Background: Recent advances in comprehensive gene analysis revealed the heterogeneity of mouse lung fibroblasts. However, direct comparisons between these subpopulations are limited due to challenges in isolating target subpopulations without gene-specific reporter mouse lines. In addition, the properties of lung lipofibroblasts remain unclear, particularly regarding the appropriate cell surface marker and the niche capacity for alveolar epithelial cell type 2 (AT2), an alveolar tissue stem cell.
View Article and Find Full Text PDFAberrant immune responses to viral pathogens contribute to pathogenesis, but our understanding of pathological immune responses caused by viruses within the human virome, especially at a population scale, remains limited. We analyzed whole-genome sequencing datasets of 6,321 Japanese individuals, including patients with autoimmune diseases (psoriasis vulgaris, rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), pulmonary alveolar proteinosis (PAP) or multiple sclerosis) and coronavirus disease 2019 (COVID-19), or healthy controls. We systematically quantified two constituents of the blood DNA virome, endogenous HHV-6 (eHHV-6) and anellovirus.
View Article and Find Full Text PDFBackground And Objective: The association between interstitial lung abnormalities (ILA) and various conditions and diseases, including drug-related pneumonitis (DRP), has been reported. However, the association of the presence of ILA with developing DRP in patients undergoing cytotoxic agent-based chemotherapy, one of the standard treatments for malignancies, remains unclear. This warrants urgent investigation.
View Article and Find Full Text PDFBackground: Progressive pulmonary fibrosis (PPF) is a critical concern in interstitial lung disease (ILD) management. The HAL score, which incorporates honeycombing (H), age >75 years (A), and serum lactate dehydrogenase >222 U/L (L), can predict acute exacerbations in patients with idiopathic interstitial pneumonia (IIP). This study aims to evaluate the predictive utility of the HAL score for PPF development.
View Article and Find Full Text PDFBackground: Despite the development of biologics for severe asthma, individuals with uncontrolled status persist, posing a significant social problem. This multicenter prospective study aimed to identify factors associated with the uncontrolled status of patients with severe asthma in the biologic era assessed using the Asthma Control Questionnaire (ACQ).
Methods: Subjects with severe asthma diagnosed by respiratory specialists were enrolled from 11 hospitals.
Rationale: Accurate prognostic awareness (PA) and knowledge of the disease are critical for decision-making regarding treatment options, advance care planning, and end-of-life care. However, they have not been investigated in patients with interstitial lung disease (ILD).
Objectives: To determine the prevalence of patients with ILD who have accurate PA and/or knowledge of acute exacerbation.
Background: Patients with idiopathic pulmonary fibrosis (IPF) may experience insomnia and use hypnotics. However, the effect of the use of hypnotics on their clinical course remains unclear.
Research Question: Is the use of hypnotics associated with an increased risk of mortality in patients with IPF?
Study Design And Participants: This study included 99 patients with IPF from the Hamamatsu hospital-based cohort and 123 patients with IPF from the Seirei hospital-based cohort, as well as 30,218 patients with IPF from the National Database of Health Insurance Claims and Specific Health Checkups of Japan (the NDB cohort).
Background: Constipation is associated with the prognosis of several chronic diseases. However, the effect of constipation on the prognosis of idiopathic interstitial pneumonias (IIPs) remains unclear. This study aimed to investigate the association between constipation and the prognosis of patients with IIPs.
View Article and Find Full Text PDFMutations in ABCA3 can result in surfactant deficiency, leading to respiratory distress syndrome in term neonates, and interstitial lung disease (ILD) in children. Here, we report an extremely rare case of ILD in an identical twin with novel ABCA3 germline mutations. Interestingly, they showed mostly similar, but slightly different, clinical features.
View Article and Find Full Text PDFBackground: Hypersensitivity pneumonitis (HP) is a complex and heterogenous interstitial lung disease (ILD) that occurs in susceptible individuals due to certain inhaled antigens. Fibrotic-HP is a major underlying disease of progressive pulmonary fibrosis. Therefore, in addition to the radiological features of HP, quantitatively measuring fibrosis is important to evaluate disease severity and progression.
View Article and Find Full Text PDFBackground: Usual interstitial pneumonia (UIP) cases without honeycombing (possible UIP) included various CT features and was often difficult to diagnose.
