Search

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

Background and objectives For older patients with kidney failure, lowering symptom burden may be more important than prolonging life. Dialysis initiation may affect individual kidney failure-related symptoms differently, but the change in symptoms before and after start of dialysis has not been studied. Therefore, we investigated the course of total and individual symptom number and burden before and after starting dialysis in older patients.Design, setting, participants, & measurements The European Quality (EQUAL) study is an ongoing, prospective, multicenter study in patients >= 65 years with an incident eGFR

Introduction: Predicting the timing and occurrence of kidney replacement therapy (KRT), cardiovascular events, and death among patients with advanced chronic kidney disease (CKD) is clinically useful and relevant. We aimed to externally validate a recently developed CKD G4+ risk calculator for these outcomes and to assess its potential clinical impact in guiding vascular access placement. Methods: We included 1517 patients from the European Quality (EQUAL) study, a European multicentre prospective cohort study of nephrology-referred advanced CKD patients aged ≥65 years. Model performance was assessed based on discrimination and calibration. Potential clinical utility for timing of referral for vascular access placement was studied with diagnostic measures and decision curve analysis (DCA). Results: The model showed a good discrimination for KRT and “death after KRT,” with 2-year concordance (C) statistics of 0.74 and 0.76, respectively. Discrimination for cardiovascular events (2-year C-statistic: 0.70) and overall death (2-year C-statistic: 0.61) was poorer. Calibration was fairly accurate. Decision curves illustrated that using the model to guide vascular access referral would generally lead to less unused arteriovenous fistulas (AVFs) than following estimated glomerular filtration rate (eGFR) thresholds. Conclusion: This study shows moderate to good predictive performance of the model in an older cohort of nephrology-referred patients with advanced CKD. Using the model to guide referral for vascular access placement has potential in combating unnecessary vascular surgeries.

Background Various prediction models have been developed to predict the risk of kidney failure in patients with CKD. However, guideline-recommended models have yet to be compared head to head, their validation in patients with advanced CKD is lacking, and most do not account for competing risks. Methods To externally validate 11 existing models of kidney failure, taking the competing risk of death into account, we included patients with advanced CKD from two large cohorts: the European Quality Study (EQUAL), an ongoing European prospective, multicenter cohort study of older patients with advanced CKD, and the Swedish Renal Registry (SRR), an ongoing registry of nephrology-referred patients with CKD in Sweden. The outcome of the models was kidney failure (defined as RRT-treated ESKD). We assessed model performance with discrimination and calibration. Results The study included 1580 patients from EQUAL and 13,489 patients from SRR. The average c statistic over the 11 validated models was 0.74 in EQUAL and 0.80 in SRR, compared with 0.89 in previous validations. Most models with longer prediction horizons overestimated the risk of kidney failure considerably. The 5-year Kidney Failure Risk Equation (KFRE) overpredicted risk by 10%-\18%. The four- and eight-variable 2-year KFRE and the 4-year Grams model showed excellent calibration and good discrimination in both cohorts. Conclusions Some existing models can accurately predict kidney failure in patients with advanced CKD. KFRE performed well for a shorter time frame (2 years), despite not accounting for competing events. Models predicting over a longer time frame (5 years) overestimated risk because of the competing risk of death. The Grams model, which accounts for the latter, is suitable for longer-term predictions (4 years).

© 2020 The Authors. Respirology published by John Wiley & Sons Australia, Ltd on behalf of Asian Pacific Society of Respirology. Pulmonary complications in CTD are common and can involve the interstitium, airways, pleura and pulmonary vasculature. ILD can occur in all CTD (CTD-ILD), and may vary from limited, non-progressive lung involvement, to fulminant, life-threatening disease. Given the potential for major adverse outcomes in CTD-ILD, accurate diagnosis, assessment and careful consideration of therapeutic intervention are a priority. Limited data are available to guide management decisions in CTD-ILD. Autoimmune-mediated pulmonary inflammation is considered a key pathobiological pathway in these disorders, and immunosuppressive therapy is generally regarded the cornerstone of treatment for severe and/or progressive CTD-ILD. However, the natural history of CTD-ILD in individual patients can be difficult to predict, and deciding who to treat, when and with what agent can be challenging. Establishing realistic therapeutic goals from both the patient and clinician perspective requires considerable expertise. The document aims to provide a framework for clinicians to aid in the assessment and management of ILD in the major CTD. A suggested approach to diagnosis and monitoring of CTD-ILD and, where available, evidence-based, disease-specific approaches to treatment have been provided.

