Association between the HAL score and the development of progressive pulmonary fibrosis in idiopathic interstitial pneumonia: A prospective observational study.

Background: 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.

Case of familial interstitial lung disease attributed to ATP-binding cassette transporter 3 gene mutation in identical twins.

Mutations 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.

Variability of radiological and clinical features in cases with usual interstitial pneumonia without honeycombing.

Background: 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.

Radiological and histopathological features and treatment response by subtypes of interstitial pneumonia with autoimmune features: A prospective, multicentre cohort study.

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.

Association between increased peripheral blood CD86-positive plasmacytoid dendritic cells and immune-related adverse events in patients with non-small cell lung cancer.

The occurrence of immune-related adverse events (irAEs) after immune checkpoint inhibitors (ICIs) is unpredictable. Profiles of peripheral blood mononuclear cells (PBMCs) represent the host immune system and have the potential to predict irAEs. We analyzed PBMC subsets using multicolor flow cytometry before and at weeks 2 and 8 after the start of ICIs in patients with non-small cell lung cancer.

Design and rationale of the Japanese Idiopathic Interstitial Pneumonias (JIPS) Registry.

Background: Numerous studies investigated patients with IPF; however, only a few examined patients with idiopathic interstitial pneumonias (IIPs).

Methods: The Japanese Idiopathic Interstitial Pneumonias (JIPS) Registry, which was initiated in December 2016, is a multicenter prospective observational study of patients newly diagnosed with IIPs from 86 facilities treating ILDs. The plan is to enroll more than 600 new patients during the 2-year enrolment period and to follow their progress for 3 years after the last case enrolment.

Prognostic significance of radiological pleuroparenchymal fibroelastosis in complex lung disease: a multicentre retrospective cohort study.

Background: complex (MAC) causes chronic respiratory infectious diseases with diverse clinical features and prognoses. Pleuroparenchymal fibroelastosis (PPFE) is a rare disease characterised by pleural fibrosis with subjacent intra-alveolar fibrosis and alveolar septal elastosis, with unique chest high-resolution CT (HRCT) features (radiological PPFE). An association between recurrent respiratory infections and PPFE formation has been hypothesised; however, the clinical significance of PPFE in MAC lung disease remains unclear.

Frequency, proportion of PF-ILD, and prognostic factors in patients with acute exacerbation of ILD related to systemic autoimmune diseases.

Background: Acute exacerbation (AE) of systemic autoimmune disease-related interstitial lung diseases (SAID-ILD) is less common than AE of idiopathic pulmonary fibrosis (IPF) and the details of AE-SAID-ILD have not been elucidated, but the prognosis is similarly devastating. This study was undertaken to determine the incidences of AE-ILD in each SAID and to elucidate the proportion of progressive fibrosing (PF)-ILD in AE-SAID-ILD.

Methods: We retrospectively analysed data for patients with SAID-ILD who were diagnosed and observed at our hospital between 1999 and 2020.

CD109 on Dendritic Cells Regulates Airway Hyperreactivity and Eosinophilic Airway Inflammation.

Asthma is a chronic airway inflammatory disease characterized by airway hyperreactivity (AHR) and eosinophilic airway inflammation. Dendritic cells (DCs) are essential for the development of asthma via presenting allergens, causing T-helper cell type 2 (Th2) skewing and eosinophil inflammation. Recent studies have revealed that CD109, a glycosylphosphatidylinositol-anchored glycoprotein, is involved in the pathogenesis of inflammatory diseases such as rheumatoid arthritis and psoriasis.

Nationwide retrospective observational study of idiopathic dendriform pulmonary ossification: clinical features with a progressive phenotype.

Background: Diffuse pulmonary ossification is a specific lung condition that is accompanied by underlying diseases. However, idiopathic dendriform pulmonary ossification (IDPO) is extremely rare, and the clinical features remain unclear. In this study, we aimed to report the clinical characteristics of IDPO.

Associations of serum long-chain fatty acids with multiple organ involvement in patients with sarcoidosis.

Background: Fatty acids have diverse immunomodulatory functions and the potential to be associated with inflammatory responses in sarcoidosis.

Methods: The serum levels of multiple long-chain fatty acids (LCFAs) were compared between 63 patients with sarcoidosis and 38 healthy controls. The associations of LCFAs with clinical outcomes of sarcoidosis were also evaluated.

Idiopathic pleuroparenchymal fibroelastosis: three-dimensional computed tomography assessment of upper-lobe lung volume.

Background: Idiopathic pleuroparenchymal fibroelastosis (iPPFE) is a rare interstitial lung disease characterised by predominant upper-lobe fibrosis involving the pleura and subpleural lung parenchyma. Despite its poor prognosis, there is no consensus on prognostic determinants of iPPFE to date. Because volume loss in the upper lobe is a distinct feature of iPPFE, we hypothesised that the lung volume of the bilateral upper lobes (upper-lobe volume) accurately indicates disease severity and mortality risk in iPPFE patients.

Chemotherapy for patients with advanced lung cancer with interstitial lung disease: a prospective observational study.

