One-Year Change in Quantitative Computed Tomography Is Associated with Meaningful Outcomes in Fibrotic Lung Disease.

Rationale: Whether change in fibrosis on high-resolution CT (HRCT) is associated with near- and longer-term outcomes in patients with fibrotic interstitial lung disease (fILD) remains unclear.

Objectives: We evaluated the association between 1-year change in quantitative fibrosis scores (DTA) and subsequent forced vital capacity (FVC) and survival in patients with fILD.

Methods: The primary cohort included fILD patients evaluated from 2017-2020 with baseline and 1-year follow-up HRCT and FVC.

Use of Spirometry in Pulmonary Function Evaluation.

Spirometry is a safe, quick, and readily available test that can be used in the office, pulmonary physiology laboratory, and at home to evaluate respiratory symptoms. Its primary measurements are the forced vital capacity (FVC), forced expiratory volume in 1 second (FEV), and their ratio (FEV/FVC). Spirometry measurements can be used for the prognosis in certain disease states, in evaluating progression as well as therapeutic response, and in monitoring at risk patients.

Emerging Concepts in Therapeutic Interventions for Idiopathic Pulmonary Fibrosis.

Idiopathic pulmonary fibrosis (IPF) is a rare but devastating diagnosis for patients with only two approved drug therapies. Extensive preclinical studies have identified and characterized novel pathways driving IPF pathogenesis, and researchers have identified several new promising therapeutic targets to help treat IPF. However, translating these preclinical models into viable treatment modalities has proven challenging.

Development and validation of a clinical, CT, genomic classifier and BAL scoring system for diagnosing idiopathic pulmonary fibrosis.

Background: The utility of incorporating a usual interstitial pneumonia (UIP) genomic classifier (GC) and bronchoalveolar lavage (BAL) cell count analysis alongside traditional clinical-imaging assessment in aiding in the multidisciplinary diagnosis of idiopathic pulmonary fibrosis (IPF) in patients with a non-definite high-resolution computed tomography (HRCT) UIP pattern is uncertain.

Methods: We reviewed consecutive adult patients presenting with fibrotic interstitial lung disease (fILD) and a non-definite HRCT UIP pattern who underwent BAL and GC. The initial fILD diagnoses were re-evaluated after bronchoscopy and a final multidisciplinary consensus diagnosis was provided.

Diagnostic Testing in Exercise-Induced Bronchoconstriction.

Exercise-induced bronchoconstriction (EIB), a reversible airflow obstruction triggered by exercise, should be considered in patients presenting with symptoms of dyspnea, cough, wheeze, and chest tightness during or after vigorous exercise. Over the past several years, various diagnostic modalities have been developed and evaluated for the diagnosis of EIB, giving the clinician multiple options for diagnostic testing. Here, the authors present a review of the various testing options that can be used in the diagnosis of EIB, with a discussion of testing protocols and considerations for choosing the appropriate diagnostic test.

Pirfenidone in fibrotic hypersensitivity pneumonitis: a double-blind, randomised clinical trial of efficacy and safety.

Background: Fibrotic hypersensitivity pneumonitis (FHP) is an irreversible lung disease with high morbidity and mortality. We sought to evaluate the safety and effect of pirfenidone on disease progression in such patients.

Methods: We conducted a single-centre, randomised, double-blinded, placebo-controlled trial in adults with FHP and disease progression.

Signatures of Mitochondrial Dysfunction and Impaired Fatty Acid Metabolism in Plasma of Patients with Post-Acute Sequelae of COVID-19 (PASC).

Exercise intolerance is a major manifestation of post-acute sequelae of severe acute respiratory syndrome coronavirus infection (PASC, or "long-COVID"). Exercise intolerance in PASC is associated with higher arterial blood lactate accumulation and lower fatty acid oxidation rates during graded exercise tests to volitional exertion, suggesting altered metabolism and mitochondrial dysfunction. It remains unclear whether the profound disturbances in metabolism that have been identified in plasma from patients suffering from acute coronavirus disease 2019 (COVID-19) are also present in PASC.