Background Depressive symptoms are associated with adverse clinical outcomes in patients with end-stage kidney disease; however, few small studies have examined this association in patients with earlier phases of chronic kidney disease (CKD). We studied associations between baseline depressive symptoms and clinical outcomes in older patients with advanced CKD and examined whether these associations differed depending on sex. Methods CKD patients (≥65 years; estimated glomerular filtration rate ≤20 mL/min/1.73 m2) were included from a European multicentre prospective cohort between 2012 and 2019. Depressive symptoms were measured by the five-item Mental Health Inventory (cut-off ≤70; 0–100 scale). Cox proportional hazard analysis was used to study associations between depressive symptoms and time to dialysis initiation, all-cause mortality and these outcomes combined. A joint model was used to study the association between depressive symptoms and kidney function over time. Analyses were adjusted for potential baseline confounders. Results Overall kidney function decline in 1326 patients was –0.12 mL/min/1.73 m2/month. A total of 515 patients showed depressive symptoms. No significant association was found between depressive symptoms and kidney function over time (P = 0.08). Unlike women, men with depressive symptoms had an increased mortality rate compared with those without symptoms [adjusted hazard ratio 1.41 (95% confidence interval 1.03–1.93)]. Depressive symptoms were not significantly associated with a higher hazard of dialysis initiation, or with the combined outcome (i.e. dialysis initiation and all-cause mortality). Conclusions There was no significant association between depressive symptoms at baseline and decline in kidney function over time in older patients with advanced CKD. Depressive symptoms at baseline were associated with a higher mortality rate in men.

To evaluate whether attending a face-to-face pre-operative joint replacement education in a regional setting reduces overall hospital costs and length of stay (LOS) following total knee replacement (TKR) or total hip replacement (THR). A retrospective clinical audit reviewed the medical records of all patients who underwent an elective THR or TKR at Rockhampton Hospital in regional Queensland, Australia, between 03/2015 and 12/2016 (22 months). The pre-operative joint replacement education class was provided by a multidisciplinary team that included a physiotherapist, an occupational therapist, a dietician, a pharmacist and a social worker. In addition to demographic data, we extracted and analysed data related to total acute care and total healthcare cost, prevalence of post-operative complications, discharge destination and comorbidities (using the Functional Comorbidity Index). Out of 326 cases that were included in the analysis, 115 cases with TKR and 51 cases with THR attended a pre-operative education class. Demographic characteristics between those attending and not attending the class were largely similar, except from more females attending in the THR group. There was no difference in hospital costs or LOS between those who attended the class compared to those who did not for both the TKR and THR groups. Outcomes related to total acute stay costs, total cost including travel and education and score for Functional Comorbidities Index were similar between those who attended the class and those who did not. Pre-operative education does not reduce hospital costs (surgery and hospital stay) in Central Queensland.

Introduction Estimation of left ventricular systolic function (LVEF) commonly underpins clinical decision making in Cardiology. TTE has previously been demonstrated to underestimate LVEF compared with CMR. Sheffield Teaching Hospitals Trust (STH) recently upgraded to the latest generation of TTE scanners (GE E95) and CMR scanner (Siemens Magnetom Aera 1.5T). This service evaluation investigated correlation and bias between TTE and CMR LVEF reporting at our institution. Methods Consecutive TTE studies at STH from April-October 2018 were cross-referenced, identifying patients who had also undergone CMR during this period. All patients – in and outpatients - with both TTE and CMR within 30 days were included. Patients with missing data, reversible aetiology or poor endocardial definition were excluded. The determination of LV function by echo was made using Simpson’s biplane where endocardial definition was sufficient. If not, it was determined by visual assessment and a 5% range quoted. A second opinion was sought if LV function was assessed as 55% for normal. Results 12301 TTE and 398 CMR scans were performed in the study period. 120 patients met inclusion criteria with TTE and CMR within 30 days. Reasons for exclusion included missing data (n=2), reversible cause for LV dysfunction (n=1) and poor endocardial definition (n=4) leaving a study population of 113 patients. LVEF by TTE and CMR (figure 1) were strongly correlated (R=0.85, p Conclusion These results show good correlation of LVEF by TTE and CMR with the latest generation of scanners with a signal of systematic bias where CMR LVEF reads 4.4% higher than TTE. These results are used locally to guide clinical decision making and form part of on-going quality assurance processes. Further work to improve agreement between modalities is required. Conflict of Interest None

