Background: The incidence of silicosis has increased due to occupational silica exposure from artificial stone, with no treatments proven to halt or reverse the disease. Whole lung lavage (WLL) involves the instillation of fluid into the lungs to wash out silica particles and disease-causing inflammatory cells. This study aimed to determine the feasibility, safety, and possible benefit of WILL in patients with artificial stone silicosis. Methods: In this prospective observational study, people with progressive silicosis with ground glass predominant radiological changes underwent WLL. High resolution computed tomography (HRCT) chest, X-ray velocimetry (XV), lung function tests, forced oscillation technique (FOT), and cardiopulmonary exercise tests (CPET) were performed before and six months after the procedure. Results: Eight patients underwent WLL between June 2021 and November 2022. Five participants had an improvement in the International Classification of High Resolution Computed Tomography for Occupational and Environmental Respiratory Diseases (ICOERD) CT scores and reduction in XV regional ventilation distribution pre- and six months post-WLL. There was no difference in lung function [annualized rate of change in forced vital capacity (FVC) % predicted mean difference (MD) 1.81; 95% CI: -1.53 to 5.15, P=0.27; forced expiratory volume in 1 second (FEV1) % predicted MD -1.13, 95% CI: -5.08 to 2.83, P=0.55; diffusing capacity for carbon monoxide (DLCO) % predicted MD -2.62, 95% CI: -10.04 to 4.80, P=0.46]. There was no significant difference in CPET or FOT measurements. Following WILL, all patients experienced transient throat discomfort, one had fever and two required oral antibiotics. There were no serious adverse events. Conclusions: WILL for artificial stone silicosis is safe in an expert centre who has experience in performing WILL in this population, and there may be limited benefit in selected patients. Further research is required to select those who will derive the most benefit.

Background: Autoimmune pulmonary alveolar proteinosis (aPAP) results from impaired macrophage-mediated clearance of alveolar surfactant lipoproteins. Whole lung lavage has been the first-line treatment but recent reports suggest the efficacy of granulocyte–macrophage colony-stimulating factor (GM-CSF). We aimed to review the efficacy and safety of nebulised GM-CSF in aPAP. Methods: We conducted a systematic review and meta-analysis searching Embase, CINAHL, MEDLINE and Cochrane Collaborative databases (1946–1 April 2022). Studies included patients aged >18 years with aPAP receiving nebulised GM-CSF treatment and a comparator cohort. Exclusion criteria included secondary or congenital pulmonary alveolar proteinosis, GM–CSF allergy, active infection or other serious medical conditions. The protocol was prospectively registered with PROSPERO (CRD42021231328). Outcomes assessed were St George's Respiratory Questionnaire (SGRQ), 6-min walk test (6MWT), gas exchange (diffusing capacity of the lung for carbon monoxide (DLCO) % predicted) and arterial–alveolar oxygen gradient. Results: Six studies were identified for review and three for meta-analysis, revealing that SGRQ score (mean difference −8.09, 95% CI −11.88– −4.3, p<0.0001), functional capacity (6MWT) (mean difference 21.72 m, 95% CI −2.76–46.19 m, p=0.08), gas diffusion (DLCO % predicted) (mean difference 5.09%, 95% CI 2.05–8.13%, p=0.001) and arterial–alveolar oxygen gradient (mean difference −4.36 mmHg, 95% CI −7.19– −1.52 mmHg, p=0.003) all significantly improved in GM-CSF-treated patients with minor statistical heterogeneity (I2=0%). No serious trial-related adverse events were reported. Conclusions: Patients with aPAP treated with inhaled GM-CSF demonstrated significant improvements in symptoms, dyspnoea scores, lung function, gas exchange and radiology indices after treatment with nebulised GM-CSF of varying duration. There is an important need to review comparative effectiveness and patient choice in key clinical outcomes between the current standard of care, whole lung lavage, with the noninvasive treatment of nebulised GM-CSF in aPAP.

Abstract We report the case of a man with severe Guillain‐Barré syndrome who developed a persistent tracheocutaneous fistula (TCF) following prolonged tracheostomy and mechanical ventilation. Following tracheostomy decannulation, the TCF had a deleterious effect on non‐invasive positive pressure ventilation efficacy and ability to effectively clear airway secretions due to air leaking from the patent stoma. This case highlights a non‐surgical approach to TCF management that is not well‐described in the literature and presents an alternative management option for cohorts of patients in which the risk associated with surgical interventions may be undesirable.

