Weight loss with GLP-1 analogues in preparation for transplantation.

This case describes a woman in her 20s with a 6-month history of progressive exertional dyspnoea and cough. Examination revealed hypoxia on room air, sinus tachycardia, finger clubbing and bibasal inspiratory crackles. Inflammatory markers were mildly elevated and empirical antimicrobial therapy was commenced. A multidisciplinary discussion consensus diagnosis of acute interstitial pneumonitis was made based on the findings of high-resolution CT of the chest, macrophage predominant bronchoalveolar lavage cell differential and surgical lung biopsy. There was clinical and radiological deterioration despite glucocorticoids and antifibrotic therapy. A body mass index of 37.5 kg/m2 precluded her from lung transplant assessment and consideration. Following consultation with the weight management service, she was commenced on glucagon-like peptide 1 (GLP-1) analogue therapy. She had a remarkable response within 6 months, was listed for lung transplantation, and within 18 months of her initial presentation, a double lung transplantation was performed.

Diffuse pulmonary meningotheliomatosis: a rare cause of multiple pulmonary nodules.

This case describes a woman in her 50s who presented with recurrent lower respiratory tract infections. She was an ex-smoker and had worked on a livestock farm for many years. Chest radiograph and CT of the chest revealed multiple bilateral pulmonary nodules. Bronchoalveolar lavage and transbronchial biopsy did not confirm a unifying diagnosis and thus, surgical biopsy was pursued. Video-assisted thoracoscopic surgical guided biopsy of the right upper, middle and lower lobes demonstrated intraparenchymal minute nodules, consisting of bland epithelioid cells without any evidence of malignancy. The nodules stained positive for neural cell adhesion molecule (CD56) and progesterone receptor with weakly positive epithelial membrane antigen and smooth muscle actin. The combination of this characteristic staining pattern, the diffuse subcentimetre nature of the nodules and this clinical presentation fit with a diagnosis of the ultra-rare pulmonary disease, diffuse pulmonary meningotheliomatosis (DPM). This case highlights a rare cause of bilateral diffuse pulmonary nodules and thus, the breadth of differential diagnoses that need to be considered when approaching such a finding. Careful history-taking and thorough workup is often needed, typically requiring input from multiple specialties. DPM, while rare, should not be overlooked when considering the underlying cause of this presentation, especially in female patients. This case reiterates how common clinical presentations can unveil rare conditions and the contributions of physicians, pathologists and radiologists in the diagnosis and management of these complex diseases.

Analysis of tissue lipidomics and computed tomography pulmonary fat attenuation volume (CTPFAV ) in idiopathic pulmonary fibrosis.

BACKGROUND AND OBJECTIVE: There is increasing interest in the role of lipids in processes that modulate lung fibrosis with evidence of lipid deposition in idiopathic pulmonary fibrosis (IPF) histological specimens. The aim of this study was to identify measurable markers of pulmonary lipid that may have utility as IPF biomarkers. STUDY DESIGN AND METHODS: IPF and control lung biopsy specimens were analysed using a unbiased lipidomic approach. Pulmonary fat attenuation volume (PFAV) was assessed on chest CT images (CTPFAV ) with 3D semi-automated lung density software. Aerated lung was semi-automatically segmented and CTPFAV calculated using a Hounsfield-unit (-40 to -200HU) threshold range expressed as a percentage of total lung volume. CTPFAV was compared to pulmonary function, serum lipids and qualitative CT fibrosis scores. RESULTS: There was a significant increase in total lipid content on histological analysis of IPF lung tissue (23.16 nmol/mg) compared to controls (18.66 mol/mg, p = 0.0317). The median CTPFAV in IPF was higher than controls (1.34% vs. 0.72%, p < 0.001) and CTPFAV correlated significantly with DLCO% predicted (R2 = 0.356, p < 0.0001) and FVC% predicted (R2 = 0.407, p < 0.0001) in patients with IPF. CTPFAV correlated with CT features of fibrosis; higher CTPFAV was associated with >10% reticulation (1.6% vs. 0.94%, p = 0.0017) and >10% honeycombing (1.87% vs. 1.12%, p = 0.0003). CTPFAV showed no correlation with serum lipids. CONCLUSION: CTPFAV is an easily quantifiable non-invasive measure of pulmonary lipids. In this pilot study, CTPFAV correlates with pulmonary function and radiological features of IPF and could function as a potential biomarker for IPF disease severity assessment.

A Man With Malaise, Myalgia, and Rapidly Progressive Interstitial Lung Disease.

CASE PRESENTATION: A 45-year-old man sought treatment at the ED during the third wave of the COVID-19 pandemic with a month-long history of fatigue, cough, myalgia, and hand stiffness. He did not report dyspnea. He had no past medical history and previously was fit and active, working as a farmer. He was a lifelong nonsmoker and had no family history of lung disease.

Exosomes from EGFR-Mutated Adenocarcinoma Induce a Hybrid EMT and MMP9-Dependant Tumor Invasion.

Exosomes, a class of extra cellular nano-sized vesicles (EVs), and their contents have gained attention as potential sources of information on tumor detection and regulatory drivers of tumor progression and metastasis. The effect of exosomes isolated from patients with an Epidermal Growth Factor Receptor (EGFR)-mutated adenocarcinoma on the promotion of epithelial-mesenchymal transition (EMT) and invasion were examined. Exosomes derived from serum of patients with EGFR-mutated non-small cell lung cancer (NSCLC) mediate the activation of the Phosphoinositide 3-kinase (PI3K)/AKT/ mammalian target of rapamycin (mTOR) pathway and induce an invasion through the up-regulation of matrix metalloproteinase-9 (MMP-9) in A549 cells. We observed a significant increase in the expression of vimentin, a mesenchymal marker, while retaining the epithelial characteristics, as evidenced by the unaltered levels of E-cadherin and Epithelial cell adhesion molecule (EPCAM). We also observed an increase of nuclear factor erythroid 2-related factor 2 (NFR2) and P-cadherin expression, markers of hybrid EMT. Exosomes derived from EGFR-mutated adenocarcinoma serum could be a potential mediator of hybrid EMT and tumor invasion. Understanding how cancerous cells communicate and interact with their environment via exosomes will improve our understanding of lung cancer progression and metastasis formation.