Background and objective Current guidelines for the diagnosis of idiopathic pulmonary fibrosis (IPF) provide specific criteria for diagnosis in the setting of multidisciplinary discussion (MDD). We evaluate the utility and reproducibility of these diagnostic guidelines, using clinical data from the Australian IPF Registry. Methods All patients enrolled in the registry undergo a diagnostic review whereby international IPF guidelines are applied via a registry MDD. We investigated the clinical applicability of these guidelines with regard to: (i) adherence to guidelines, (ii) Natural history of IPF diagnostic categories and (iii) Concordance for diagnostic features. Results A total of 417 participants (69% male, 70.6 ± 8.0 years) with a clinical diagnosis of IPF underwent MDD. The 23% of participants who did not meet IPF diagnostic criteria displayed identical disease behaviour to those with confirmed IPF. Honeycombing on radiology was associated with a worse prognosis and this translated into poorer prognosis in the ‘definite’ IPF group. While there was moderate agreement for IPF diagnostic categories, agreement for specific radiological features, other than honeycombing, was poor. Conclusion In clinical practice, physicians do not always follow IPF diagnostic guidelines. We demonstrate a cohort of IPF patients who do not meet IPF diagnostic guideline criteria, based largely on their radiology and lack of lung biopsy, but who have outcomes identical to those with IPF.

Idiopathic pulmonary fibrosis (IPF) is a chronic disease characterised by progressive decline in pulmonary function. The rate of decline can vary, with some patients remaining stable over longer periods of time and others rapidly progressing [1]. tThe variable progression of this disease makes it difficult to elucidate pathogenic pathways involved in the initiation and progression of IPF. Advances in high-throughput gene-expression analyses have led to improvements in our understanding of disease biology and prognostic gene signatures. We hypothesise that IPF has a unique circulatory transcriptional profile compared to healthy controls, with additional differences between stable and progressive disease likely related to disease pathogenesis. Footnotes This manuscript has recently been accepted for publication in the European Respiratory Journal . It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article. Conflict of interest: Miss Clynick has nothing to disclose. Conflict of interest: Dr. Jo has nothing to disclose. Conflict of interest: Dr. Corte has nothing to disclose. Conflict of interest: Dr. Glaspole has nothing to disclose. Conflict of interest: Dr. Grainge has nothing to disclose. Conflict of interest: Dr. Hopkins has nothing to disclose. Conflict of interest: Dr. Reynolds has nothing to disclose. Conflict of interest: Dr. Chapman has nothing to disclose. Conflict of interest: Dr. Walters has nothing to disclose. Conflict of interest: Dr. Zappala has nothing to disclose. Conflict of interest: Dr. Keir has nothing to disclose. Conflict of interest: Dr. Cooper has nothing to disclose. Conflict of interest: Dr. Mahar has nothing to disclose. Conflict of interest: Dr. Ellis has nothing to disclose. Conflict of interest: Dr. Goh has nothing to disclose. Conflict of interest: Dr. Baltic has nothing to disclose. Conflict of interest: Miss Ryan has nothing to disclose. Conflict of interest: Dr. Tan has nothing to disclose. Conflict of interest: Dr. Moodley has nothing to disclose.

BackgroundIdiopathic pulmonary fibrosis (IPF) is a progressive lung disease in which circulatory biomarkers have the potential for guiding management in clinical practice. We assessed the prognostic role of serum biomarkers in three independent IPF cohorts: Australian Idiopathic Pulmonary Fibrosis Registry (AIPFR), Trent Lung Fibrosis (TLF) and Prospective Observation of Fibrosis in the Lung Clinical Endpoints (PROFILE).MethodsIn the AIPFR cohort, candidate proteins were assessed by ELISA as well as in an unbiased proteomic approach. LASSO (least absolute shrinkage and selection operator) regression was used to restrict the selection of markers that best accounted for the progressor phenotype at 1 year in the AIPFR cohort, and subsequently prospectively selected for replication in the validation TLF cohort and assessed retrospectively in the PROFILE cohort. Four significantly replicating biomarkers were aggregated into a progression index model based on tertiles of circulating concentrations.Results189 participants were included in the AIPFR cohort, 205 participants from the TLF cohort and 122 participants from the PROFILE cohort. Differential biomarker expression was observed by ELISA and replicated for osteopontin, matrix metallopeptidase-7, intercellular adhesion molecule-1 and periostin for those with a progressor phenotype at 1 year. Proteomic data did not replicate. The progression index in the AIPFR, TLF and PROFILE cohorts predicted risk of progression, mortality and progression-free survival. A statistical model incorporating the progression index demonstrated the capacity to distinguish disease progression at 12 months, which was increased beyond the clinical GAP (gender, age and physiology) score model alone in all cohorts, and significantly so within the incidence-based TLF and PROFILE cohorts.ConclusionA panel of circulatory biomarkers can provide potentially valuable clinical assistance in the prognosis of IPF patients.