Introduction: Although recent advances in chemotherapy for lung cancer are remarkable, most clinical trials have excluded patients with interstitial lung disease (ILD) due to the concern of developing acute exacerbation (AE) of ILD. Hence, accumulating original evidence of cancer treatment for this population is important.

Methods: Between 2016 and 2020, a prospective observational study was conducted across 11 Japanese hospitals.

Standardised 3D-CT lung volumes for patients with idiopathic pulmonary fibrosis.

Background: The assessment of lung physiology via pulmonary function tests (PFTs) is essential for patients with idiopathic pulmonary fibrosis (IPF). However, PFTs require active participation, which can be challenging for patients with severe respiratory failure, such as during moments of acute exacerbation (AE) of IPF. Recent advances have enabled the re-construction of 3-dimensional computed-tomography (3D-CT) images.

Effects of long-acting muscarinic antagonists on promoting ciliary function in airway epithelium.

Background: Mucociliary clearance (MCC) is an essential defense mechanism in airway epithelia for removing pathogens from the respiratory tract. Impaired ciliary functions and MCC have been demonstrated in asthma and chronic obstructive pulmonary disease (COPD). Long-acting muscarinic antagonists (LAMAs) are a major class of inhaled bronchodilators, which are used for treating asthma and COPD; however, the effects of LAMAs on ciliary function remain unclear.

Impact of end-of-life respiratory modalities on quality of dying and death and symptom relief in patients with interstitial lung disease: a multicenter descriptive cross-sectional study.

Background: Respiratory modalities applied at the end of life may affect the burden of distressing symptoms and quality of dying and death (QODD) among patients with end-stage interstitial lung disease (ILD); however, there have been few studies into respiratory modalities applied to these patients near death. We hypothesized that high-flow nasal cannula (HFNC) might contribute to improved QODD and symptom relief in patients with end-stage ILD.

Objectives: This multicenter study examined the proportion of end-of-life respiratory modalities in a hospital setting and explored its impact on QODD and symptom relief among patients dying with ILD.

Impact of antifibrotic therapy on lung cancer development in idiopathic pulmonary fibrosis.

Patients with idiopathic pulmonary fibrosis (IPF) are at a high risk of lung cancer (LC). Antifibrotic therapy slows disease progression and possibly prolongs survival. However, whether antifibrotic therapy affects LC development in patients with IPF remains unknown.

Trimethoprim-sulfamethoxazole induced eosinophilic pneumonia: A case report.

We report herein a case of trimethoprim-sulfamethoxazole (TMP-SMX) induced eosinophilic pneumonia in a 27-year-old woman with radiological features of bilateral nonsegmental airspace consolidation resembling cryptogenic organizing pneumonia at the peripheral lung fields. Organizing pneumonia with eosinophil infiltration in the lung specimens and marked eosinophilia in the peripheral blood and bronchoalveolar lavage fluid were observed. Discontinuation of TMP-SMX improved eosinophilia and radiological abnormality, which confirmed the association between the use of TMP-SMX and onset of eosinophilic pneumonia.

Acute exacerbation of rheumatoid arthritis-associated interstitial lung disease: mortality and its prediction model.

Background: Patients with rheumatoid arthritis-associated interstitial lung disease (RA-ILD), like those with idiopathic pulmonary fibrosis (IPF), might develop an unexpected acute exacerbation (AE)-a rapidly progressing and deadly respiratory decline. Although AE incidence and risk factors in RA-ILD patients are known, their post-AE clinical course remains unknown owing to the rarity of AE-RA-ILD. This multicentre retrospective study evaluated post-AE mortality and prognostic variables in AE-RA-ILD patients and created a mortality prediction model for AE-RA-ILD.

Multiple organ infarction caused by aortic thrombus in a lung cancer patient with the mutation.

A 72-year-old male patient with advanced lung adenocarcinoma harboring a mutation had received treatment with a BRAF inhibitor and a MEK inhibitor. Treatment was ceased after 40 days because of disease progression. Twenty-four days after treatment cessation, the man was referred to our hospital with worsening abdominal and back pain over 2 weeks.

Involvement of autophagy in exacerbation of eosinophilic airway inflammation in a murine model of obese asthma.

Obesity is a common comorbidity in patients with asthma, and obese asthma patients present the most refractory phenotype among patients with severe asthma. Similar to the observations in non-obese asthma patients, clinical studies have revealed heterogeneity in obese asthma patients, including the occurrences of T helper (Th)2-high and Th2-low phenotypes. However, the mechanisms underlying obesity-related asthma are not completely understood.

-Mutated Lung Adenocarcinoma Successfully Treated With Osimertinib After Spontaneous Transformation to SCLC and Adenocarcinoma With Neuroendocrine Differentiation: Case Report.

Lineage transformation from lung adenocarcinoma (LUAD) to SCLC is associated with resistance to EGFR tyrosine kinase inhibitors. In addition to loss of p53 and RB, transformed SCLCs are usually not dependent on EGFR signaling, which renders the tumors unresponsive to EGFR tyrosine kinase inhibitors. Here, we present a case of spontaneous transformation from -mutant LUAD with loss of p53 and RB to EGFR expression-positive SCLC and neuroendocrine-differentiated LUAD, which was successfully treated with osimertinib.