Interstitial Lung Disease Associated with Anti-Ku Antibodies: A Case Series of 19 Patients.

Antibodies against Ku have been described in patients with various connective tissue diseases. The objective of this study was to describe the clinical, functional, and imaging characteristics of interstitial lung disease in patients with anti-Ku antibodies. : This single-center, retrospective observational study was conducted at a tertiary referral institution.

Chronic rhinosinusitis with nasal polyps: Key considerations in the multidisciplinary team approach.

Background: Chronic rhinosinusitis with nasal polyps (CRSwNP) is a recurrent inflammatory disease associated with several comorbidities and a significant disease burden for patients. Treatments include corticosteroids and sinonasal surgery, but these can be associated with the risk of adverse events and nasal polyp recurrence. Biologic treatments such as mepolizumab can be used as an add-on treatment and are effective at reducing surgery and corticosteroid use.

Continued Treatment with Nintedanib in Patients with Progressive Pulmonary Fibrosis: Data from INBUILD-ON.

Purpose: In the INBUILD trial in patients with progressive pulmonary fibrosis (PPF), nintedanib slowed the decline in forced vital capacity (FVC) versus placebo, with a safety profile characterised mainly by gastrointestinal events. INBUILD-ON, the open-label extension of INBUILD, assessed the safety of nintedanib during longer-term treatment. Data on FVC were collected.

Identification of progressive pulmonary fibrosis: consensus findings from a modified Delphi study.

Background: We sought consensus among practising respiratory physicians on the prediction, identification and monitoring of progression in patients with fibrosing interstitial lung disease (ILD) using a modified Delphi process.

Methods: Following a literature review, statements on the prediction, identification and monitoring of progression of ILD were developed by a panel of physicians with specialist expertise. Practising respiratory physicians were sent a survey asking them to indicate their level of agreement with these statements on a binary scale or 7-point Likert scale (- 3 to 3), or to select answers from a list.

Vasodilator drugs and heart-related outcomes in systemic sclerosis: an exploratory analysis.

Background And Aims: Systemic sclerosis (SSc) is an autoimmune connective disease characterised by excessive extracellular matrix deposition and widespread skin and internal organ fibrosis including various cardiac manifestations. Heart involvement is one of the leading causes of death among patients with SSc. In this study, we aimed to assess the effect of various vasodilator treatments.

Fibroblast-derived extracellular vesicles contain SFRP1 and mediate pulmonary fibrosis.

Idiopathic pulmonary fibrosis (IPF) is a lethal chronic lung disease characterized by aberrant intercellular communication, extracellular matrix deposition, and destruction of functional lung tissue. While extracellular vesicles (EVs) accumulate in the IPF lung, their cargo and biological effects remain unclear. We interrogated the proteome of EV and non-EV fractions during pulmonary fibrosis and characterized their contribution to fibrosis.

Teatime: epigallocatechin gallate targets fibroblast-epithelial cell crosstalk to combat lung fibrosis.

Epigallocatechin gallate (EGCG) is a polyphenol plant metabolite abundant in tea that has demonstrated antifibrotic properties in the lung. In this issue of the JCI, Cohen, Brumwell, and colleagues interrogated the mechanistic action of EGCG by investigating lung biopsies of patients with mild interstitial lung disease (ILD) who had undergone EGCG treatment. EGCG targeted the WNT inhibitor SFRP2, which was enriched in fibrotic fibroblasts and acted as a TGF-β target, with paracrine effects leading to pathologic basal metaplasia of alveolar epithelial type 2 cells.

Quantitative Imaging Methods in Combined Pulmonary Fibrosis and Emphysema.

Topic Importance: Combined pulmonary fibrosis and emphysema (CPFE) is an underdiagnosed syndrome in which individuals have variable degrees of pulmonary fibrosis and emphysema. Patients with CPFE have high morbidity, including poor exercise tolerance and increased development of comorbidities. CPFE mortality also seems to outpace that of lone emphysema and pulmonary fibrosis.

