記述言語：英語   掲載種別：研究論文（学術雑誌）  

Human metapneumovirus (hMPV), which can cause severe lower respiratory tract disease and, rarely, extrapulmonary complications, was identified in a 64-year-old immunocompetent man who presented with acute hypoxemia, bronchoscopic evidence of diffuse alveolar hemorrhage (DAH), and clinically diagnosed acute myocarditis. Notably, hMPV was detected by multiplex PCR on a bronchoalveolar lavage (BAL) sample despite an initial negative upper airway evaluation. The patient's condition was severe and required invasive mechanical ventilation and inotropic support. After the viral etiology was identified, empiric steroids were discontinued, the patient recovered, and his left ventricular ejection fraction improved from ~9% to 40% at discharge. This case underscores the fact that during the hMPV season, acute hypoxemia with diffuse opacities should prompt consideration of infectious DAH and that lower-respiratory multiplex PCR (from BAL or deep tracheal aspirate) can identify pathogens when upper airway tests are negative. It also highlights that, in unexplained DAH, empirical immunosuppression should be used cautiously to perform a full infectious evaluation. Furthermore, when myocarditis is suspected, timely hemodynamic assessment and consideration of mechanical circulatory support may influence the outcomes.

DOI： 10.2169/internalmedicine.6423-25

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a progressive disease often complicated by pulmonary hypertension (PH), leading to a worse prognosis. In this study, we use the term “secondary PH” to refer to PH that develops as a complication of IPF (i.e., IPF-PH), and “the ERPE (Exercise-Responsive PAP Elevation) group, defined as patients without resting PH but with potential PAP elevation during exertion, indicating early pulmonary vascular stress.” This study aimed to evaluate the impact of pulmonary vascular resistance (PVR) on the clinical course, activities of daily living (ADL), and exercise tolerance in IPF patients. Additionally, we sought to determine whether PVR influences prognosis within a short-term period of just two years. METHODS: This exploratory single-center study was conducted as an interim analysis of a prospective trial targeting patients with IPF complicated by secondary PH or ERPE. The analysis included 49 IPF patients who received conventional IPF management without pulmonary arterial hypertension (PAH)-specific therapies and completed a two-year follow-up. Pulmonary circulation parameters, including PVR and pulmonary artery pressure (PAP), were assessed via right heart catheterization. Lung function, ADL, and exercise tolerance were also evaluated. Statistical analyses were performed to assess the association between PVR and prognosis. RESULTS: Higher PVR was significantly associated with poorer prognosis and worsening restrictive lung impairment. PVR correlated with modified Medical Research Council (mMRC) scores (Spearman’s ρ = 0.47, p = 0.0007) and 6-minute walk distance (Spearman’s ρ = -0.41, p = 0.0042). Proportional hazard analysis identified PVR as a significant predictor of overall survival (hazard ratio [HR]: 1.28, 95% confidence interval [CI]: 1.0055–1.53, p = 0.013). PVR also strongly correlated with maximum exercise tolerance (Spearman’s ρ = -0.60, p < 0.0001). Importantly, even within the short two-year follow-up, elevated PVR significantly impacted both home-stay survival (HR: 1.20, 95% CI: 1.0072–1.41, p = 0.032) and overall survival, underscoring its predictive value over a short time frame. CONCLUSIONS: PVR is associated with prognosis, ADL, and exercise tolerance in IPF patients. Importantly, even within a short two-year period, PVR was significantly linked to clinical outcomes. While causality cannot be established, our findings suggest that PVR may serve as a potentially useful early prognostic marker. Regular PVR monitoring could provide valuable insights into disease progression and may support earlier therapeutic interventions to improve outcomes. TRIAL REGISTRATION: This study is registered in the UMIN Clinical Trials Registry (UMIN000055468). Trial registration at 10/09/2024. Retrospectively registered. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1186/s12890-025-03837-0.