Idiopathic pulmonary fibrosis (IPF) is a fibrosing interstitial lung disease associated with debilitating symptoms of dyspnoea and cough, resulting in respiratory failure, impaired quality of life and ultimately death. Diagnosing IPF can be challenging, as it often shares many features with other interstitial lung diseases. In this article, we summarise recent joint position statements on the diagnosis and management of IPF from the Thoracic Society of Australia and New Zealand and Lung Foundation Australia, specifically tailored for physicians across Australia and New Zealand. Main suggestions: A comprehensive multidisciplinary team meeting is suggested to establish a prompt and precise IPF diagnosis. Antifibrotic therapies should be considered to slow disease progression. However, enthusiasm should be tempered by the lack of evidence in many IPF subgroups, particularly the broader disease severity spectrum. Non-pharmacological interventions including pulmonary rehabilitation, supplemental oxygen, appropriate treatment of comorbidities and disease-related symptoms remain crucial to optimal management. Despite recent advances, IPF remains a fatal disease and suitable patients should be referred for lung transplantation assessment.

Idiopathic pulmonary fibrosis (IPF) is a fibrosing interstitial lung disease (ILD) of unknown aetiology with a median survival of only 2–5 years. It is characterized by progressive dyspnoea and worsening lung function, ultimately resulting in death. Until recently, there were no effective therapies for IPF; however, with the publication of two landmark clinical trials in 2014, the anti-fibrotic therapies, nintedanib and pirfenidone, have gained widespread approval. This position paper aims to highlight the current evidence for the treatment of IPF, with particular application to the Australian and New Zealand population. We also consider areas in which evidence is currently lacking, especially with regard to the broader IPF severity spectrum and treatment of co-morbid conditions. The utility of non-pharmacological therapies including pulmonary rehabilitation, oxygen as well as symptom management thought to be important in the holistic care of IPF patients are also discussed.

Background and objective Studies analysing the effect of worsening pulmonary physiological impairment in idiopathic pulmonary fibrosis (IPF) with respect to quality of life have been limited to single centres or highly selected trial populations. The aim of this study was to determine the principal determinants of baseline and longitudinal health-related quality of life (HRQoL) in a large unselected IPF population. Methods We used the Australian IPF Registry to examine the relationship between HRQoL, measured using the St George Respiratory Questionnaire (SGRQ), and demographic features, physiological features, co-morbidities and symptoms. Linear regression analysis was performed to identify predictors of baseline HRQoL, linear mixed model analysis to determine the effect of time and forced vital capacity (FVC) on SGRQ and Cox proportional hazards regression to examine the relationship between HRQoL and all-cause mortality. Results Baseline data from 516 patients were available (347 males; mean (SD) age: 71.3 ± 8.6 years). Univariate analysis showed significant associations between HRQoL and demographic, clinical and physiological features. However, multivariate analysis demonstrated independent associations only between SGRQ and dyspnoea (University of California San Diego Shortness of Breathlessness Questionnaire (UCSD-SOBQ); R2 = 0.71, P