Vaping-Associated Lung Injury: A Review.

Since commercial development in 2003, the usage of modern electronic cigarette (e-cigarette) continues to increase amongst people who have never smoked, ex-smokers who have switched to e-cigarettes, and dual-users of both conventional cigarettes and e-cigarettes. With such an increase in use, knowledge of the irritative, toxic and potential carcinogenic effects on the lungs is increasing. This review article will discuss the background of e-cigarettes, vaping devices and explore their popularity. We will further summarise the available literature describing the mechanism of lung injury caused by e-cigarette or vaping use.

Pulmonary renal syndrome: a clinical review.

The term "pulmonary renal syndrome" describes a clinical syndrome which is characterised by the presence of both diffuse alveolar haemorrhage and glomerulonephritis. It encompasses a group of diseases with distinctive clinical and radiological manifestations, as well as different pathophysiological processes. The most common diseases implicated are anti-neutrophil cytoplasm antibodies (ANCA)-positive small vessel vasculitis and anti-glomerular basement membrane (anti-GBM) disease. Prompt recognition is required as respiratory failure and end-stage renal failure can rapidly occur. Treatment includes a combination of glucocorticoids, immunosuppression, plasmapheresis and supportive measures. The use of targeted treatments has significantly reduced mortality. Thus, an understanding of pulmonary renal syndrome is essential for the respiratory physician.

Characteristics of chronic thromboembolic pulmonary hypertension in Ireland.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare and under-recognised complication of acute pulmonary embolism. Information regarding the characteristics of CTEPH in Ireland is limited, and the aim of this retrospective cohort study was to address this knowledge gap. Seventy-two cases of CTEPH were diagnosed in the National Pulmonary Hypertension Unit (NPHU) in Ireland between 2010 and 2020. This accounted for 6% of all referrals to the unit and translates to an estimated annual incidence of 1.39 per million population (95% confidence interval, 0.33-2.46). The prevalence of diagnosed CTEPH in Ireland in 2020 was estimated at 12.05 per million population (95% CI 9.00-15.10). The average duration of symptoms prior to CTEPH diagnosis was 23 (±22) months. Patients with CTEPH were more likely to be male (n = 40, 56%), older (60 ± 17 years) and have identifiable risk factors for CTEPH (n = 61, 85%) at diagnosis. Regarding treatment, pulmonary hypertension (PH) vasodilator therapy was prescribed in 75% (n = 54) within 12 months of diagnosis, inferior vena cava filters were placed in 24% (n = 17) and 97% (n = 70) of cases were anticoagulated. Pulmonary endarterectomy was performed in 35% (n = 25), balloon pulmonary angioplasty in 6% (n = 4). One-, three- and five-year survival was 93%, 80% and 65% from the time of diagnosis, and this was significantly better in patients who underwent pulmonary endarterectomy (p = 0.01). This is the first study describing the characteristics of CTEPH in Ireland and highlights suboptimal disease recognition and referral for the assessment for pulmonary endarterectomy.

Unclassifiable, or simply unclassified interstitial lung disease?

PURPOSE OF REVIEW: Unclassifiable interstitial lung disease (ILD) comprises a subset of ILDs which cannot be classified according to the current diagnostic framework. This is a likely a heterogeneous group of diseases rather than a single entity and it is poorly defined and hence problematic for prognosis and therapy. RECENT FINDINGS: With increased treatment options for progressive fibrosing ILD it is increasingly relevant to correctly categorise ILD. SUMMARY: This review article will summarise the definition and reasons for a diagnosis of unclassifiable ILD, the current management options and possible future approaches to improve diagnosis and differentiation within this broad subset. Finally, we will describe the implications of the labelling of unclassifiable ILD in clinical practice and research and whether the term 'unclassified' should be used, implying a less definitive diagnosis.

A 34-Year-Old Man With a Chylothorax and Bony Pain.

CASE PRESENTATION: A 34-year-old man presented to a community hospital with sudden-onset pleuritic chest pain on a background of a 12-month indolent history of progressive exertional dyspnea. He denied cough, fevers, night sweats, or weight loss. He reported some low back pain and ache. He had a history of gastroesophageal reflux and was a current smoker with a 20-pack year history. There were no known occupational or environmental exposures and there was no family history of any lung disease.

'When you hear hooves, think zebras, not horses'; two challenging cases of interstitial lung disease (ILD).

Our case series describes two siblings with complex fibrosing lung diseases. The first patient was initially given a diagnosis of sarcoidosis based on imaging and exclusion of alternative diagnoses. A number of years after diagnosis, he had rapid deterioration of his disease and following surgical lung biopsy, his lung fibrosis was re-classified as chronic hypersensitivity pneumonitis (cHP) with a usual interstitial pneumonia pattern. He subsequently underwent successful lung transplantation. The second patient presented with rapidly progressing exertional dyspnoea. His bloods, imaging, bronchoalveolar lavage and histology were discussed at our multidisciplinary team meeting. His histology was most in keeping with subacute on cHP with overlapping imaging features between the two siblings. He was treated accordingly but unfortunately succumbed to his illness shortly after diagnosis. These cases highlight the difficulties differentiating between the various interstitial lung disease (ILD) subtypes and the challenges in management while also increasing awareness of familial ILD.