Purpose: This study aimed to classify the cases with possible UIP on CT features using cluster analysis and evaluate the features of subsets of participants and the correlation of prognosis.
Materials And Methods: The study included 85 patients with possible UIP in the 2011 idiopathic pulmonary fibrosis (IPF) guideline with radiological diagnosis.
Am J Physiol Lung Cell Mol Physiol
September 2024
Obesity is a risk factor for increased morbidity and mortality in viral respiratory infection. Mucociliary clearance (MCC) in the airway is the primary host defense against viral infections. However, the impact of obesity on MCC is unclear, prompting this study.
View Article and Find Full Text PDFBackground And Objective: Pooled analyses of previous randomized controlled trials reported that antifibrotics improved survival in patients with idiopathic pulmonary fibrosis (IPF), but the results were only based on short-term outcome data from selected patients who met strict criteria. Observational studies/meta-analyses also suggested that antifibrotics improve survival, but these studies failed to control for immortal time bias that considerably exaggerates drug effects. Therefore, whether antifibrotics truly improve long-term survival in patients with IPF in the real world remains undetermined and requires external validity.
View Article and Find Full Text PDFPurpose: We evaluated the efficacy and safety of antiemetic therapy with olanzapine, a neurokinin-1 receptor antagonist (RA), a 5-hydroxytryptamine-3 (5-HT) RA, and dexamethasone for preventing chemotherapy-induced nausea and vomiting in patients receiving carboplatin-containing chemotherapy.
Patients And Methods: Chemotherapy-naïve patients scheduled to receive carboplatin (AUC ≥5) were randomly assigned to receive either olanzapine 5 mg once daily (olanzapine group) or placebo (placebo group) in combination with aprepitant, a 5-HT RA, and dexamethasone. The primary end point was the complete response (CR; no vomiting and no rescue therapy) rate in the overall phase (0-120 hours).
Purpose: Understanding the function of BRAF mutants is crucial for determining the best treatment strategy. This study aimed to characterize a rare variant, , which was identified in a patient with lung adenocarcinoma (LUAD) by comprehensive genomic profiling.
Materials And Methods: We report a case of LUAD with treated with dabrafenib and trametinib.
Background: Patients with idiopathic interstitial pneumonia (IIP) have a favourable prognosis when they have interstitial pneumonia with autoimmune features (IPAF). However, precise IPAF-related findings from high-resolution computed tomography (HRCT) and lung histopathological specimens and the treatment response have not been fully determined. Therefore, this study was conducted to evaluate the relationship between findings on HRCT or lung histopathological specimens and the progression of interstitial pneumonia in patients with IPAF.
View Article and Find Full Text PDFBackground: COVID-19 patients with preexisting interstitial lung disease (ILD) were reported to have a high mortality rate; however, this was based on data from the early stages of the pandemic. It is uncertain how their mortality rates have changed with the emergence of new variants of concern as well as the development of COVID-19 vaccines and treatments. It is also unclear whether having ILD still poses a risk factor for mortality.
View Article and Find Full Text PDFPulmonary fibrosis is a fatal condition characterized by fibroblast and myofibroblast proliferation and collagen deposition. TGF-β plays a pivotal role in the development of pulmonary fibrosis. Therefore, modulation of TGF-β signaling is a promising therapeutic strategy for treating pulmonary fibrosis.
View Article and Find Full Text PDFBackground: Antifibrotic therapy is widely used for patients with progressive fibrotic interstitial lung disease (ILD), regardless of etiology. There is an urgent need for a simple, inexpensive, and repeatable biomarker to evaluate disease severity and mortality risk.
Methods: This retrospective multicohort study assessed the neutrophil-lymphocyte ratios (NLRs) of 416 patients with ILD who received antifibrotic therapy (Hamamatsu cohort, n = 217; Seirei cohort, n = 199).
Background: Drug-induced interstitial lung disease (DIILD) is a serious adverse event potentially induced by any antineoplastic agent. Whether cancer patients are predisposed to a higher risk of DIILD after receiving immune checkpoint inhibitors (ICIs) is unknown.
Methods: This study retrospectively assessed the cumulative incidence of DIILD in consecutive cancer patients who received post-ICI antineoplastic treatment within 6 months from the final dose of ICIs.