7The 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, p<0.001) were strong predictors of mortality. Patients receiving anti-fibrotic medications had better survival (hazard ratio 0.56, 95% CI 0.34-0.92, p=0.022) than those not on anti-fibrotic medications, independent of underlying disease severity.The AIPFR provides important insights into the understanding of the natural history and clinical management of IPF.

BackgroundIdiopathic 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).MethodsUsing 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.ResultsOf 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.ConclusionIPF 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 materialThe online version of this article (10.1186/s12890-018-0575-y) contains supplementary material, which is available to authorized users.

Interstitial lung diseases (ILD) are a diverse group of pulmonary diseases for which accurate diagnosis is critical for optimal treatment outcomes. Diagnosis of ILD can be challenging and a multidisciplinary approach is recommended in international guidelines. The purpose of this position paper is to review the evidence for the use of the multidisciplinary meeting (MDM) in ILD and suggest an approach to its governance and constitution, in an attempt to provide a standard methodology that could be applied across Australia and New Zealand. This position paper is endorsed by the Thoracic Society of Australia and New Zealand (TSANZ) and the Lung Foundation Australia (LFA).

Background: The majority of NSCLC patients treated with immunecheckpoint inhibitors (ICI) develop acquired resistance. Conventional cytotoxic chemotherapy remains the treatment of choice for those patients. There are case reports on re-administration of ICIs for advanced NSCLC; however, these case series are difficult to draw definitive conclusions. We therefore retrospectively reviewed the efficacy of retreatment with ICI in our hospital. Method: Patients with pathologically confirmed advanced NSCLC who were treated with ICI in Kindai University hospital were retrospectively reviewed from December 2015 to July 2017. Among 212 NSCLC patients treated with ICIs, 10 patients (4.7 %) were retreated with ICI. Result: Number of patients treated with Nivolumab, Pembrolizumab and Atezolizumab were four, five and one, respectively. The best response of initial treatment with ICIs among 10 patients were five partial response (PR), two stable disease (SD) and three progressive disease (PD). Whereas, three patients (30%) showed SD and the others (70%) had PD to ICI retreatment. No severe adverse events attributable to the ICIs were noted. Conclusion: In the limited number of retrospective study, we could not find good responders for retreatment with ICIs. Best overall response during the previous treatment period is not related to the efficacy of retreatment with ICIs. At present, former responder to ICI therapy may not be the proper candidate for ICI re-challenge treatment strategy. Further biomarker analysis and treatment strategy is warranted for the patients and physicians to retreat with ICIs.

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.Using the Australian IPF Registry, we obtained data from all individuals who had completed the self-reported Hospital Anxiety and Depression Scale (HADS) at baseline and at 12 months of follow-up [2,3]. HADS scores were classified according to standard criteria where a score >10 indicates a probable case of clinically significant anxiety or depression and a score of 8-10 indicates borderline probability. We defined prolonged anxiety and depression as being present when subjects had HADS scores ⩾8 at baseline and at 12 months follow-up. We defined worsening risk of anxiety and depression as moving from "no case" to "borderline" or "case", or moving from "borderline" to "case", at 12 months.Analysis included demographic and physiologic data, and patient-reported medical comorbidities. Breathlessness was measured using the UCSD shortness of breath questionnaire (UCSDSOBQ) which, like the HADS scale, explores symptoms over the preceding week [4]. Cough severity was rated using an unvalidated visual analogue scale (VAS), measured between 0 and 100 mm, with anchors of "no cough" and "worst cough imaginable" The persistence of anxiety and depression over 12 months of follow-up was evaluated using the Fisher's exact test. Univariate relationships were tested using Pearson's Chi-squared test for categorical variables and one-way analysis of variance for continuous variables. Variables with a relationship to prolonged anxiety or depression ( p<0.1) were entered into a logistic regression model. Model fit and the proportion of participants correctly classified by the model were evaluated using the Chi-squared test.