Introduction and Aim: Spirometry is the gold standard for monitoring lung function in CF, however has been reported to cause patient discomfort. IOS is an alternative diagnostic tool to monitor lung function. The aim of this study is to compare patient preference and assess patient discomfort among adult CF patients in using IOS and spirometry. Methods: Two prospective studies are being conducted simultaneously to compare the sensitivity of IOS and spirometry in detecting lung function improvements in CF patients commencing on ORKAMBI® and receiving treatment for pulmonary exacerbations respectively. Patients were given questionnaires after lung function measurements to assess patient preference between the two diagnostic methods. The patients were asked to rate their level of discomfort on a scale of 0 to 10 for each test. The same questionnaire was used in both prospective studies and results were combined for data analysis. Results: Total of 37 patients were recruited; 18 patients from CF exacerbation study and 19 from ORKAMBI® study. The mean±SD age was 32.2±7.6 years; 31 patients were males (82%). The majority of patients (76%, n=28) preferred performing IOS. Eight patients (22%) preferred spirometry in comparison to IOS while one patient (3%) had no preference. Eight patients (22%) reported experiencing discomfort while performing spirometry compared to one patient (3%) using IOS. Spirometry had an overall mean rating score of 2.2±2.3 compared to mean rating score of 0.6±1.0 in IOS, which was statistically different (p Conclusion: Adult CF patients prefer to perform IOS. More patients experienced discomfort when performing spirometry.

Introduction: Spirometry is the gold standard for detecting and monitoring lung function decline in CF exacerbation. IOS is a newer diagnostic tool that detects airway resistance. The aim of this study is to compare IOS and spirometry in evaluating the effectiveness of intravenous antibiotic treatment for CF exacerbation. Methods: CF patients commencing intravenous antibiotic treatment for pulmonary exacerbations were prospectively recruited into the study. IOS and spirometry were performed prior to and weekly during antibiotic treatment. Results: Eleven patients aged 31.9±8.1 years(mean±SD) have completed the study to date. Mean pre-exacerbation FEV1 and FVC were 2.9±1.0L(71.2±22.8% predicted) and 4.2±1.3L(85.6±19.1% predicted). Subjects received antibiotics for mean 17.2 days(median 14 to 28 days). Paired t-tests were used to determine statistical significance pre and post antibiotic treatment. Significant improvement in spirometry was seen but not in the IOS parameters(Figure 1). Using linear mixed-effects model controlling for repeated measurements over time, correlations were seen between spirometry and IOS resistance values. Conclusion: Although we have not shown a significant change in IOS parameters post treatment, modelling demonstrates a significant correlation between spirometry and IOS resistance values over time, suggesting that with increased patient numbers we will also demonstrate significant change in IOS parameters.

Invasive tracheobronchial aspergillosis is a rare disease with most reported cases in the literature occurring in immunocompromised hosts. We report an unusual case of a patient with persistent cough and dyspnoea in the context of prior chemoradiotherapy for primary lung cancer. Chest computed tomography (CT) demonstrated an abnormal soft tissue mass surrounding the trachea and carina, with focal moderate fluorodeoxyglucose (FDG) activity on positron emission tomography (PET) scan. Bronchoscopic biopsy revealed fungal hyphae associated with necrosis and cartilage invasion, subsequently confirmed to be Aspergillus fumigatus complex. The patient was commenced on antifungal therapy promptly and had a good clinical response to treatment.

Idiopathic pulmonary fibrosis (IPF) is a chronic disease characterised by an overall poor prognosis. The rate of disease decline and progression to death can vary, with some patients remaining stable over longer periods of time and others displaying rapid progression [1, 2]. The highly heterogeneous nature of this disease makes it difficult to elucidate the pathways involved in the initiation and progression of IPF and what determines the clinical trajectory of these patients. Advances in high-throughput gene expression analyses have led to improvements in our understanding of disease biology with insights into prognostic and predictive gene signatures [3]. This study sought to characterise differences in gene expression profiles found in the circulation between “stable” and “progressive” IPF patients, to help identify relevant biomarkers that may provide insights into the pathogenesis of progressive IPF. Footnotes This manuscript has recently been accepted for publication in the European Respiratory Journal . It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article. Conflict of interest: Miss Clynick has nothing to disclose. Conflict of interest: Dr. Jo has nothing to disclose. Conflict of interest: Dr. Corte has nothing to disclose. Conflict of interest: Dr. Glaspole has nothing to disclose. Conflict of interest: Dr. Grainge has nothing to disclose. Conflict of interest: Dr. Hopkins has nothing to disclose. Conflict of interest: Dr. Reynolds has nothing to disclose. Conflict of interest: Dr. Chapman has nothing to disclose. Conflict of interest: Dr. Walters has nothing to disclose. Conflict of interest: Dr. Zappala has nothing to disclose. Conflict of interest: Dr. Keir has nothing to disclose. Conflict of interest: Dr. Cooper has nothing to disclose. Conflict of interest: Dr. Mahar has nothing to disclose. Conflict of interest: Dr. Ellis has nothing to disclose. Conflict of interest: Dr. Goh has nothing to disclose. Conflict of interest: Dr. Baltic has nothing to disclose. Conflict of interest: Miss Ryan has nothing to disclose. Conflict of interest: Dr. Tan has nothing to disclose. Conflict of interest: Dr. Moodley has nothing to disclose.

