Lauren Troy

Prevalence and prognosis of unclassifiable interstitial lung disease

To the Editor: We read with interest the recent article by Ryerson et al. [1], describing the prevalence and characteristics of patients with unclassifiable interstitial lung disease (ILD) presenting to a specialist centre. This study is the first to target specifically this newly defined disease category, in parallel with publication of the updated American Thoracic Society/European Respiratory Society classification of the idiopathic interstitial pneumonias (IIPs) [2]. The authors identified 10% of their ILD patient population as having unclassifiable ILD following multidisciplinary discussion (MDD). The major reasons for diagnostic uncertainty related to either inability or unwillingness of the patient to undergo surgical lung biopsy, or inadequacy of the tissue specimen sampled. Only a minority of cases remained ambiguous after a reasonable tissue sample had been obtained. The study detailed the clinical characteristics of this hybrid group, with many of the mean baseline demographics and disease behaviours falling between the two reference groups of patients with confirmed idiopathic pulmonary fibrosis (IPF) and non-IPF diagnoses. Multivariate analysis revealed low diffusing capacity of …

Clinical impact of the interstitial lung disease multidisciplinary service

Multidisciplinary discussions (MDDs) have been shown to improve diagnostic accuracy in interstitial lung disease (ILD) diagnosis. However, their clinical impact on patient care has never been clearly demonstrated. We describe the effect that an ILD multidisciplinary service has upon the diagnosis and management of patients with suspected ILD.

Exercise pathophysiology and the role of oxygen therapy in idiopathic interstitial pneumonia

Exercise limitation is a common feature in idiopathic interstitial pneumonia (IIP). There are multiple contributing pathophysiological mechanisms, including ventilatory mechanical limitation, impaired gas exchange, pulmonary vascular insufficiency and peripheral muscle dysfunction. Progressive exertional dyspnoea and functional incapacity impact significantly on quality of life. Exercise‐induced desaturation is frequently observed and is predictive of poorer outcomes. Tests to assess the cardiorespiratory system under stress (e.g. cardiopulmonary exercise testing and the 6‐min walk test) can provide important physiologic and prognostic information as adjuncts to resting measurements of lung function. Despite many advances in understanding disease mechanisms, therapies to improve exercise capacity, symptom burden and quality of life are lacking. Exercise training and supplemental oxygen are two potential interventions that require closer evaluation in patients with IIP.

Sleep disordered breathing in interstitial lung disease: A review

Patients with interstitial lung disease commonly exhibit abnormal sleep architecture and increased sleep fragmentation on polysomnography. Fatigue is a frequent complaint, and it is likely that poor sleep quality is a significant contributor. A number of studies have shown that sleep disordered breathing is prevalent in this population, particularly in the idiopathic pulmonary fibrosis subgroup. The factors that predispose these patients to obstructive sleep apnoea are not well understood, however it is believed that reduced caudal traction on the upper airway can enhance collapsibility. Ventilatory control system instability may also be an important factor, particularly in those with increased chemo-responsiveness, and in hypoxic conditions. Transient, repetitive nocturnal oxygen desaturation is frequently observed in interstitial lung disease, both with and without associated obstructive apnoeas. There is increasing evidence that sleep-desaturation is associated with increased mortality, and may be important in the pathogenesis of pulmonary hypertension in this population.

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.

Current Australasian practice for diagnosis and management of idiopathic pulmonary fibrosis: Where are we now?

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.

Therapy for idiopathic pulmonary fibrosis: lessons from pooled data analyses.

In 2014, two landmark clinical trials (ASCEND and INPULSIS) were published demonstrating, for the first time, a treatment benefit for patients with idiopathic pulmonary fibrosis (IPF). Both pirfenidone and nintedanib were shown to have a clear therapeutic benefit in slowing the relentless disease progression evident in IPF patients. The results of these studies have had a major impact upon management of IPF patients worldwide, with subsequent changes made to international guidelines, which now recommend in favour of antifibrotic therapy. However, this is not the whole story: there are many unanswered questions with regard to the real-world treatment benefit and use of antifibrotic therapy. In this issue of the European Respiratory Journal, Noble et al. [1] present the pooled data of the three multinational, phase 3, placebo-controlled trials of pirfenidone in IPF patients, the ASCEND, and CAPACITY 004 and 006 studies. Examining the combined patient population reveals important insights about specific subgroups along with broader inferences that are only possible through pooled analysis. The pooled analysis of three large phase 3 trials sheds further light on the role of antifibrotic therapy in IPF http://ow.ly/UNTWQ

Prevalence and Utility of Positive Pneumococcal Urinary Antigen Tests in Australian Patients with Community-Acquired Pneumonia

Background and Objectives. The pneumococcal urinary antigen test (UAT) has superior sensitivity to other investigations in determining the aetiology of community-acquired pneumonia (CAP), but data specific to Australian populations is limited. This study aimed to establish the prevalence and clinical utility of positive UAT in patients admitted to hospital with CAP, as well as associations with positive testing. Methods. A prospective, cross-sectional, single-centre study was performed. Urine antigen tests were performed on all adult patients admitted to hospital with the diagnosis of CAP. Sputum and blood culture results, CURB-65 score of severity, current and prior antibiotics, comorbidities, mortality, and length of hospital stay were recorded. Results. There was a positive test prevalence of 13/170 [7.6% (95% confidence intervals 4.3–13%)]. The overall prevalence of pneumococcal pneumonia was 19/170 (11%), including 8 patients confirmed on positive UAT alone. Patients with a positive UAT result had a higher mean CURB-65 score compared with those with a negative result (𝑃=0.01), and a greater likelihood of requiring intensive care support (𝑃=0.006). Conclusions. The prevalence of positive UAT was low. Positive results were more often recorded in those with greater severity pneumonia. The clinical utility of the test in this cohort of patients was low.

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.

Diagnosing Lung Cancer: The Complexities of Obtaining a Tissue Diagnosis in the Era of Minimally Invasive and Personalised Medicine

The role of the respiratory physician in diagnosing lung cancer has increased in complexity over the last 20 years. Adenocarcinoma is now the prevailing histopathological sub-type of non-small cell lung cancer (NSCLC) resulting in more peripheral cancers. Conventional bronchoscopy is often not sufficient to obtain adequate tissue samples for diagnosis. Radiologically guided transthoracic biopsy is a sensitive alternative, but carries significant risks. These limitations have driven the development of complimentary bronchoscopic navigation techniques for peripheral tumour localisation and sampling. Furthermore, linear endobronchial ultrasound with transbronchial needle aspiration (EBUS-TBNA) is increasingly being chosen as the initial diagnostic procedure for those with central lesions and/or radiological evidence of node-positive disease. This technique can diagnose and stage patients in a single, minimally invasive procedure with a diagnostic yield equivalent to that of surgical mediastinoscopy. The success of molecular targeted therapies and immune checkpoint inhibitors in NSCLC has led to the increasing challenge of obtaining adequate specimens for accurate tumour subtyping through minimally invasive procedures. This review discusses the changing epidemiology and treatment landscape of lung cancer and explores the utility of current diagnostic options in obtaining a tissue diagnosis in this new era of precision medicine.