Samantha Ellis

Relation of interlobar collaterals to radiological heterogeneity in severe emphysema.

Background: A study was undertaken to assess the prevalence of interlobar collateral ventilation in patients with severe emphysema to identify factors that may help to predict patients with significant collateral ventilation. Methods: Between April 2002 and August 2003, ex vivo assessment of the lungs 17 consecutive patients with smoking related severe emphysema was performed. To assess collateral flow, all lobes of explanted specimens were selectively intubated using a wedged cuffed microlaryngeal intubation tube and then manually ventilated using a bagging circuit. Interlobar collateral ventilation was defined as the ability to easily inflate a non-intubated lobe at physiological pressures. Pre-transplant demographic characteristics, physiological data, radiological results, and explant histology were assessed for retrospective relationships with the degree of interlobar collateral ventilation in the explanted lung. Results: A total of 23 lungs were evaluated, 15 of which (66%) had significant collateral interlobar airflow. There were no significant differences in any demographic, physiological, or pathological variables between patients with collateral ventilation and those with no collateral ventilation. However, there was a significant relationship between the presence of interlobar collateral ventilation and radiological scores (p<0.05). Conclusions: Interlobar collateral ventilation occurs to a much greater extent in patients with radiologically homogeneous emphysema than in those with heterogeneous emphysema. Heterogeneity of emphysema may predict patients with a significantly reduced risk of interlobar collateral ventilation.

Acute Fibrinoid Organizing Pneumonia after Lung Transplantation

RATIONALE The barrier to long-term success after lung transplantation is the development of chronic lung allograft dysfunction. As the experience with lung transplantation accrues, it has become increasingly apparent that not all chronic allograft dysfunction is consistent with the traditionally recognized small-airway histological process of obliterative bronchiolitis (OB). OBJECTIVES To identify and describe chronic allograft dysfunction that is not consistent with the well-described bronchiolitis obliterans syndrome and to further characterize a novel histopathological process, acute fibrinoid organizing pneumonia (AFOP), that has led invariably to respiratory decline and death after lung transplantation. METHODS We evaluated 194 bilateral lung transplant recipients, identifying 87 individuals who developed chronic allograft dysfunction. They were then classified according to features on spirometry, chest imaging, and histopathological specimens. MEASUREMENTS AND MAIN RESULTS Two main phenotypes of chronic allograft dysfunction were identified; 39 (45%) recipients were categorized as having developed OB and 22 (25%) as having AFOP. Survival in those who developed AFOP was significantly worse than in those who developed OB (median time to death 101 vs. 294 d; P = 0.02), with all exhibiting a rapid decline in respiratory function leading to death. CONCLUSIONS AFOP is a novel form of chronic allograft dysfunction exhibiting spirometric, radiological, and histopathological characteristics that differentiate it from OB. The further characterization of chronic allograft dysfunction and its heterogeneous manifestations will allow the targeting of clinical and experimental efforts to prevent and treat chronic allograft dysfunction.

Clinical determinants of the 6-Minute Walk Test in bronchiectasis

BACKGROUND The 6-Minute Walk Test (6MWT) is a widely used measurement of functional exercise capacity in chronic lung disease. While exercise intolerance has been identified in patients with bronchiectasis, the clinical determinants of the 6MWT in this population have not been examined. The aim of this study was to 1) establish the relationship between the 6-Minute Walk Distance (6MWD), disease severity and Health-Related Quality of Life (HRQOL) and 2) identify predictors of exercise tolerance in adults with bronchiectasis. METHODS The 6MWT was performed in 27 patients with bronchiectasis (mean [SD] FEV(1) 73.9% predicted [23.4]). Disease severity was assessed using spirometry and HRCT scoring while HRQOL was evaluated using the St Georges Respiratory Questionnaire (SGRQ) and the Short-Form 36 (SF-36). The relationships were evaluated using correlation and multiple regression. RESULTS The 6MWD correlated positively with FVC (r=0.52, p<0.01), generations of bronchopulmonary divisions (r(s)=0.38, p<0.05) and SF-36 physical summary (r=0.71, p<0.001) while a negative correlation was observed between all domains of the SGRQ (all correlations r>0.5, p<0.001). Multiple regression analysis indicated that the SGRQ activity, symptom scores and generations of bronchial divisions involved were identified as independent predictors of the 6MWD, explaining 76% of the variance. CONCLUSIONS Measures of HRQOL demonstrated a stronger association with the 6MWD compared to physiological measures of disease severity in patients with predominantly mild to moderate bronchiectasis.