We report the case of a previously well 36-year-old man presenting to a COVID-19 screening clinic with 9 days of objective fevers, dyspnoea, non-productive cough and sore throat. This was associated with myalgias preceded by a rash on the dorsal aspect of his hands bilaterally. Given his presentation, he underwent testing for COVID19 and was advised to self-isolate. Despite a negative SARS-CoV-2 polymerase chain reaction (PCR) test, his persisting symptoms prompted a visit to his general practitioner. He was given oral amoxicillin and sent home with a diagnosis of a lower respiratory tract infection. His symptoms worsened despite antibiotics and he eventually presented to the emergency department with a profound reduction in exercise tolerance to less than 20 m. Routine laboratory tests showed lymphopenia (lymphocytes 0.4 10/L) and mild mixed deranged liver function tests. The patient was admitted to a dedicated isolation ward and underwent two further SARS-CoV-2 PCR tests. During this time, physical interaction with clinicians was limited. Once de-isolated, patient history and examination were revisited. Physical examination revealed marked periorbital oedema (Fig. 1A,B), tender anterior thigh muscles bilaterally, and grade 4/5 proximal weakness in the upper limbs. Examination of the hands revealed erythematous papules and plaques without ulceration over the dorsal metacarpophalangeal, proximal interphalangeal and distal interphalangeal joints, thickening of the palmar aspect of the fingers, and symmetrical small joint polyarthritis (Fig. 1C,D). Given the subacute presentation and the accompanying cutaneous findings, further imaging and an autoimmune screen with myositis panel was requested. Anti-nuclear antigen was positive with a speckled pattern and 1:80 titre, and creatine kinase was 10-fold the upper limit of normal. High-resolution computed tomography of the chest demonstrated non-specific ground glass nodular opacities. Subsequent respiratory function tests demonstrated a reduced forced vital capacity suggestive of a moderate restrictive defect, as well as mildly reduced gas transfer and markedly reduced maximal respiratory pressures suggestive of decreased muscle strength. The myositis antibody panel revealed a positive result for anti-melanoma differentiation-associated gene 5 antibodies, confirming the diagnosis of anti-MDA5 dermatomyositis. He was commenced on intravenous methylprednisolone for 3 days, followed by oral prednisolone, rituximab and intravenous immunoglobulin. Early recognition and prompt commencement of treatment is important to prevent significant morbidities associated with anti-MDA5 dermatomyositis. First described in 2005, anti-MDA5 dermatomyositis has a particular phenotype that is associated with rapidly progressive interstitial lung disease (ILD). Those with anti-MDA5 antibodies have up to a 20-fold increased risk of developing severe and often fatal ILD compared with other dermatomyositis patients. There are often characteristic cutaneous signs consistent with this disease, including cutaneous ulceration, periorbital oedema, mechanic’s hands and a symmetrical polyarthropathy. Although most cases are amyotrophic, our patient had evidence of muscle involvement.

Pulmonary alveolar proteinosis (PAP) is a rare respiratory syndrome, which can be challenging to diagnose given its non-specific presentation and imaging findings. While most primary cases of PAP have an autoimmune basis, the triggers for the disease are uncertain with occupational factors increasingly thought to be important. We report the unusual complication of pneumomediastinum and bilateral pneumothoraces following endobronchial ultrasound-guided transbronchial needle aspirate in the setting of PAP. We discuss the possible physiological mechanisms of this complication, which appears to be more common in conditions with reduced lung compliance.

BackgroundPatients with interstitial lung disease (ILD) are at risk of developing nocturnal hypoxaemia due to ventilatory restriction and impaired gas exchange that worsen with supine posture and reduced ventilatory drive during sleep. This systematic review synthesised literature on the diagnostic evaluation, epidemiology, associations, management and prognosis of nocturnal hypoxaemia in ILD.MethodsOvid MEDLINE, Embase and CENTRAL databases were searched for eligible studies. Meta-analyses with subgroup analyses were conducted, where possible.ResultsFifty-three studies were included (total participant number=2590). The most common definition for clinically significant nocturnal hypoxaemia was ≥10% of total sleep time with oxyhaemoglobin saturation <90%, with pooled prevalence of 37%. There were no significant differences in pooled prevalence according to ILD subtype and comorbid obstructive sleep apnoea status. Study heterogeneity precluded meta-analysis of associations and prognosis. Diffusing capacity for carbon monoxide (DLCO) and echocardiographic features for pulmonary hypertension were consistently associated with nocturnal hypoxaemia. There were inconsistent associations between nocturnal hypoxaemia with ILD subtype and severity. Multivariable analyses in most studies demonstrated significant associations of nocturnal hypoxaemia with survival. Two small short-term intervention studies demonstrated that supplemental oxygen of 1–3 L/min corrected nocturnal hypoxaemia, with improved heart rate control during in-laboratory observation and increased serum antioxidant levels after 1 month of therapy.ConclusionNocturnal hypoxaemia is common, associated with DLCO impairment and markers suggestive of pulmonary hypertension, and a potential prognostic factor in patients in ILD. There is a need to establish a consensus definition of nocturnal hypoxaemia and evaluate long-term effects of nocturnal supplemental oxygen in ILD.