Weakly-supervised learning-based pathology detection and localization in 3D chest CT scans.

Background: Recent advancements in anomaly detection have paved the way for novel radiological reading assistance tools that support the identification of findings, aimed at saving time. The clinical adoption of such applications requires a low rate of false positives while maintaining high sensitivity.

Purpose: In light of recent interest and development in multi pathology identification, we present a novel method, based on a recent contrastive self-supervised approach, for multiple chest-related abnormality identification including low lung density area ("LLDA"), consolidation ("CONS"), nodules ("NOD") and interstitial pattern ("IP").

Muscle involvement in systemic sclerosis: high mortality not associated with nature of histological lesions.

Objectives: The aim of this study was to determine the association between different histological patterns and prognosis in patients with SSc and histologically proven muscle involvement.

Methods: A multicentre retrospective study was conducted of a cohort of scleroderma patients who had undergone muscle biopsy. The biopsies were reviewed in a coordinated manner to classify patients based on histological findings.

CD206 macrophages are relevant non-invasive imaging biomarkers and therapeutic targets in experimental lung fibrosis.

Background: Interstitial lung diseases (ILDs) include a large number of diseases associated with progressive pulmonary fibrosis (PPF), including idiopathic pulmonary fibrosis (IPF). Despite the rarity of each of the fibrotic ILDs individually, they cumulatively affect a considerable number of patients. PPF is characterised by an excessive collagen deposition leading to functional decline.

Measurement of thoraco-abdominal synchrony using respiratory inductance plethysmography: technical aspects and a proposal to overcome its limitations.

Background: Thoraco-abdominal asynchrony (TAA) is usually assessed by respiratory inductance plethysmography. The main parameter used for its assessment is the calculation of the phase angle based on Lissajous plots. However, there are some mathematical limitations to its use.

Ultra-low dose chest CT for the diagnosis of pulmonary arteriovenous malformation in patients with hereditary hemorrhagic telangiectasia.

Purpose: The purpose of this study was to compare ultra-low dose (ULD) and standard low-dose (SLD) chest computed tomography (CT) in terms of radiation exposure, image quality and diagnostic value for diagnosing pulmonary arteriovenous malformation (AVM) in patients with hereditary hemorrhagic telangiectasia (HHT).

Materials And Methods: In this prospective board-approved study consecutive patients with HHT referred to a reference center for screening and/or follow-up chest CT examination were prospectively included from December 2020 to January 2022. Patients underwent two consecutive non-contrast chest CTs without dose modulation (i.

Genetics and Genomics of Pulmonary Fibrosis: Charting the Molecular Landscape and Shaping Precision Medicine.

Recent genetic and genomic advancements have elucidated the complex etiology of idiopathic pulmonary fibrosis (IPF) and other progressive fibrotic interstitial lung diseases (ILDs), emphasizing the contribution of heritable factors. This state-of-the-art review synthesizes evidence on significant genetic contributors to pulmonary fibrosis (PF), including rare genetic variants and common SNPs. The promoter variant is unusual, a common SNP that markedly elevates the risk of early and established PF.

Towards the adoption of quantitative computed tomography in the management of interstitial lung disease.

The shortcomings of qualitative visual assessment have led to the development of computer-based tools to characterise and quantify disease on high-resolution computed tomography (HRCT) in patients with interstitial lung diseases (ILDs). Quantitative CT (QCT) software enables quantification of patterns on HRCT with results that are objective, reproducible, sensitive to change and predictive of disease progression. Applications developed to provide a diagnosis or pattern classification are mainly based on artificial intelligence.

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.

Pulmonary function test results are correlated with 6-minute walk distance, distance-saturation product, and 6-minute walk work in patients with lymphangioleiomyomatosis.

Background And Objective: Lymphangioleiomyomatosis (LAM) is a rare multicystic lung disease. Although a correlation between pulmonary function test (PFT) results and exercise capacity appears probable, it has not yet been demonstrated. The aim of this study was to assess whether PFT results correlate with 6-minute walk test (6MWT) results in patients with LAM.