DOI： 10.1186/s12890-025-03837-0

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND AND OBJECTIVE: The optimal treatment modality for interstitial lung disease (ILD) associated with anti-aminoacyl-tRNA synthetase (ARS) antibodies remains controversial. This study aimed to compare the efficacy and safety of pulse corticosteroid therapy with that of conventional corticosteroid therapy in patients with anti-ARS ILD. METHODS: This retrospective cohort study included 62 patients with anti-ARS ILD. Patients were divided into two groups: Those who received pulse corticosteroid therapy (500-1000 mg of methylprednisolone intravenously for three days) and those who received conventional corticosteroid therapy. Primary outcomes included initial treatment response at one year and disease recurrence. Secondary outcomes were alterations in pulmonary function tests, KL-6 levels, prednisolone dose, and adverse events. RESULTS: Both the pulse corticosteroid therapy group and the conventional therapy group had similar rates of initial treatment improvement (90.3 % vs. 77.4 %, p = 0.301), with no significant differences in recurrence-free survival. Improvements in pulmonary function tests were comparable between the two groups. At 12 months, the mean daily prednisolone dose was 3.9 mg in the pulse therapy group compared with 6.0 mg in the conventional therapy group. The pulse corticosteroid therapy group also experienced fewer adverse events (25.8 % vs. 61.3 %, p = 0.010). CONCLUSION: Pulse corticosteroid therapy provides similar treatment efficacy, earlier reduction in corticosteroid dosage, and a lower incidence of adverse events compared to conventional therapy in patients with anti-ARS ILD. These findings highlight the potential benefit of a steroid-sparing strategy, suggesting that pulse corticosteroid therapy may be considered an effective and safer option in managing this condition.

DOI： 10.1016/j.rmed.2025.108070

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Enfortumab vedotin (EV), an antibody-drug conjugate targeting Nectin-4, has demonstrated efficacy against advanced urothelial carcinoma. While initially considered rare, EV-induced interstitial lung disease (ILD) is increasingly recognized, yet its pathological features remain poorly characterized. We report a case of a 60-year-old man with metastatic urothelial carcinoma who developed fever, fatigue, and cough after two cycles of EV therapy. His treatment history included right nephroureterectomy, platinum-based chemotherapy, and immune checkpoint inhibitors nivolumab and pembrolizumab. Laboratory tests revealed elevated serum ILD markers (Krebs von den Lungen-6527.7 U/mL, surfactant protein-D 294.5 ng/mL), and chest computed tomography showed new infiltrative shadows with air bronchogram predominantly in subpleural regions of the right lower lobe, consistent with organizing pneumonia pattern. Bronchoalveolar lavage from the right middle lobe showed 92 % macrophages with negative cultures. Transbronchial lung cryobiopsy revealed fibrosing nonspecific interstitial pneumonia with prominent fibrosis around bronchovascular bundles, lymphocytic infiltration in vessel walls and alveolar septa with myxofibrous thickening, epithelial injury, and fibrin exudation into alveolar spaces-representing previously undocumented features of EV-induced ILD. Drug discontinuation alone proved insufficient, but the patient improved markedly with methylprednisolone pulse therapy. This case highlights two key findings: detailed histopathological characterization through cryobiopsy documents distinct pathological features of EV-induced ILD; and early bronchoscopic evaluation helped guide therapeutic decision-making, supporting aggressive corticosteroid therapy. These findings advance our understanding of both the pathological features and management of EV-induced ILD, particularly relevant as EV-pembrolizumab combination becomes standard first-line treatment.

DOI： 10.1016/j.rmcr.2025.102237

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

INTRODUCTION: Noninvasive evaluation of airway conditions is considered useful in the management of COPD, although assessing airway remodeling remains difficult in routine clinical practice. The impulse oscillometry system used in this study allows separate analysis of inspiratory and expiratory phases, offering detailed insights into airway function. This study examined the effects of inhaled corticosteroids (ICSs) on airway remodeling and assessed the utility of this system in COPD management. METHODS: Stable COPD patients on LAMA/LABA for over a year were assessed by spirometry and impulse oscillometry at baseline and after 48 weeks of ICS/LAMA/LABA therapy. Symptoms, imaging, and blood tests were also evaluated. RESULTS: Among 52 patients (mean baseline %FEV1/predicted: 56.9% ± 22.1%), all had one to two moderate exacerbations in the past year despite LAMA/LABA therapy. Significant correlations were observed between spirometry and MostGraph (e.g., baseline FEV1 vs. R5: r = -0.54). Although spirometry showed no significant changes, Fres improved significantly (-2.11 ± 0.35, p < 0.0001), with reductions in both expiratory and inspiratory phases. CONCLUSIONS: Fres measured by MostGraph significantly improved after ICS addition, whereas no significant changes were observed in spirometry or resistance parameters. Fres also showed significant correlations with FEV1, suggesting that it may capture airway changes not detected by spirometry. These findings support further investigation into its role as a noninvasive marker in COPD. TRIAL REGISTRATION: UMIN-CTR Clinical Trial: UMIN000040764 (https://upload.umin.ac.jp/cgi-open-bin/ctr_e/ctr_view.cgi?recptno=R000042394).

