Automated system for diagnosing pulmonary fibrosis using crackle analysis in recorded lung sounds based on iterative envelope mean fractal dimension filter.

Patients with pulmonary fibrosis (PF) often experience long waits before getting a correct diagnosis, and this delay in reaching specialized care is associated with increased mortality, regardless of the severity of the disease. Early diagnosis and timely treatment of PF can potentially extend life expectancy and maintain a better quality of life. Crackles present in the recorded lung sounds may be crucial for the early diagnosis of PF.

Automated system for diagnosing pulmonary fibrosis using crackle analysis in recorded lung sounds based on iterative envelope mean fractal dimension filter.

Patients with pulmonary fibrosis (PF) often experience long waits before getting a correct diagnosis, and this delay in reaching specialized care is associated with increased mortality, regardless of the severity of the disease. Early diagnosis and timely treatment of PF can potentially extend life expectancy and maintain a better quality of life. Crackles present in the recorded lung sounds may be crucial for the early diagnosis of PF.

Patient-centered care in pulmonary fibrosis: access, anticipate, and act.

Comprehensive care integrates individual patient needs and is highly valued for patients with pulmonary fibrosis (PF). The importance of a patient-centered care approach is rooted in the unpredictable progressiveness of the disease course in PF. The respiratory impairment associated with PF has a major impact on the quality of life for both patients and their caregivers.

Reliability of crackles in fibrotic interstitial lung disease: a prospective, longitudinal study.

Background: Although crackles on chest auscultation represent a fundamental component of the diagnostic suspect for fibrotic interstitial lung disease (ILD), their reliability has not been properly studied. We assessed the agreement among respiratory physicians on the presence and changes over time of audible crackles collected in a prospective longitudinal cohort of patients with fibrotic ILD.

Methods: Lung sounds were digitally recorded at baseline and after 12 months at eight anatomical sites.

Spatial transcriptomic validation of a biomimetic model of fibrosis enables re-evaluation of a therapeutic antibody targeting LOXL2.

Matrix stiffening by lysyl oxidase-like 2 (LOXL2)-mediated collagen cross-linking is proposed as a core feedforward mechanism that promotes fibrogenesis. Failure in clinical trials of simtuzumab (the humanized version of AB0023, a monoclonal antibody against human LOXL2) suggested that targeting LOXL2 may not have disease relevance; however, target engagement was not directly evaluated. We compare the spatial transcriptome of active human lung fibrogenesis sites with different human cell culture models to identify a disease-relevant model.

Concordance and Prognostic Relevance of Different Definitions of Systemic Sclerosis Interstitial Lung Disease Progression.

Interstitial lung disease (ILD) in systemic sclerosis (SSc) is a common complication that has a varied progression rate and prognosis. Different progression definitions are available, including minimal clinically important worsening of FVC, EUSTAR (European Scleroderma Trials and Research Group) progression, OMERACT (Outcome Measures in Rheumatology Clinical Trials) progression, and Erice ILD working group progression. Pulmonary function and symptom changes may act as specific confounding factors when applying these definitions in SSc.

Promising advances in treatments for the management of idiopathic pulmonary fibrosis.

Introduction: Following the INPULSIS and ASCEND studies, leading to the first two approved antifibrotic therapies for patients with IPF, ongoing investigations are firmly exploring novel agents for a targeted effective and better tolerated therapy able to improve the natural history of the disease.

Areas Covered: This review aims to analyze recent advances in pharmacological research of IPF, discussing the currently available treatments and the novel drugs under investigation in phase 3 trials, with particular emphasis on BI 1015550 and inhaled treprostinil. The literature search utilized Medline and Clinicaltrials.

Pamrevlumab for Idiopathic Pulmonary Fibrosis: The ZEPHYRUS-1 Randomized Clinical Trial.

Importance: Current treatments for idiopathic pulmonary fibrosis slow the rate of lung function decline, but may be associated with adverse events that affect medication adherence. In phase 2 trials, pamrevlumab (a fully human monoclonal antibody that binds to and inhibits connective tissue growth factor activity) attenuated the progression of idiopathic pulmonary fibrosis without substantial adverse events.

Objective: To assess the efficacy and safety of pamrevlumab for patients with idiopathic pulmonary fibrosis.