Background and objective Recent international consensus statements have refined evidence-based guidelines for the diagnosis and management of idiopathic pulmonary fibrosis (IPF). This study sought to investigate how closely these guidelines are adhered to and to compare current practices with those of a similar cohort 15 years ago. Methods A questionnaire on IPF diagnosis and management was distributed to respiratory physicians practising in Australia and New Zealand, in 2012-2013, and results were compared with a similar survey conducted in 1999. Results A total of 172 and 144 questionnaires were completed in 1999 and 2012-2013, respectively. The most important investigations in both survey populations were high-resolution computed tomography scans, spirometry, diffusing capacity for carbon monoxide, chest X-ray, static lung volumes and autoimmune serology. In 1999, physicians were more likely to perform arterial blood gases, bronchoalveolar lavage and transbronchial lung biopsy. In the 2012-2013 cohort, 6-min walk tests and pulse oximetry were more widely utilized. Treatment choices differed considerably between the two survey populations. In 1999, the majority would offer a steroid-based regimen, whereas most would not use any specific treatment or would refer for trial participation in 2012-2013. Conclusions Approach to IPF diagnosis and management is not uniform and has changed over 15 years. Surveyed respiratory physicians were generally practising in accordance with clinical guidelines, although significant variation in practice was identified in both cohorts. This study identifies the need to standardize care of IPF patients across Australia and New Zealand.

Background Idiopathic pulmonary fibrosis (IPF) is a progressive and fatal fibrosing lung disease of unknown cause. The advent of anti-fibrotic medications known to slow disease progression has revolutionised IPF management in recent years. However, little is known about the natural history of IPF patients with mild physiological impairment. We aimed to assess the natural history of these patients using data from the Australian IPF Registry (AIPFR). Methods Using our cohort of real-world IPF patients, we compared FVC criteria for mild physiological impairment (FVC ≥ 80%) against other proposed criteria: DLco ≥ 55%; CPI ≤40 and GAP stage 1 with regards agreement in classification and relationship with disease outcomes. Within the mild cohort (FVC ≥ 80%), we also explored markers associated with poorer prognosis at 12 months. Results Of the 416 AIPFR patients (mean age 70.4 years, 70% male), 216 (52%) were classified as ‘mild’ using FVC ≥ 80%. There was only modest agreement between FVC and DLco (k = 0.30), with better agreement with GAP (k = 0.50) and CPI (k = 0.48). Patients who were mild had longer survival, regardless of how mild physiologic impairment was defined. There was, however, no difference in the annual decline in FVC% predicted between mild and moderate-severe groups (for all proposed criteria). For patients with mild impairment (n = 216, FVC ≥ 80%), the strongest predictor of outcomes at 12 months was oxygen desaturation on a 6 min walk test. Conclusion IPF patients with mild physiological impairment have better survival than patients with moderate-severe disease. Their overall rate of disease progression however, is comparable, suggesting that they are simply at different points in the natural history of IPF disease. Electronic supplementary material The online version of this article (10.1186/s12890-018-0575-y) contains supplementary material, which is available to authorized users.