DOI： 10.1111/crj.70105

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

INTRODUCTION: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare complication during dupilumab therapy. However, the optimal treatment strategy for dupilumab-triggered EGPA remains unclear, particularly the timing and role of anti-interleukin-5 therapy. CASE STUDY: A 48-year-old female with severe eosinophilic rhinosinusitis and asthma, increased blood eosinophils (2098/μL) and myeloperoxidase-specific antineutrophil cytoplasmic antibody (MPO-ANCA) (46.1 U/mL) without vasculitic symptoms, developed systemic symptoms, including fever, arthralgia, and peripheral neuropathy immediately after dupilumab administration. RESULTS: Physical assessment revealed bilateral expiratory wheezes and laboratory tests revealed marked elevations in blood eosinophil (11,889/μL) and MPO-ANCA levels (125.0 U/mL). Other conditions, including parasitic infections, allergic bronchopulmonary aspergillosis, and FIP1L1-PDGFRA-positive disease, were excluded. Early intervention with mepolizumab (300 mg), methylprednisolone pulse therapy, and intravenous immunoglobulin (IVIG) was initiated after discontinuing dupilumab, resulting in rapid normalization of blood eosinophil counts and clinical improvement. Residual neuropathy was successfully treated with intravenous immunoglobulin (IVIg). Prednisolone was reduced to 15 mg daily with negative MPO-ANCA one month after treatment initiation. CONCLUSION: This case emphasizes the importance of monitoring preexisting MPO-ANCA and blood eosinophils before and during dupilumab therapy. Early intervention with mepolizumab combined with conventional therapy is considered an optimal treatment strategy for dupilumab-triggered EGPA.

DOI： 10.2147/JAA.S513640

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Background: Ixekizumab, an interleukin-17A (IL-17A) inhibitor used in psoriasis treatment, has been linked to drug-induced interstitial lung disease (DI-ILD). The pathophysiological mechanisms and histopathological features of this adverse effect remain poorly documented. Case Presentation: A 69-year-old male with familial psoriasis developed respiratory symptoms after 18 months of ixekizumab therapy. His medical history included mild smoking-related interstitial pneumonia and chronic obstructive pulmonary disease. One month after treatment, he presented with cough and dyspnea. High-resolution chest CT showed bilateral ground-glass opacities, accompanied by elevated Krebs von den Lungen-6 and surfactant protein-D levels. Transbronchial lung cryobiopsy (TBLC) revealed a fibrotic non-specific interstitial pneumonia pattern with granulomatous changes. Immunohistochemical analysis demonstrated a predominance of CD4-positive cells and IL-17A-positive lymphocytes, suggesting Th17 cell involvement in the pathogenesis. The patient's condition improved following ixekizumab discontinuation. Conclusions: This case identifies distinct histopathological features in ixekizumab-induced DI-ILD, particularly the presence of granulomatous changes and Th17 cell involvement. The findings suggest that IL-17A inhibition may trigger pulmonary inflammation through Th17 cell function dysregulation. This observation supports the importance of careful pulmonary monitoring in patients receiving biologic therapies for psoriasis, particularly those with pre-existing lung conditions. TBLC may contribute to understanding the pathogenesis of this drug-induced complication.