Proximal and distal gastro‐oesophageal reflux in chronic obstructive pulmonary disease and bronchiectasis

The aims of this observational study were (i) to examine the prevalence of symptomatic and clinically silent proximal and distal gastro‐oesophageal reflux (GOR) in adults with chronic obstructive pulmonary disease (COPD) or bronchiectasis, (ii) the presence of gastric aspiration, and (iii) to explore the possible clinical significance of this comorbidity in these conditions.

Thoracic ultrasound demonstrates variable location of the intercostal artery

Background: Ultrasound (US) guidance is advocated to reduce complications from thoracocentesis or intercostal catheter (ICC) insertion. Although imaging of the intercostal artery (ICA) with Doppler US has been reported, current thoracic guidelines do not advocate this, and bleeding from a lacerated ICA continues to be a rare but serious complication of thoracocentesis or ICC insertion. Objectives: It was the aim of this study to describe a method to visualise the ICA at routine US-guided thoracocentesis and map its course across the posterior chest wall. Method: The ICA was imaged in 22 patients undergoing US-guided thoracocentesis, at 4 positions across the back to the axilla. Its location, relative to the overlying rib, was calculated as the fraction of the intercostal space (ICS) below the inferior border of that rib. Results: An ICA was identified in 74 of 88 positions examined. The ICA migrated from a central ‘vulnerable’ location within the ICS near the spine (0.28, range 0.21–0.38; p < 0.001) towards the overlying rib (0.08, range 0.05–0.11; p < 0.001) in the axilla. Conclusions: The ICA can be visualised with US and is more exposed centrally within the ICS in more posterior positions; however, there is a marked variation between individuals, such that the ICA may lie exposed in the ICS even as far lateral as the axilla. Future studies need to identify which patients are at risk for a ‘low-lying’ ICA to further define the role of US imaging of the ICA during thoracocentesis or ICC insertion.

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.

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.

Baseline characteristics of idiopathic pulmonary fibrosis: analysis from the Australian Idiopathic Pulmonary Fibrosis Registry

The 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. Data from the Australian IPF registry shows anti-fibrotic therapy and baseline physiology predict survival in IPF http://ow.ly/Ete2305OkU9

Isavuconazole as salvage therapy for mucormycosis

Mucormycosis carries a high mortality rate with few therapeutic options available. We describe a man with pulmonary/splenic mucormycosis complicating hypoplastic myelodysplastic syndrome on a background of chronic kidney disease, who achieved a complete response with salvage isavuconazole therapy following intolerance of consecutive courses of liposomal amphotericin and posaconazole therapy.

Positive Expiratory Pressure via Mask Does Not Improve Ventilation Inhomogeneity More than Huffing and Coughing in Individuals with Stable Chronic Obstructive Pulmonary Disease and Chronic Sputum Expectoration

Background: Positive expiratory pressure (PEP) has been used to promote airway clearance in individuals with chronic obstructive pulmonary disease (COPD) for many years; however, its mechanism of action and benefits are unclear. Previous authors have suggested that PEP improves collateral ventilation via changes in lung volumes. Objectives: It was the aim of this study to determine whether PEP improves ventilation inhomogeneity more than controlled huffing and coughing in individuals with stable COPD. Methods: Twelve participants with COPD (mean forced expiratory volume in 1 s 45% predicted) and chronic sputum expectoration performed PEP therapy (10-20 cm H2O) or controlled huffing and coughing in random order on alternate study days with a 48-hour washout. Measures of acinar and conductive airway ventilation (Sacin, Scond), lung volumes, spirometry and sputum wet weight were recorded before, immediately after and 90 min following treatment. Ease of expectoration [visual analogue scale (VAS)] and oxyhaemoglobin saturation were assessed immediately following treatment. Results: There were no significant differences between the effect of either test condition at any time point for any test parameter. Mean Sacin immediately following PEP and control conditions was 0.465 and 0.438 litre-1, respectively (p = 0.45 for comparison between conditions) and mean Scond was 0.042 and 0.039 litre-1 (p = 0.55). PEP therapy did not significantly enhance total mean sputum expectoration compared to controlled huffing and coughing (7.06 vs. 6.15 g; p = 0.51) and did not improve ease of expectoration (VAS PEP 4.8 cm vs. control 4.1 cm; p = 0.53). Conclusion: Any therapeutic benefits of PEP in individuals with COPD and chronic sputum expectoration are unlikely to be mediated by improvements in ventilation or lung volumes.