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.

IntroductionRheumatoid arthritis (RA) affects approximately 0.5%–1% of the general population. Clinically significant interstitial lung diseases (ILD) develops in just under 10% of people with RA, and subclinical disease is more common. Little is known about RA-ILD in New Zealand (NZ), or the number of persons with RA in Canterbury, NZ. This study aims to determine: (1) incidence and prevalence of RA, (2) incidence and prevalence of RA-ILD, (3) clinical characteristics and risk factors for the development of RA-ILD, (4) long-term outcomes of RA-ILD, in the population resident within the Canterbury District Health Board (CDHB) catchment area.Methods and analysisPersons aged 18 years of age and older, and resident in the region covered by the CDHB with RA as well as RA-ILD will be identified by retrospective review of medical records. Prevalent as well as incident cases of RA between 1 January 2006 and 31 December 2008 and between 1 January 2011 and 31 December 2013 will be identified, and followed until 30 June 2019. Existing as well as incident cases of RA-ILD during this time will be identified. The association between the development of ILD and clinical characteristics and environmental exposures will be examined using Cox-proportional hazard models. Kaplan-Meier methods will be used to estimate survival rates for patients with RA-ILD. Mortality for people with RA and RA-ILD will also be compared with the general population of the CDHB.Ethics and disseminationData will be obtained by retrospective review of medical records. Deidentified patient data will be stored in a secure online database. Data on individual patients will not be released, and all results will only be published in aggregate. Ethical approval has been obtained from the University of Otago Human Research Ethics Committee (REF HD18/079). Results will be published in peer-reviewed medical journals and presented at conferences.Trial registration numberACTRN12619001310156; Pre-results.

Coal mine lung dust disease (CMDLD) and artificial stone (AS) silicosis are preventable diseases which have occurred in serious outbreaks in Australia recently. This has prompted a TSANZ review of Australia's approach to respiratory periodic health surveillance. While regulating respirable dust exposure remains the foundation of primary and secondary prevention, identification of workers with early disease assists with control of further exposure, and with the aims of preserving lung function and decreasing respiratory morbidity in those affected. Prompt detection of an abnormality also allows for ongoing respiratory specialist clinical management. This review outlines a medical framework for improvements in respiratory surveillance to detect CMDLD and AS silicosis in Australia. This includes appropriate referral, improved data collection and interpretation, enhanced surveillance, the establishment of a nationwide Occupational Lung Disease Registry and an independent advisory group. These measures are designed to improve health outcomes for workers in the coal mining, AS and other dust-exposed and mining industries.

AIM:In light of new therapies and guidelines for the management of idiopathic pulmonary fibrosis (IPF), and in the absence of local epidemiological data, we sought to ascertain a current estimate of the prevalence of IPF in Canterbury and to audit local practices.METHODS:We performed a retrospective observational study of patients with IPF in Canterbury, New Zealand and the wider region. Patients were identified through a systematic search of hospital records and included if they were alive on 1 January 2017, had a histological or radiological diagnosis of usual interstitial pneumonia and clinical correlation consistent with a diagnosis of IPF. Clinical data was extracted from the clinical record. Follow up was complete until April 2018.RESULTS:Sixty-eight patients were included, median follow up 33 (14-49) months. Fifteen (22.1%) patients died during follow up, median survival 19 (6.5-54) months. Estimated prevalence of IPF was 6.53/100,000 persons. Six (8.8%) patients were discussed at the Interstitial lung disease multi-disciplinary meeting. Resting Sp02 and end-of-life discussions were documented in 44 (64.7%) and 19 (27.9%) patients respectively, while oxygen therapy was prescribed to 15 (22.7%). 20/36 (55.5%) patients eligible for pirfenidone were treated. Those treated were more likely to have undergone a six-minute walk test (5/20 vs 3/48, p<0.05) or have been hospitalised in the last 12 months (12/20 vs 3/48, p<0.05). 7/20 patients remained on treatment at the end of follow-up (eight discontinued, five deceased).CONCLUSION:In this study the estimated prevalence of IPF in the Canterbury region is 6.53/100,000 persons. Furthermore, we have identified limitations in local practice relevant for service development.

Rheumatoid arthritis (RA) is a common chronic autoimmune disorder that characteristically causes joint inflammation and damage. In addition, many patients develop extraarticular manifestations which may cause significant comorbidity and premature mortality. Some respiratory tract involvement of the upper and lower airways and parenchymal disease features are unique to RA, including cricoarytenoid arthritis and RA pulmonary nodulosis, and others, especially the interstitial parenchymal involvement, occur in many other idiopathic and autoimmune diseases. The pathophysiology of lung disease is not well understood. Rheumatoid lung disease may even predate the onset of joint disease, and could be triggered by chronic airway and alveolar epithelial injury. Chronic systemic inflammation and risk factors such as cigarette smoking, infection, host genetics, and immune dysregulation are contributors. Treatment of the respiratory disease is directed at reducing the systemic inflammation of RA. Less well understood is the management of the interstitial lung disease of RA, for which antifibrotic and immune suppressive agents may be helpful. The management of RA-related lung disease is perhaps the major remaining hurdle in reduction of the disease burden related to extraarticular manifestations of this disease.