DOI： 10.1080/09546634.2024.2424338

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: Transbronchial lung cryobiopsy (TBLC) has emerged as a promising diagnostic tool for interstitial lung disease (ILD). This study aimed to assess the initial experience, procedural learning curve, and influence of sedative medications on complication rates, particularly bleeding and pneumothorax, in the implementation of a TBLC program for ILD diagnosis. METHODS: In this retrospective cohort study, we analysed 119 patients who underwent TBLC at Nippon Medical School Hospital from April 2021 to March 2024. Procedural times, complication rates, and histopathological outcomes were evaluated. The learning curve was assessed using cumulative sum (CUSUM) analysis, focusing on procedure time and biopsy yield. The association between sedative medication dosages and bleeding risk was also examined. RESULTS: The overall diagnostic yield was high, with alveolar tissue obtained in 97.5% of cases and a definitive pathological diagnosis achieved in 81.5% of patients. CUSUM analysis revealed a proficiency threshold at approximately 56 cases, with improved efficiency and biopsy yield in the consolidation phase. Fentanyl dosage was significantly associated with reduced bleeding complications (odds ratio 0.51, 95% confidence interval 0.27-0.97, p = 0.041). CONCLUSIONS: TBLC is a safe and effective diagnostic tool for ILDs, with a manageable learning curve for procedural efficiency. Sedation, particularly fentanyl dosage, may plays a crucial role in minimizing complications, but further research is needed to clarify this relationship. These findings support the adoption of TBLC as a standard diagnostic approach for ILD and highlight the importance of adequate training and optimized sedation protocols to ensure safety and efficacy in clinical practice.

DOI： 10.1186/s12890-024-03359-1

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: In compatible with fibrotic hypersensitivity pneumonitis (HP) of the computed tomography (CT) classification using the American Thoracic Society (ATS)/Japanese Respiratory Society (JRS)/Latin American Thoracic Association (ALAT) HP guidelines, the lung fibrosis pattern was classified as either a usual interstitial pneumonia (UIP) pattern or a diffuse ground-glass opacity (GGO) pattern with subtle fibrosis. We investigated whether patients with the same imaging classification had different disease progression. We also attempted to reclassify these patients using the CHEST HP guidelines. METHODS: Patients with fibrotic HP who had compatible CT pattern in the ATS/JRS/ALAT classification were investigated retrospectively. RESULTS: With 62 patients in the UIP pattern group and 25 patients in the diffuse GGO pattern group, 87 patients with fibrotic HP had compatible pattern on CT. Annual forced vital capacity changes in the UIP pattern group and diffuse GGO pattern group were -2.7% and +3.3% (P=0.004), respectively. The 5-year survival rates in the UIP pattern group and diffuse GGO pattern group were 86% and 100% (P=0.02). In UIP pattern group in the ATS/JRS/ALAT classification, 27% patients were classified as typical fibrotic HP pattern in the CHEST guidelines. In the diffuse GGO pattern group, 52% patients were classified as typical pattern of fibrotic HP. In the CHEST guidelines, more patients in the GGO pattern were classified as typical pattern compared with those in the UIP pattern (P=0.02). CONCLUSIONS: The two patterns in compatible with fibrotic HP of CT classification using the ATS/JRS/ALAT HP guidelines had different disease progression. Typical patterns were more frequent in the CHEST guidelines than the ATS/JRS/ALAT guidelines.

DOI： 10.21037/jtd-23-1845

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Abemaciclib, a cyclin-dependent kinase 4/6 inhibitor, is crucial in treating hormone receptor-positive, human epidermal growth factor receptor 2-negative metastatic or recurrent breast cancer. However, its association with drug-induced interstitial lung disease (DI-ILD) is concerning. We present an 82-year-old woman with breast cancer receiving abemaciclib, who developed persistent cough and malaise. Initial diagnostics suggested pneumonia, supported by ground-glass opacities and consolidations on chest high-resolution computed tomography. Suspecting DI-ILD, a transbronchial lung cryobiopsy (TBLC) was performed, revealing fibrosing organizing pneumonia and confirming abemaciclib-induced ILD. Discontinuing abemaciclib led to significant symptom improvement, supporting the diagnosis. This case report describes the clinical presentation and diagnostic approach in a patient with suspected abemaciclib-induced ILD, including the use. To our knowledge, this is the first reported case of fibrosing organizing pneumonia as a histopathological pattern in abemaciclib-induced ILD, expanding knowledge of this therapy's pulmonary adverse events. Histopathological features included diffuse lymphocytic infiltration, polypoid intra-alveolar fibrosis, intraluminal granulation tissue plugs with dense hyalinization, hyalinized fibrotic alveolar septa lesions, and obliterative fibrotic processes affecting alveolar ducts. Our case suggests that TBLC might be useful in recognizing DI-ILD by providing detailed lung tissue examination, which can facilitate early diagnosis and guide management. Identifying fibrosing organizing pneumonia indicated a potentially corticosteroid-responsive pathology, suggesting a more favorable prognosis compared with patterns like diffuse alveolar damage. This case highlights the potential for abemaciclib-induced ILD to occur even after prolonged treatment periods, emphasizing the importance of vigilance and consideration of diagnostic intervention for patients on cyclin-dependent kinase 4/6 inhibitors presenting with respiratory symptoms. Timely recognition and appropriate management may mitigate adverse outcomes. Further studies are needed to confirm these findings and to better understand the role of TBLC and histopathological examination in diagnosing and managing abemaciclib-induced ILD.

