cases annually. Twenty-seven additional features were considered to be either highly desirable or desirable features based on the degree of consensus. Although our findings on desirable features are similar to the current literature, several of these remain controversial and warrant further research. The study also showed an agreement among participants on several future concepts to improve the ILD MDM, such as performing regular self-assessments and conducting research into shared practices to develop an international expert guideline statement on ILD MDMs. Conclusions : This Delphi study showed consensus among international ILD experts on essential and desirable features of an ILD MDM. Our data represent an important step toward potential collaborative research into future standardization of ILD MDMs. Postprint

Purpose Idiopathic pulmonary fibrosis (IPF) is a type of interstitial lung disease found mostly in elderly persons, characterized by a high symptom burden and frequent encounters with health services. This study aimed to quantify the economic burden of IPF in Australia with a focus on resource utilization and associated direct costs. Methods Participants were recruited from the Australian IPF Registry (AIPFR) between August 2018 and December 2019. Data on resource utilization and costs were collected via cost diaries and linked administrative data. Clinical data were collected from the AIPFR. A “bottom up” costing methodology was utilized, and the costing was performed from a partial societal perspective focusing primarily on direct medical and non-medical costs. Costs were standardized to 2021 Australian dollars ($). Results The average annual total direct costs per person with IPF was $31,655 (95% confidence interval (95% CI): $27,723–$35,757). Extrapolating costs based on prevalence estimates, the total annual costs in Australia are projected to be $299 million (95% CI: $262 million–$338 million). Costs were mainly driven by antifibrotic medication, hospital admissions and medications for comorbidities. Disease severity, comorbidities and antifibrotic medication all had varying impacts on resource utilization and costs. Conclusion This cost-of-illness study provides the first comprehensive assessment of IPF-related direct costs in Australia, identifies the key cost drivers and provides a framework for future health economic analyses. Additionally, it provided insight into the major cost drivers which include antifibrotic medication, hospital admissions and medications related to comorbidities. Our findings emphasize the importance of the appropriate management of comorbidities in the care of people with IPF as this was one of the main reasons for hospitalizations.

Nasal high flow therapy (NHF) is increasingly used in acute care settings. In this review, we consider recent advances in the utilization of NHF in chronic obstructive pulmonary disease (COPD), terminal cancer and symptom management. Considerations around NHF use during the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic are also discussed.NHF enables humidification and high flows to be provided together with titrated, supplemental oxygen therapy. Compared to conventional oxygen therapy, NHF improves respiratory physiology by reducing workload, enhancing muco-ciliary clearance and improving dead space washout. Some studies suggest that early use of NHF in people being cared for in the emergency department leads to lower rates of invasive ventilation and noninvasive ventilation. There is also emerging evidence for NHF use in people with COPD and chronic respiratory failure, and in palliative care. NHF is comfortable, well-tolerated and safe for use in the management of breathlessness in people with cancer. NHF can be delivered by face mask to patients with SARS-CoV-2 infection, to ease the burden on critical care resources.The evidence base for NHF is rapidly growing and offers promise in relieving troublesome symptoms and for people receiving palliative care.

Idiopathic pulmonary fibrosis (IPF) is a progressive and universally fatal lung disease, characterised by increasing fibrosis of the lung parenchyma. In this study, we aimed to quantify the health state utility values (HSUVs) for Australians with IPF and to identify the factors affecting these HSUVs.Participants of the Australian IPF Registry (AIPFR), with data on EuroQoL five dimension-five level (EQ-5D-5L) profiles were included. Pulmonary function tests (PFTs) were used to assess disease severity using three IPF -based classification systems. Stepwise multivariable linear regression models assessed the relationship between HSUVs and important demographic and clinical parameters.Query RESULTS: A total of 155 participants provided data for the analysis of HSUVs. For our base case, HSUVs ranged from - 0.57 to 1.00. Mean HSUVs for all participants was 0.65 (95% CI 0.61-0.70). In general, HSUVs decreased with increasing disease severity under all disease severity classification systems. Multivariable linear regression demonstrated a negative association between HSUVs, disease severity and having more than 2 comorbidities.Our study has shown that EQ-5D-5L has exhibited discriminatory sensitivity for the study population. We have demonstrated that disease severity and having more than two comorbidities was associated with lower HSUVs in Australians with IPF. Our findings support early diagnosis and appropriate evidence-based treatment to slow or prevent IPF progression; and identification and treatment of associated comorbidities to potentially improve health-related quality of life in people with IPF.

Background Total intravenous anesthesia usage in children remains relatively unpopular in the UK and Ireland. A postal survey by Hill et al in 2008 indicated that only 26% of Consultants used a propofol infusion at least once a month. Aims Following an increase in teaching and training opportunities in pediatric total intravenous anesthesia in the UK, we repeated the survey among Consultant members of Association of Paediatric Anaesthetists of Great Britain and Ireland and Society for Intravenous Anaesthesia, to see if this had affected total intravenous anesthesia usage in children and how practice may have changed. Methods We used an anonymous online survey sent to Association of Paediatric Anaesthetists of Great Britain and Ireland and Society for Intravenous Anaesthesia members. Results A total of 291 responses were analyzed. Total intravenous anesthesia was the default method of anesthesia in 8% of respondents and a further 46% used total intravenous anesthesia at least monthly. Overall total intravenous anesthesia usage had increased in the past year in 53%. The main indications were malignant hyperthermia susceptibility, and postoperative nausea and vomiting. Ear/nose/throat surgery was the most popular surgical specialty. The main reasons for not using total intravenous anesthesia were that it was too "fiddly" and lack of confidence in the user. Most respondents used propofol in combination with remifentanil. Over 80% used propofol target-controlled infusion. The potential for propofol-related infusion syndrome concerned many, with 74% limiting infusion duration as a result. Bispectral Index was not used routinely by the majority of anesthetists. Even though most anesthetists did not use total intravenous anesthesia routinely, 98% felt they would be confident to anesthetize an malignant hyperpyrexia-positive patient using the technique. Conclusion This survey has shown that although total intravenous anesthesia is not the default anesthetic technique for most anesthetists, overall usage in children has more than doubled in the past 10 years, with many happy to use it in a wide variety of patients and procedures.