BACKGROUND:Smoking cessation intervention is a key component in the management of chronic obstructive pulmonary disease (COPD).AIMS:To evaluate the prescribing of smoking cessation therapies (SCT) among hospital clinicians and identify factors that may hinder delivery of effective interventions.METHODS:A retrospective analysis of medical records of patients admitted to the Royal Hobart Hospital with an acute exacerbation of COPD was performed. A survey of hospital clinicians was also performed to ascertain levels of training and confidence in prescribing SCT.RESULTS:Nearly all medical and non-medical hospital clinicians self-reported confidence in offering SCT (91.1 vs 82.5%, respectively, P = 0.216). However, of the 122 eligible patients in our study population, the majority did not have any form of SCT initiated during their admission (n = 68, 55.7%) and only 21 patients (17.2%) were referred to the nurse-led smoking cessation service. Very few patients were initiated on efficacious regimes such as combination-nicotine replacement therapy (n = 8, 6.6%) or varenicline (n = 2, 1.6%). Only a small proportion of hospital doctors reported confidence in prescribing varenicline and bupropion (17.2 and 6.9%, respectively). Furthermore, very few hospital doctors reported ever receiving formal training in SCT compared to non-medical hospital staff (42.2 vs 84.5%, P < 0.001).CONCLUSION:Our study highlights the real-life challenges in tackling nicotine dependence in hospitals: under-utilisation of evidence-based pharmacotherapies, limited access to formal training for doctors and poor uptake of nurse-led smoking cessation services. Granting limited prescribing rights for specialised nurses may help hospital clinicians to alleviate gaps in current clinical practice.

Interstitial lung diseases (ILD) encompass a heterogeneous group of inflammatory and fibro-proliferative disorders of varying aetiologies. Amongst these, idiopathic pulmonary fibrosis (IPF) is the most studied and archetypal fibrosing ILD. It is now recognized that a significant proportion of patients with other fibrosing ILD manifest a progressive phenotype, commonly termed as progressive fibrosing ILD (PF-ILD), with a similar disease course and underlying pathophysiology to IPF. The emergence of antifibrotic therapies has transformed the landscape of IPF management, with established efficacy in slowing disease progression, reducing acute exacerbation rates and improving survival. Similar therapeutic benefits have been shown in recent clinical trials of antifibrotic therapies in PF-ILD populations. While there is increasing clinical and research interest in PFILD, many questions remain unanswered. Using a prospective ILD registry of 346 well-characterized patients with multidisciplinary diagnosis, we explored the patient characteristics and potential healthcare implications of extending antifibrotic therapies to the PF-ILD cohort. The diagnostic criteria of PF-ILD are yet to be formalized; commonly proposed criteria include a combination of physiological, clinical and imaging progression over a 2-year period. In the INBUILD trial, the progressive phenotype was defined as: a relative forced vital capacity (FVC) decline ≥10% predicted or a combination of ≥2 of the following criteria: a relative FVC decline of 5–10% predicted, progressive symptoms or imaging changes. Of the 118 patients with non-IPF fibrosing ILD in our registry, 47.5% (n = 56) met the INBUILD trial criteria of progressive phenotype within 2-year intervals during a median follow-up of 4.3 (interquartile range 3.1–7.7) years. Most patients exhibited progression as a relative decline in FVC ≥10% predicted (n = 53). The most common diagnoses of this PF-ILD cohort were unclassifiable ILD (n = 16), hypersensitivity pneumonitis (n = 14) and connective tissue disease-associated ILD (n = 12). Time from diagnosis to progression was highly variable in our cohort (Fig. 1), with median duration of 2.24 (interquartile range 0.99–3.25) years. Contrary to previously reported physician expectations of the development of a progressive phenotype occurring 11–15 months following the diagnosis, more than 20% of patients demonstrated progression after 4 years from diagnosis. Given our data set was retrospectively analysed, there were variations in the frequency of follow-up which may have implications for when and how frequently the progressive phenotype was recognized. Nevertheless, our findings are reflective of contemporary clinical practice at a quaternary institution. Hence, longitudinal follow-up with serial assessment in non-IPF fibrosing ILD patients is warranted, including in those who may initially appear to have stable disease. The majority of PF-ILD patients (n = 43; 76.8%) in our cohort had received immunosuppressive therapy consisting of prednisolone, mycophenolate, methotrexate and azathioprine, either alone or in combination. This highlights the urgent need for alternate treatments in this population to address the deficiencies in existing therapeutic approaches. While increasing age is a major risk factor for IPF, non-IPF ILD can affect patients across all ages. We hypothesize that the PF-ILD population will be younger than that seen in IPF. Concordantly, our PF-ILD group had a significantly lower mean age of onset than the IPF group (67.4 8.8 vs 75.3 8.1 years; P < 0.01). Given this younger age of the PF-ILD population and the resultant risks of cumulative impairment over a prolonged period of time, the importance of slowing or halting disease progression is heightened in these patients. With the inflammatory pathogenic basis of many PF-ILD, concurrent use of antifibrotic and immunosuppressive therapy could be considered but would need to be weighed up against the potential higher rates of drug-related adverse effects, and requires further exploration. While there is currently minimal realworld experience on the utilization of antifibrotic therapies in PF-ILD, our local experience with IPF suggests