DOI： 10.1177/11782234241301314

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

A 69-year-old woman was diagnosed with idiopathic interstitial pneumonia (IIP). The patient underwent a combination therapy of steroid therapy and intravenous cyclophosphamide, long-term oxygen therapy, and the initiation of Nintedanib. However, there was no improvement in IIP, and as a result, the activities of daily living also declined. As one of the various examinations conducted, the results of the right heart catheterization diagnosed the patient with mild pulmonary hypertension, and Macitentan therapy was initiated. The subsequent clinical course appeared to show an improvement in Idiopathic Interstitial Pneumonia (IIP) by adding Macitentan therapy to Nintedanib therapy.

DOI： 10.1016/j.rmcr.2024.102058

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記述言語：日本語   出版者・発行元：(NPO)日本肺癌学会  

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その他リンク： https://search.jamas.or.jp/default/link?pub_year=2019&ichushi_jid=J01244&link_issn=&doc_id=20190314320011&doc_link_id=%2Fec7jaluc%2F2019%2F005901%2F011%2F0088-0093%26dl%3D0&url=https%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fec7jaluc%2F2019%2F005901%2F011%2F0088-0093%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: The prevalence of chronic obstructive pulmonary disease (COPD) is 8.6% in Japan and 10% worldwide. Unfortunately, many patients with COPD are not correctly identified and appropriately educated regarding the condition. In this paper, we demonstrate that some citizens of Ebina City with symptoms suspicious for COPD, such as cough, sputum production, and shortness of breath, have undiagnosed COPD. We describe our activities to raise awareness of COPD through a 10-year campaign. METHODS: From 2006 to 2015, we developed activities to raise awareness of COPD, including public lectures, utilization of pulmonary function tests, and questionnaires on subjective symptoms and knowledge of COPD. RESULTS: Among 1,206 participants aged>40 years, COPD was suspected in 5.6%, as indicated by airway obstruction (i.e. forced expiratory volume in 1 second (FEV1)/forced vital capacity ratio (FVC) <0.70). However, most of these participants were not diagnosed with COPD. Furthermore, half of these participants had not consulted a medical institution despite demonstrating symptoms. Results of the COPD awareness questionnaire, which was administered to 1,055 people, indicated that 65% of survey respondents were unaware of COPD. CONCLUSIONS: There are individuals with symptoms suspicious for COPD who are unaware of the disease at the Plaza in Ebina City. Clinicians have a responsibility to raise public awareness of COPD and to reduce the prevalence of COPD and its associated mortality.

DOI： 10.1272/jnms.JNMS.2019_86-6

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記述言語：日本語   出版者・発行元：(一社)日本呼吸器内視鏡学会  

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記述言語：日本語   出版者・発行元：(一社)日本呼吸器内視鏡学会  

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記述言語：日本語   出版者・発行元：(一社)日本呼吸器内視鏡学会  

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記述言語：日本語   出版者・発行元：(一社)日本呼吸器学会  

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記述言語：日本語   出版者・発行元：(一社)日本呼吸器学会  

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記述言語：日本語   出版者・発行元：(一社)日本呼吸器学会  

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記述言語：日本語   出版者・発行元：(一社)日本呼吸器学会  

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記述言語：日本語   出版者・発行元：日本医科大学医学会  

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記述言語：日本語   出版者・発行元：日本サルコイドーシス  

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記述言語：日本語   出版者・発行元：(一社)日本呼吸器内視鏡学会  

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記述言語：英語   掲載種別：研究発表ペーパー・要旨（国際会議）  

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記述言語：日本語   出版者・発行元：(一社)日本呼吸器内視鏡学会  

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記述言語：日本語   出版者・発行元：(一社)日本呼吸器内視鏡学会  

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記述言語：日本語   出版者・発行元：(一社)日本呼吸器学会  

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記述言語：日本語   出版者・発行元：(一社)日本呼吸器学会  

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記述言語：日本語   出版者・発行元：(NPO)日本肺癌学会  

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