Breathe最新文献

Nasal nitric oxide (nNO) measurement is important in the primary ciliary dyskinesia (PCD) diagnostic pathway because levels are consistently very low in most patients. Machine type, environmental factors, respiratory manoeuvres and report interpretation are fundamental considerations when performing nNO testing. A European Respiratory Society Task Force recently published standards for testing which we summarise and discuss in this article. There are two main types of nNO machines: chemiluminescence and electrochemical analysers. Chemiluminescence analysers are highly accurate, reliable, real-time and have been validated in multicentre studies but are less portable and more expensive to purchase and maintain in comparison to electrochemical devices. Several factors may influence nNO levels and need to be addressed during patient preparation for testing. Factors including acute viral infections and nose bleeds may contribute to falsely low nNO levels, whereas high ambient NO levels may falsely increase nNO. Tidal breathing, breath-hold and exhalation against resistance are the three main respiratory manoeuvres used in nNO sampling and require a minimal, modest and high level of patient cooperation respectively. Finally, standardised reporting of nNO testing and the correct interpretation helps clinicians to formulate an appropriate clinical plan towards an accurate PCD diagnosis.

Idiopathic pulmonary fibrosis (IPF) and progressive pulmonary fibrosis (PPF) are characterised by an irreversible progression of pulmonary fibrosis and functional lung decline. Current antifibrotic therapies (nintedanib and pirfenidone for IPF and nintedanib for PPF) can reduce disease progression but not halt or reverse it. PPF and IPF share common pathophysiological pathways that need to be further elucidated for the development of novel therapeutic strategies. The educational aim of this review is to explain the pathogenic pathways that have led to the discovery of new therapeutic agents and their favourable implementation in phase 2 and 3 studies. This includes phosphodiesterase 4 inhibitors, αvβ6 and αvβ1 integrin inhibitors, lymphosphatidic acid antagonists, inhaled treprostinil, hedgehog inhibitors, tyrosine kinase inhibitors and angiotensin type 2 receptor agonists. The aim is also to better understand current therapeutic challenges and future perspectives, including cellular therapies, exosomes and their cargoes, as well as the integration of transcriptomics and proteomics, plus gene therapy.

Sarcoidosis-associated pulmonary hypertension can manifest with a broad range of phenotypes. There may be a role for genetic testing in specific cases of severe disease when the relevance of comorbid sarcoidosis is unclear. https://bit.ly/42kscnz.

Connective tissue disease (CTD)-associated interstitial lung disease (ILD) is a complex condition arising in various autoimmune disorders, such as systemic sclerosis, Sjögren disease, systemic lupus erythematosus and idiopathic inflammatory myopathies. The broader term of systemic autoimmune rheumatic diseases (SARDs) and SARD-ILD are increasingly adopted in various guidelines to allow inclusion of other rheumatic diseases such as rheumatoid arthritis. SARD-ILD significantly impacts morbidity and mortality, with disease manifestations ranging from mild to severe and life-threatening. Epidemiological data show varying ILD prevalence rates amongst SARDs, with fibrosis being a key pathological component secondary to immune-mediated inflammation and tissue remodelling. SARD-ILD presents diverse histological patterns, primarily nonspecific interstitial pneumonia and usual interstitial pneumonia, each informing prognosis and guiding therapeutic strategies. Diagnosis relies on a comprehensive evaluation of clinical, serological, radiological and histological data, involving a multidisciplinary team. Immunosuppressive therapy is the cornerstone of treatment, with concurrent use of anti-fibrotic agents in specific progressive cases. Disease management is stratified by severity, with distinct guidelines for stable, progressive and rapidly progressive ILD. The prognosis varies across SARD-ILD types, influenced by specific markers, imaging features, and response to therapy. In severe cases, lung transplantation may be considered. Early recognition remains critical in optimising outcomes for SARD-ILD patients.