Background Chronic hypersensitivity pneumonitis (CHP) is an immune-mediated interstitial lung disease (ILD) caused by inhalational exposure to environmental antigens, resulting in parenchymal fibrosis. By definition, a diagnosis of CHP assumes a history of antigen exposure, but only half of all patients eventually diagnosed with CHP will have a causative antigen identified. Individual clinician variation in eliciting a history of antigen exposure may affect the frequency and confidence of CHP diagnosis. Methods A list of potential causative exposures were derived from a systematic review of the literature. A Delphi method was applied to an international panel of ILD experts to obtain consensus regarding technique for the elicitation of exposure to antigens relevant to a diagnosis of CHP. The consensus threshold was set at 80% agreement, and median ≤ 2, interquartile range = 0 on a 5-point Likert scale (1, strongly agree; 2, tend to agree; 3, neither agree nor disagree; 4, disagree; 5, strongly disagree). Results In two rounds, 36/40 experts participated. Experts agreed on 18 exposure items to ask every patient with suspected CHP. Themes included CHP inducing exposures, features that contribute to an exposure's relevance, and quantification of a relevant exposure. Based on the results from the literature review and Delphi process, a CHP exposure assessment instrument was derived. Using cognitive interviews, the instrument was revised by patients with ILD for readability and usability. Conclusions This Delphi survey provides items that ILD experts agree are important to ask in all patients presenting with suspected CHP and provides basis for a systematically derived CHP exposure assessment instrument. Clinical utility of this exposure assessment instrument may be affected by different local prevalence patterns of exposures. Ongoing research is required to clinically validate these items and consider their impact in more geographically diverse settings.

Clinically meaningful change in systemic sclerosis (SSc) related interstitial lung (SSc-ILD) disease is unknown. The aim of this study was to quantify change in pulmonary function as a predictor of outcome in SSc-ILD.All patients had SSc-ILD defined by HRCT chest. All PFTs during follow-up, including FVC (L), DLCO (ml/min/mmHg) and KCO (DLCO/alveolar volume ratio; DLCO/VA) (ml/min/mmHg/L) were retrieved. The rate of change over the first four years, and percentage change in the first year of follow-up were used in ROC curve analysis to determine the best cut-off points to predict adverse outcome (home oxygen, lung transplantation, or death).Among 264 patients, there were 49 events (38 deaths, 10 supplemental oxygen, one lung transplant) over a mean (±SD) follow-up of 3.0 (±1.7) years. The rates of decline over time and percentage change over one year in each of FVC, DLCO and KCO were predictive of adverse outcome. Stable PFTs over four years gave the optimal negative predictive values (NPVs) of 88-96%. The best sensitivity-specificity trade-off was a decline in FVC of 10% and in DLCO and KCO of 15% with NPVs of 92-93%.The course that SSc-ILD takes is evident within the first 1-4 years of follow up. Patients who have no decline in PFTs over 4 years have better outcomes. A decline within one year in DLCO or KCO of 15% or more is a poor prognostic factor, and identifies patients who should be monitored more closely and considered for therapy.

There is little Australian epidemiologic data on idiopathic pulmonary fibrosis (IPF), a relatively uncommon but devastating disease. The vast geographic distances in Australia have been a major impediment for collaborative research into IPF. A collaborative national effort, the Australian IPF Registry, has been formed, launched and is recruiting successfully (n = 359, January 2014). Our experience provides unique insights for others wishing to set up IPF registries and in time for a global IPF registry.

Little is known about the prevalence of anxiety in interstitial lung disease (ILD), and the contributors to depression are not clear. The aim of this study was to determine the prevalence and predictors of anxiety and depression in people with ILD.One hundred and twenty-four individuals with ILD (age 64 years (standard deviation 12), 48 idiopathic pulmonary fibrosis) participated. Anxiety and depression were assessed using the Hospital Anxiety and Depression Scale to determine likely cases and borderline cases. Associations with demographic data, respiratory function, 6-min walk and Modified Medical Research Council Dyspnoea Scale (MMRC) were examined.The prevalence of anxiety was 31%, with clinically significant anxiety in 12%. Depression was present in 23% of individuals, with 7% having clinically significant depression. Independent predictors of anxiety were a higher MMRC score (P = 0.005, odds ratio (OR) for case 2.60, 95% confidence interval 1.37 to 4.92) and higher nadir SpO2 during walking (P = 0.003, OR for case 1.16, 1.04-1.30). Independent predictors of depression were a higher MMRC score (P = 0.006, case OR 3.84, 1.25-11.78, borderline case OR 2.44, 1.14-5.19) and a greater number of comorbidities (P = 0.003, case OR 2.02, 0.97-4.21, borderline case OR 2.26, 1.30-3.93).Anxiety and depression are present in a significant minority of individuals with ILD. Dyspnoea and comorbidities are important contributors that may be amenable to intervention.