Introduction Idiopathic pulmonary fibrosis (IPF) is a progressive disease ultimately resulting in respiratory failure and death. While decline in FVC is often used as an endpoint in clinical trials, it is unclear whether this reflects longer term outcomes, especially given significant phenotypic heterogeneity of IPF. Methods Patients from the Australian IPF Registry with ≥ 2 lung function tests performed within 1 yr were included. Using regression modelling the trajectory of FVC %pred was obtained for each patient over 1yr. Patients were then categorised as: ‘decliners’ if annual slope change was ≥10% decline or ‘stable’. Baseline characteristics were compared and outcomes post 1 yr were assessed. Results 217 subjects (Age 70.2±8.2yrs, 74% male) with IPF were included: 148 stable; 69 decliners. Patients who declined had higher baseline FVC%pred (85.4% vs 79.1%; p= 0.034), but there was no other differences in demographics or other measures of lung function. There were 57 (26%) deaths recorded, with no difference found for mortality between the two groups using Cox analysis (p=0.376). There was also no difference in the post 1 yr annual FVC %pred decline using linear mixed models, with initial decliners falling 3.9% p.a. thereafter, while the initially stable patients fell 4.3% p.a. (p=0.827). The median post-first yr follow-up time was 1.1 yrs (IQR 0.64 to 1.88 yrs), amounting to a total of 280 patient years. Conclusion It is not possible to predict patient outcomes on the basis of the 1 yr decline in FVC. There is a need for improved biomarkers to predict disease behaviour in individual patients, and inform management decisions.

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.

Idiopathic pulmonary fibrosis (IPF) is progressive with a median survival of 2-3yrs. The natural history is highly variable and difficult to predict for an individual presenting with early IPF. Aim: To identify markers associated with disease progression for IPF patients; especially those with early disease. Method: The Australian IPF Registry recruits IPF patients across Australia collating clinical, physiological and questionnaire data. Using this real-world longitudinal cohort we compared mild disease(FVC >80%) with FVC 10% or DLco>15% ± death). Result: Baseline data in 631 participants revealed: 433(69%) male, mean age 70.1±8.5yrs; mean FVC 81±22% and DLco 49±17%. Patients with mild disease(n=235) were older(p=0.001) and more often female(p Reduced PFS was associated with: male gender, impaired quality of life(SGRQ), depression, cough severity, and lower baseline FVC and DLco. Patients with mild disease had improved PFS compared to those with FVC In the subset of patients with mild disease, 41 (18%) had disease progression at 12mths. In mild IPF, disease progression at 12mths was associated with: 6MWT end SpO2 (OR 0.91, 95%CI 0.84-0.99; p=0.028), 6MWT distance and cough severity. On multivariate regression, the variable most closely associated with death at 12mths was 6MWT end SpO2. Conclusion: Impaired baseline lung function is associated with a worse PFS in IPF patients. However a significant proportion of patients with mild disease also show disease progression at 12mths.

The prevalence of idiopathic pulmonary fibrosis (IPF), a fatal and progressive lung disease, is estimated at 1.25–63 out of 100 000, making large population studies difficult. Recently, the need for large longitudinal registries to study IPF has been recognised.The Australian IPF Registry (AIPFR) is a national registry collating comprehensive longitudinal data of IPF patients across Australia. We explored the characteristics of this IPF cohort and the effect of demographic and physiological parameters and specific management on mortality.Participants in the AIPFR (n=647, mean age 70.9±8.5 years, 67.7% male, median follow up 2 years, range 6 months–4.5 years) displayed a wide range of age, disease severity and co-morbidities that is not present in clinical trial cohorts. The cumulative mortality rate in year one, two, three and four was 5%, 24%, 37% and 44% respectively. Baseline lung function (forced vital capacity, diffusing capacity of the lung for carbon monoxide, composite physiological index) and GAP (gender, age, physiology) stage (hazard ratio 4.64, 95% CI 3.33–6.47, pThe AIPFR provides important insights into the understanding of the natural history and clinical management of IPF.

Oral interviews in focus groups and written surveys were conducted with 877 men and women, including administrators, faculty members, postdoctoral associates, and graduate students, during one-day site visits to chemistry and chemical engineering departments at 28 Ph.D.-granting institutions. This report is a preliminary review of the perceptions of the situation for female tenured and tenure-track academic chemists based on the data collected during these visits. Some interesting differences are seen in responses at departments with more female faculty members as compared with departments with fewer female faculty members. Although many women are thriving, some feel isolated and marginalized. Gender barriers to success persist on both individual and institutional levels; changing this presents a serious and continuing challenge.