Pulmonary alveolar proteinosis (PAP) is a rare lung disease caused by accumulation of surfactant in the alveoli, leading to debilitating respiratory symptoms and impaired gas exchange. The recent European Respiratory Society guidelines provide evidence-based recommendations for its diagnosis and management. Autoimmune PAP (aPAP) is the most common form, driven by granulocyte-macrophage colony-stimulating factor (GM-CSF) autoantibodies. Recommended diagnostic tools include bronchoalveolar lavage and quantitative GM-CSF antibody testing. Whole lung lavage and inhaled GM-CSF are first-line treatments for symptomatic or progressive aPAP. Rituximab, plasmapheresis, and lung transplantation are options for refractory disease. Referral to expert centres is advised for diagnostic and therapeutic guidance. This case-based summary for clinicians highlights the best clinical approach to patients with suspicion or confirmation of PAP.

Interstitial lung diseases (ILDs) are now the most common indication for lung transplant internationally. Given that many lung transplant candidates with idiopathic pulmonary fibrosis are older, referral to a pulmonary rehabilitation programme is important to help mitigate the adverse outcomes associated with frailty. Despite this increase many patients with ILD who would potentially benefit from lung transplant are either not referred or referred too late. Particularly relevant in ILD which may have prominent extra-pulmonary manifestations is a multidisciplinary assessment of comorbidities which may impact on post lung transplant outcomes. Particular challenges in lung transplant for ILD are increasing age, comorbidities, donor lung sizing and the risk-benefit balance of single versus bilateral lung transplant. Evidence is continuing to evolve for lung transplant in rarer ILDs, including surfactant protein associated ILD and TERT mutations. Unfortunately, the number of potential lung transplant recipients exceeds available donor organs and some patients will die without transplant. Palliative care is an important aspect of managing patients on an active lung transplant list to help optimise physical and psychological symptoms associated with uncertainty on an active lung transplant list.

Clive and Sue's story highlights the importance of support, education and research in managing RA-ILD. Their journey shows how community and awareness can improve quality of life and help create better treatment options for future patients. https://bit.ly/4jqdsJN.

Childhood interstitial lung diseases (chILDs) are rare and heterogeneous disorders associated with significant morbidity and mortality. The clinical presentation of chILD typically includes chronic or recurrent respiratory signs and symptoms with diffuse radiographic abnormalities on chest imaging. Diagnosis requires a structured, multi-step approach. Treatment options are limited, with disease-specific therapies available only in selected cases and management relying primarily on supportive care. Awareness of chILDs has been steadily increasing. New diagnoses, advanced diagnostic tests, and novel treatments are emerging each year, highlighting the importance of collaborative, multidisciplinary teams in providing comprehensive care for children and families affected by these complex conditions. On behalf of the European Respiratory Society Clinical Research Collaboration for chILD (ERS CRC chILD-EU), this review provides an updated overview of the diagnostic approach and management strategies for chILDs.

The healthcare sector is one of the primary emitters of greenhouse gases in the public sector; this viewpoint's distillation of expert opinions emphasises what more sustainable asthma care could look like and presents possible solutions to achieving it https://bit.ly/41fQ364.

This review summarises some of the key features of interstitial lung diseases (ILDs) from a translational science point of view and brings insights into potential therapeutic options. Genetic predisposition and environmental factors like smoking, pollution and infections significantly impact the onset, progression and treatment response in ILDs, highlighting the need for personalised management. Fibroblasts are central to ILD pathology, influencing the tissue microenvironment, immune cell interactions and extracellular matrix (ECM) production, making them critical therapeutic targets. Monocyte-derived M2 macrophages drive fibrosis in idiopathic pulmonary fibrosis by secreting cytokines and remodelling the ECM. Understanding macrophage subtypes and their dynamics offers new therapeutic possibilities. Chronic type 2 immunity contributes to fibrosis, emphasising the need to enhance protective markers in order to even out the balance shift of pathological immune responses in ILD treatments. Serum biomarkers like Krebs von den Lungen-6 (KL-6), surfactant protein (SFTP) D, matrix metalloproteinase-7 (MMP-7), and C-C motif chemokine ligand (CCL)-18 are valuable for diagnosing and predicting ILD progression, although more research is needed for clinical application. Animal models, especially bleomycin-based models, offer insights into ILD pathology, but challenges like lung hyperinflation highlight the need for careful model selection and translational research to bridge preclinical and clinical findings.