Case of a 33-Year-Old Woman With Hemoptysis and Migrant Nodular Cavitary Lesions.

We describe the case of a young 33-year-old woman that was referred to our clinic for evidence of migrant cavitary nodules at CT scan, dyspnea, and blood sputum. Her physical examination showed translucent and thin skin, evident venous vascular pattern, vermilion of the lip thin, micrognathia, thin nose, and occasional Raynaud phenomenon. We prescribed another CT scan that showed multiple pulmonary nodules in both lungs, some of which had evidence of cavitation.

Zinpentraxin Alfa for Idiopathic Pulmonary Fibrosis: The Randomized Phase III STARSCAPE Trial.

A phase II trial reported clinical benefit over 28 weeks in patients with idiopathic pulmonary fibrosis (IPF) who received zinpentraxin alfa. To investigate the efficacy and safety of zinpentraxin alfa in patients with IPF in a phase III trial. This 52-week phase III, double-blind, placebo-controlled, pivotal trial was conducted at 275 sites in 29 countries.

Experimental autotaxin inhibitors for the treatment of idiopathic pulmonary fibrosis.

Introduction: Idiopathic Pulmonary Fibrosis (IPF) is a progressive, irreversible, and fatal lung disease with unmet medical needs. Autotaxin (ATX) is an extracellular enzyme involved in the generation of lysophosphatidic acid (LPA). Preclinical and clinical data have suggested the ATX-LPAR signaling axis plays an important role in the pathogenesis and the progression of IPF.

Meaningful Endpoints for Idiopathic Pulmonary Fibrosis (IPF) Clinical Trials: Emphasis on 'Feels, Functions, Survives'. Report of a Collaborative Discussion in a Symposium with Direct Engagement from Representatives of Patients, Investigators, the National Institutes of Health, a Patient Advocacy Organization, and a Regulatory Agency.

Idiopathic pulmonary fibrosis (IPF) carries significant mortality and unpredictable progression, with limited therapeutic options. Designing trials with patient-meaningful endpoints, enhancing the reliability and interpretability of results, and streamlining the regulatory approval process are of critical importance to advancing clinical care in IPF. A landmark in-person symposium in June 2023 assembled 43 participants from the US and internationally, including patients with IPF, investigators, and regulatory representatives, to discuss the immediate future of IPF clinical trial endpoints.

An update on emerging drugs for the treatment of idiopathic pulmonary fibrosis: a look towards 2023 and beyond.

Introduction: Currently approved drug treatments for idiopathic pulmonary fibrosis (IPF), pirfenidone and nintedanib, have been shown to slow lung function decline and improve clinical outcomes. Since significant advances in the understanding of pathogenetic mechanisms in IPF, novel potential agents are being tested to identify new targeted and better tolerated therapeutic strategies.

Areas Covered: This review describes the evidence from IPF phase II and III clinical trials that have been completed or are ongoing in recent years.

Walking the path of treatable traits in interstitial lung diseases.

Interstitial lung diseases (ILDs) are complex and heterogeneous diseases. The use of traditional diagnostic classification in ILD can lead to suboptimal management, which is worsened by not considering the molecular pathways, biological complexity, and disease phenotypes. The identification of specific "treatable traits" in ILDs, which are clinically relevant and modifiable disease characteristics, may improve patient's outcomes.

An up-to-date review of approved and emerging antibody therapies for idiopathic pulmonary fibrosis.

Introduction: The use of pirfenidone and nintedanib in treating Idiopathic Pulmonary Fibrosis (IPF) has shown significant slowing down of the progressive functional decline in these patients. In recent times, antibody-based therapies with precise molecular targets have also been explored as alternative treatments to IPF.

Areas Covered: This review aims to summarize the available updates regarding monoclonal antibodies that have been tested in IPF.

The Impact of Telemedicine during Severe Acute Respiratory Syndrome Coronavirus 2 Pandemic and Future Perspectives: A Systematic Review.

Introduction: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) spread worldwide since December 2019, causing the COVID-19 pandemic. Several measures have taken place in many countries to avoid further spread of the virus and to manage infected people according to disease severity. Notably, telemedicine (TM) was successfully used to manage less severe patients.