Design and rationale of a randomised, double-blind trial of the efficacy and safety of pirfenidone in patients with fibrotic hypersensitivity pneumonitis.

Hypersensitivity pneumonitis (HP) is an immunologically mediated form of lung disease resulting from inhalational exposure to any of a large variety of antigens. A subgroup of patients with HP develops pulmonary fibrosis (fibrotic HP; FHP), a significant cause of morbidity and mortality. This study will evaluate the safety and efficacy of the antifibrotic pirfenidone in treating FHP.

Duration of rheumatoid arthritis and the risk of developing interstitial lung disease.

https://bit.ly/3lgjfDJ.

Idiopathic pulmonary fibrosis: the radiologist's role in making the diagnosis.

Radiologists have a critical role in the evaluation and diagnosis of suspected idiopathic pulmonary fibrosis (IPF). Accurate pattern identification on imaging is key in the multidisciplinary diagnostic process and frequently obviates the need for a surgical lung biopsy. In this review, we describe the clinical and imaging features of IPF in the context of recently revised international guidelines; contrast findings in other diseases that may inform differential diagnosis of fibrotic lung disease; and highlight common complications associated with pulmonary fibrosis.

Phagocytosis of microparticles by alveolar macrophages during acute lung injury requires MerTK.

Microparticles are a newly recognized class of mediators in the pathophysiology of lung inflammation and injury, but little is known about the factors that regulate their accumulation and clearance. The primary objective of our study was to determine whether alveolar macrophages engulf microparticles and to elucidate the mechanisms by which this occurs. Alveolar microparticles were quantified in bronchoalveolar fluid of mice with lung injury induced by LPS and hydrochloric acid.

Deletion of c-FLIP from CD11b Macrophages Prevents Development of Bleomycin-induced Lung Fibrosis.

Idiopathic pulmonary fibrosis is a progressive lung disease with complex pathophysiology and fatal prognosis. Macrophages (MΦ) contribute to the development of lung fibrosis; however, the underlying mechanisms and specific MΦ subsets involved remain unclear. During lung injury, two subsets of lung MΦ coexist: Siglec-F resident alveolar MΦ and a mixed population of CD11b MΦ that primarily mature from immigrating monocytes.

Cell Origin Dictates Programming of Resident versus Recruited Macrophages during Acute Lung Injury.

Two populations of alveolar macrophages (AMs) coexist in the inflamed lung: resident AMs that arise during embryogenesis, and recruited AMs that originate postnatally from circulating monocytes. The objective of this study was to determine whether origin or environment dictates the transcriptional, metabolic, and functional programming of these two ontologically distinct populations over the time course of acute inflammation. RNA sequencing demonstrated marked transcriptional differences between resident and recruited AMs affecting three main areas: proliferation, inflammatory signaling, and metabolism.

Selective and inducible targeting of CD11b+ mononuclear phagocytes in the murine lung with hCD68-rtTA transgenic systems.

During homeostasis two distinct macrophage (Mø) populations inhabit the lungs: tissue Mø (often called interstitial Mø) and resident alveolar Mø (resAMø). During acute lung inflammation, monocytes from the circulation migrate to areas of injury where they mature into a third Mø population: recruited Mø. Resident AMø uniquely express low levels of CD11b and high levels of CD11c.

Kinetics of the angiogenic response in lung endothelium following acute inflammatory injury with bleomycin.

Purpose/aim: Angiogenesis is a central component of normal wound healing but it has not been fully characterized in lung repair following acute inflammatory injury. The current literature lacks vital information pertaining to the extent, timing, and location of this process. This information is necessary for examining mechanisms that drive normal lung repair in resolving acute inflammatory injury.

Increased sensitivity to staphylococcal enterotoxin B following adenoviral infection.

Staphylococcal enterotoxin B induces toxic shock and is a major virulence factor of staphylococcal diseases. We examined the effects of systemic adenoviral infection on responses to staphylococcal enterotoxin B in a murine model. We found that adenoviral infection markedly increases the severity of liver injury following exposure to staphylococcal enterotoxin B without d-galactosamine sensitization.