Incidence and prognostic significance of hypoxemia in fibrotic interstitial lung disease: an international cohort study.

BACKGROUND Hypoxemia is a cardinal feature of fibrotic interstitial lung disease (ILD). The incidence, progression, and prognostic significance of hypoxemia in patients with fibrotic ILD is currently unknown. RESEARCH QUESTION What are the epidemiology of hypoxemia and its additive prognostic value in current risk prediction model in fibrotic ILD? METHODS We identified 848 patients with fibrotic ILD (258 with idiopathic pulmonary fibrosis (IPF)) in five prospective ILD registries from Australia, Canada, and Switzerland. Cumulative incidence of exertional and resting hypoxemia from the time of diagnosis was estimated at 1-year intervals in patients with baseline 6-minute walk tests, adjusted for competing risks of death and lung transplantation. Likelihood ratio tests were used to determine the prognostic significance of exertional and resting hypoxemia for 1-year mortality/transplantation when added to the ILD-GAP model. The cohort was divided into derivation and validation subsets to evaluate performance characteristics of the extended model (the "ILD-GAP-O2" model), which included oxygenation status as a predictor. RESULTS The 1-, 2-, and 5-year overall cumulative incidence was 6.1%, 17.3%, and 40.1% for exertional hypoxemia, and 2.4%, 5.6%, and 16.5% for resting hypoxemia, which were significantly higher in IPF patients compared to non-IPF patients (p<0.001 for both). Addition of exertional or resting hypoxemia to the ILD-GAP model improved 1-year mortality/transplantation prediction (p<0.001 for both). The ILD-GAP-O2 model had improved discrimination (C-index of 0.80 vs 0.75) and model fit (Akaike information criteria of 400 vs 422) in the validation cohort, with comparable calibration. INTERPRETATION IPF patients have higher cumulative incidence of exertional and resting hypoxemia than non-IPF patients. The extended ILD-GAP-O2 model provides additional risk stratification for 1-year prognosis in fibrotic ILD

Understanding the telehealth experience of care by people with ILD during the COVID-19 pandemic: What have we learnt?

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

The interstitial lung disease multidisciplinary meeting: A position statement from the Thoracic Society of Australia and New Zealand and the Lung Foundation Australia

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)

Navigating the COVID-19 pandemic: Experiences and self-management approaches adopted by people with interstitial lung disease

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

Occupational and environmental risk factors for idiopathic pulmonary fibrosis in Australia: Case-control study

Introduction Idiopathic pulmonary fibrosis (IPF) is a lung disease of unknown cause characterised by progressive scarring, with limited effective treatment and a median survival of only 2-3 years. Our aim was to identify potential occupational and environmental exposures associated with IPF in Australia. Methods Cases were recruited by the Australian IPF registry. Population-based controls were recruited by random digit dialling, frequency matched on age, sex and state. Participants completed a questionnaire on demographics, smoking, family history, environmental and occupational exposures. Occupational exposure assessment was undertaken with the Finnish Job Exposure Matrix and Australian asbestos JEM. Multivariable logistic regression was used to describe associations with IPF as ORs and 95% CIs, adjusted for age, sex, state and smoking. Results We recruited 503 cases (mean±SD age 71±9 years, 69% male) and 902 controls (71±8 years, 69% male). Ever smoking tobacco was associated with increased risk of IPF: OR 2.20 (95% CI 1.74 to 2.79), but ever using marijuana with reduced risk after adjusting for tobacco: 0.51 (0.33 to 0.78). A family history of pulmonary fibrosis was associated with 12.6-fold (6.52 to 24.2) increased risk of IPF. Occupational exposures to secondhand smoke (OR 2.1; 1.2 to 3.7), respirable dust (OR 1.38; 1.04 to 1.82) and asbestos (OR 1.57; 1.15 to 2.15) were independently associated with increased risk of IPF. However occupational exposures to other specific organic, mineral or metal dusts were not associated with IPF. Conclusion The burden of IPF could be reduced by intensified tobacco control, occupational dust control measures and elimination of asbestos at work

Australian Idiopathic Pulmonary Fibrosis Registry : vital lessons from a national prospective collaborative project

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

Treatment of idiopathic pulmonary fibrosis in Australia and New Zealand: a position statement from the Thoracic Society of Australia and New Zealand and the Lung Foundation Australia

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

Erratum: “Baseline characteristics of idiopathic pulmonary fibrosis: Analysis from the Australian Idiopathic Pulmonary Fibrosis Registry.” Helen E. Jo, Ian Glaspole, Christopher Grainge, Nicole Goh, Peter M.A. Hopkins, Yuben Moodley, Paul N. Reynolds, Sally Chapman, E. Haydn Walters, Christopher Zappala, Heather Allan, Gregory J. Keir, Andrew Hayen, Wendy A. Cooper, Annabelle M. Mahar, Samantha Ellis, Sacha Macansh and Tamera J. Corte (European Respiratory Journal (2017) 49 (1601592) DOI: 10.1183/13993003.01592-2016)

This article from the February 2017 issue of the European Respiratory Journal was published with an error in figure 5. The legend identifying “Anti-fibrotics” and “No anti-fibrotics” in figure 5c was mistakenly transposed in production. The figure has been corrected and is reproduced below. The article has been corrected and republished online