Introduction Patients with progressive idiopathic fibrotic interstitial lung disease (ILD), such as those with idiopathic pulmonary fibrosis (IPF), can have an aggressive disease course, with a median survival of only 3-5 years from diagnosis. The palliative care needs of these patients are often unmet. There are calls for new models of care, whereby the patient's usual respiratory clinician remains central to the integration of palliative care principles and practices into their patient's management, but the optimal model of service delivery has yet to be determined. Methods We developed a novel, collaborative, multidisciplinary team (MDT) meeting between our palliative care, psychology and ILD teams with the principal aim of integrating specialist care to ensure the needs of persons with ILD, and their caregivers were identified and met by referral to the appropriate service. The objective of this study was to assess the effectiveness of this novel MDT meeting on the assessment of a patient's palliative care needs. Results Significant increases in advance care planning discussions were observed, in conjunction with increased referrals to community courses and teams, following introduction of this novel MDT. Conclusions Our results suggest that our collaborative MDT is an effective platform to address patients' unmet palliative care needs. Further work is required to explore the effect of our model on achieving the preferred place of death and reductions in unplanned hospital admissions.

ObjectivesPalliative care is underused in non-malignant respiratory diseases, including interstitial lung diseases (ILDs). We investigated current practices around palliative and supportive care and explored the impact of a supportive care decision aid tool.MethodsThis was a single centre study in a UK ILD centre. Retrospective analysis of hospice referrals and patients with idiopathic pulmonary fibrosis (IPF) under the Bristol ILD (BILD) service were used to identify unmet palliative and supportive care needs. Using quality improvement methodology, we explored the impact of a supportive care decision aid on clinician behaviours for patients with ILD.Results108 patients with ILD were referred for hospice care between 2010 and 2015, representing 0.15% of all referrals, compared with a population prevalence of IPF of 0.9%. The median interval between referral and death was 124 days.Records were reviewed for 64 deceased and 89 living patients with IPF seen on July–December 2014. The decision aid was prospectively assessed with 73 patients. The deceased patients had greater markers of severity. There were no other differences between the groups.After introduction, the decision aid tool was completed for 49.3% of patients and resulted in significant increases in documented discussion of referral to palliative care (11.2%vs53.6%, p<0.01) and end-of-life discussions (15.7%vs91.8%, p<0.01). Tool completion led to an increase in referral for palliative care (2.7%vs16.7%, p<0.01).ConclusionPalliative care services are underused in ILD and a supportive care decision aid can prompt consideration of palliative and supportive care needs.

Background: The recent IPF patients9 charter has highlighted the importance of palliative care (PC) in IPF. An audit of deceased patients with fibrotic ILDs at our institution demonstrated that we did not have evidence that all patients with possible PC needs were appropriately referred. Aims: Establish existing patterns of communication and decisions around EoL and PC in IPF. Assess the impact of a supportive care decision aid for EoL and PC. Methods: We retrospectively reviewed referrals for PC, EoL and resuscitation (DNACPR) discussions amongst deceased patients and those seen in clinic between 7-12/14. We noted physiological and symptomatic markers of disease severity. We developed a supportive care decision aid, based on markers of disease severity and PC guidelines and implemented this in clinic for IPF patients, assessing its impact between 10-12/15. Results: We reviewed records for 64 deceased IPF patients and 89 patients seen 7-12/14, then prospectively assessed 73 patients. The deceased patients had greater markers of disease severity. There were no differences between the other groups. After introduction, the decision aid tool was completed for 49.3% of patients. Significantly more had documented decisions related to PC referrals and EoL/DNACPR discussions. There was no increase in referrals for PC. Conclusions: The decision aid tool increased EoL/DNACPR discussions and evidence of decision-making around PC referrals.

BACKGROUND AND OBJECTIVE:Increasing evidence implicates lymphocytes in pulmonary arterial hypertension (PAH) pathogenesis. Rats deficient in T-lymphocytes show increased propensity to develop PAH but when injected with endothelial progenitor cells are protected from PAH (a mechanism dependent on natural killer (NK) cells). A decreased quantity of circulating cytotoxic CD8+ T-lymphocytes and NK cells are now reported in PAH patients; however, the effect of lymphocyte depletion on disease outcome is unknown. METHODS:This prospective study analysed the lymphocyte profile and plasma brain natriuretic peptide (BNP) levels of patients with idiopathic PAH (IPAH), connective tissue disease-associated PAH (CTD-APAH) and matched healthy controls. Lymphocyte surface markers studied include: CD4+ (helper T-cell marker), CD8+ (cytotoxic T-cell marker), CD56/CD16 (NK cell marker) and CD19+ (mature B-cell marker). Lymphocyte deficiencies and plasma BNP levels were then correlated with clinical outcome. RESULTS:Fourteen patients with PAH (9 IPAH, 5CTD) were recruited. Three patients were deceased at 1-year follow-up; all had elevated CD4 : CD8 ratios and deficiencies of NK cells and cytotoxic CD8+ T-lymphocytes at recruitment. Patients with normal lymphocyte profiles at recruitment were all alive a year later, and none were on the active transplant list. As univariate markers, cytotoxic CD8+ T-cell and NK cell counts were linked to short-term survival. CONCLUSIONS:Deficiencies in NK cells and cytotoxic CD8+ T-cells may be associated with an increased risk of death in PAH patients. Further research is required in larger numbers of patients and to elucidate the mechanism of these findings.