Background: IPF is a progressive, fibrosing lung disease causing diminished health related quality of life (HRQoL). Aims: We sort to determine the principal determinants of HRQoL in IPF. Methods: Data from the Australian IPF Registry included demographic features, comorbidities, St George Respiratory Questionnaire (SGRQ), Hospital Anxiety & Depression (HAD-A & D) scale, UCSD Shortness of BreathQuestionnaire (UCSD), cough, oxygen use, spirometry, DLCO & 6MWT parameters. Linear regression analysis was performed to identify predictors of HRQoL. Results: Data from 516 patients was available (347 male; mean (SD) age 71.3±8.6 yrs). Mean FVC 81.3±22.5%pr, DLco 46.7±17.4%pr, 6MWD 434±135m, resting SpO 2 95.1±3.3%, end-exercise SpO 2 84.8±6.9%. SGRQ: Symptom 47.2±23.5; Activity 61.9±23.9; Impact 37.3±22.9; Total 46.6±20.9. Symptom scores: UCSD median (IQR) 38 (17-67); HAD-A Score 4 (2-7), 20.3% > 8, HAD-D Score 4 (2-7), 17.7% > 8; cough presence 88.5%; cough severity (visual analogue scale 0-100mm): 40.3±25.8. Significant associations with HRQoL on univariate analysis included: GAP index, smoking, cardiorespiratory comorbidity, oxygen therapy, UCSDSOBQ, HAD-A, HAD-D, cough presence & severity, FEV1, FVC, DLCO, 6MWD, baseline & end test oxygen saturation, & end 6MWT dyspnea score. Multivariate analysis of 109 subjects with complete data demonstrated independent association between SGRQ and UCSDSOBQ (R 2 = 0.76, p 2 = 0.07, p 2 = 0.02, p=0.003) & age (R 2 = 0.01, p=0.015). Conclusions: Cough, dyspnoea & depression, are major determinants of HRQoL in IPF. Their treatment may improve HRQoL, & should be a focus of future clinical trials.

Vibrational energy transfer from hot pyrazine (E‘ = 40 322 cm-1) to cold CO is modeled using classical trajectories. Collisional energy transfer properties are studied as a function of the initial rotational state J‘ of the CO, the length of the CO, the energy E‘ in pyrazine, the relative kinetic energy, the temperature, isotopic substitution on pyrazine, and the intermolecular potential. The energy transfer probability function P(E,E‘) exhibits distinct deviation from single exponential behavior. Collisions that transfer particularly large energy are associated with large amplitude out-of-plane motion of a C−H bond, imparting a kick to the departing CO. Slower collisions are particularly effective in relaxing pyrazine; faster collisions can add energy as often as they remove it. Energy transfer properties also depend on the initial rotational state of the CO. Temperature effects on 〈E − E‘〉 are weak in the 200−500 K range; this results from an increase in the magnitudes of both 〈ΔE〉down and 〈ΔE〉up. The f...

We have recently shown that anxiety and depression are common comorbidities for people with interstitial lung disease (ILD). In a cross-sectional single-centre study, the prevalence of anxiety was 31% and the prevalence of depression was 23% [1]. Anxiety and depression were not related to physiological parameters; however, dyspnoea and number of comorbidities were important contributors. The aims of this study were to determine the frequency of prolonged anxiety and depression among sufferers of idiopathic pulmonary fibrosis (IPF), and factors contributing to their persistence., Prolonged anxiety and depression occur frequently in IPF and strongly relate to dyspnoea and cough http://ow.ly/iWxV30cLCfl

Classical trajectory calculations for collisions between vibrationally hot pyrazine (E = 40332 cm-1) and room-temperature CO are compared with recent diode laser experiments. Ab initio calculations were carried out to determine parameters for the pairwise Lennard-Jones intermolecular potential. The trajectory results appear to show good qualitative agreement with preliminary experimental results. The highest ΔE collisions occur when the CO happens to lie above the pyrazine plane just as the underlying CH wag executes an unusually high amplitude motion. Artificially doubling the length of the CO produces results for the angular momentum transfer to the CO molecule that look quite similar to those for angular momentum transfer to CO2 in analogous pyrazine−CO2 experiments.

Quasiclassical trajectories have been used to investigate the heavy−light−heavy ion−molecule reaction, O- + HF → OH + F-, using approximate potential energy surfaces. Multiple surface effects are neglected. 1D and 3D dynamics on the same surface produce very different vibrational distributions. OH product vibrational distributions are relatively insensitive to details of the potential. Product rotational energy distributions, on the other hand, are quite sensitive to features of the potential surface, particularly the steepness of the bend. Long-range ion−dipole forces play an important role in the dynamics, particularly in the angular distributions. Product vibrational energy and angular distributions are compared to two sets of experiments.