Physical activity decline is disproportionate to decline in pulmonary physiology in IPF.

BACKGROUND AND OBJECTIVE: Patients with idiopathic pulmonary fibrosis (IPF) have reduced levels of daily physical activity (DPA); however, little is known about how DPA changes as disease progresses. We aimed to (i) describe change in DPA over 12 months, (ii) analyse its association with conventional markers of disease severity and quality of life and (iii) assess DPA as a prognostic tool. METHODS: A total of 54 patients with IPF had DPA monitored at baseline and at 6 and 12 months with a SenseWear armband for 7 consecutive days. Participants completed the Hospital Anxiety and Depression scale, St George's Respiratory Questionnaire and Leicester Cough Questionnaire at each time point and provided clinical data including forced vital capacity (FVC), diffusion capacity of carbon monoxide and 6-min walk distance (6MWD). RESULTS: Baseline and 12-month daily step count (DSC) were 3887 (395) and 3326 (419), respectively. A significant reduction in DSC (mean = 645 [260], p = 0.02) and total energy expenditure (mean = 486 kJ [188], p = 0.01) was demonstrated at 12 months. The decline in DSC over 12 months was proportionally larger than decline in lung function. Annual change in DPA had weak to moderate correlation with annual change in FVC % predicted and 6MWD (range r = 0.34-0.45). Change in physical activity was not associated with long-term survival. CONCLUSION: In IPF, decline in DPA over 12 months is significant and disproportionate to decline in pulmonary physiology and may be a useful tool for assessment of disease progression.

Ambulatory oxygen for treatment of exertional hypoxaemia in pulmonary fibrosis (PFOX trial): a randomised controlled trial.

INTRODUCTION: Interstitial lung diseases are characterised by scarring of lung tissue that leads to reduced transfer of oxygen into the blood, decreased exercise capacity and premature death. Ambulatory oxygen therapy may be used to treat exertional oxyhaemoglobin desaturation, but there is little evidence to support its efficacy and there is wide variation in clinical practice. This study aims to compare the clinical efficacy and cost-effectiveness of ambulatory oxygen versus ambulatory air in people with fibrotic interstitial lung disease and exertional desaturation. METHODS AND ANALYSIS: A randomised, controlled trial with blinding of participants, clinicians and researchers will be conducted at trial sites in Australia and Sweden. Eligible participants will be randomised 1:1 into two groups. Intervention participants will receive ambulatory oxygen therapy using a portable oxygen concentrator (POC) during daily activities and control participants will use an identical POC modified to deliver air. Outcomes will be assessed at baseline, 3 months and 6 months. The primary outcome is change in physical activity measured by number of steps per day using a physical activity monitor (StepWatch). Secondary outcomes are functional capacity (6-minute walk distance), health-related quality of life (St George Respiratory Questionnaire, EQ-5D-5L and King's Brief Interstitial Lung Disease Questionnaire), breathlessness (Dyspnoea-12), fatigue (Fatigue Severity Scale), anxiety and depression (Hospital Anxiety and Depression Scale), physical activity level (GENEActive), oxygen saturation in daily life, POC usage, and plasma markers of skeletal muscle metabolism, systematic inflammation and oxidative stress. A cost-effectiveness evaluation will also be undertaken. ETHICS AND DISSEMINATION: Ethical approval has been granted in Australia by Alfred Hospital Human Research Ethics Committee (HREC/18/Alfred/42) with governance approval at all Australian sites, and in Sweden (Lund Dnr: 2019-02963). The results will be published in peer-reviewed scientific journals, presented at conferences and disseminated to consumers in publications for lay audiences. TRIAL REGISTRATION NUMBER: ClinicalTrials.gov Registry (NCT03737409).

CT-Fluoroscopic Guidance for Performance of Targeted Transbronchial Cryobiopsy: A Preliminary Report.

BACKGROUND: Bronchoscopic transbronchial cryobiopsy is increasingly used for the histological assessment of diffuse parenchymal lung disease. Diagnostic performance may be improved by more accurate targeting of biopsy to radiologic abnormalities, and complication rates may be reduced by avoiding biopsy of pleura or larger vessels. OBJECTIVES: To report the preliminary experience of using CT-fluoroscopic guidance for accurate targeting of bronchoscopic transbronchial cryobiopsy. METHODS: Bronchoscopic cryobiopsy was performed in a hybrid CT theatre. 3D CT images were acquired following positioning of the cryoprobe in a distal airway segment. Where cryoprobe position was observed to be too close to the chest wall/diaphragm pleura, or not within the region of interest within the lung parenchyma, re-positioning of probe was undertaken and repeat 3D images were acquired to confirm positioning prior to cryobiopsy. RESULTS: CT-fluoroscopic transbronchial cryobiopsy was successfully performed in 4 patients: 3 patients with interstitial lung infiltrates, and one with an enlarging left upper lobe mass. Images were reviewed following each acquisition to accurately assess the probe position within the lung parenchyma, and relative to other thoracic structures. Intra-procedural imaging was of sufficient quality to allow the accurate positioning of the cryoprobe tip with respect to both the parenchymal region of interest and pleural surfaces. No complications were experienced, and all procedures yielded diagnostic specimens. CONCLUSIONS: Our preliminary experience confirms the feasibility of performing transbronchial cryobiopsy under CT-fluoroscopic guidance. Accurate targeting of transbronchial cryobiopsy may be achieved using CT-fluoroscopic guidance. Positioning of the probe tip, both with respect to parenchymal region of interest and to pleural surfaces, can be established with high accuracy.

Diagnosis and management of idiopathic pulmonary fibrosis: Thoracic Society of Australia and New Zealand and Lung Foundation Australia position statements summary.

INTRODUCTION: 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.

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).