Primary ciliary dyskinesia (PCD) is a rare genetic disorder associated with structural or functional ciliary defects. Its diagnosis usually requires a combination of tests, including observation of the cilium structure by transmission electron microscopy (TEM). In Japan, when TEM was previously the primary method for diagnosing PCD, there were no standardized methods for evaluating TEM findings, and the correlations with PCD-causing genes remained unclear.
Methods
Here, we evaluated 101 patients with suspected PCD using a standardized assessment of ciliary ultrastructure by TEM, based on the international guidelines that classify defects into Class 1 and Class 2.
Results
Class 1 or 2 ciliary defects on TEM were identified in 27 patients (26.7 %); Class 1 defects (n = 19) correlated well with the genotypes in Japanese patients with PCD, most commonly caused by DNAH5 and CCDC39/40. TEM proved valuable as a complementary tool, when genetic testing failed to yield a definitive diagnosis, particularly in cases where two heterozygous variants of unknown phase were identified in a single PCD-causing gene. It was especially informative in PCD patients with situs inversus. However, in cases involving homozygous DRC1 variants, which are most frequently identified among Japanese patients with PCD, only subtle ultrastructural defects were observed, highlighting the limitations of TEM as a standalone diagnostic method. Our study also demonstrated the need for and effectiveness of standardizing the assessment of ciliary ultrastructure by TEM.
Conclusions
The results of this study provide a crucial foundation for establishing a better TEM-based diagnostic system in Japan.
{"title":"Diagnostic utility of standardized assessment of ciliary ultrastructure by transmission electron microscopy in primary ciliary dyskinesia: a Japanese perspective","authors":"Akiko Miyabayashi , Keiko Wakabayashi , Hiroyuki Yamada , Masashi Ito , Kozo Morimoto , Minako Hijikata , Naoto Keicho","doi":"10.1016/j.resinv.2025.10.006","DOIUrl":"10.1016/j.resinv.2025.10.006","url":null,"abstract":"<div><h3>Background</h3><div>Primary ciliary dyskinesia (PCD) is a rare genetic disorder associated with structural or functional ciliary defects. Its diagnosis usually requires a combination of tests, including observation of the cilium structure by transmission electron microscopy (TEM). In Japan, when TEM was previously the primary method for diagnosing PCD, there were no standardized methods for evaluating TEM findings, and the correlations with PCD-causing genes remained unclear.</div></div><div><h3>Methods</h3><div>Here, we evaluated 101 patients with suspected PCD using a standardized assessment of ciliary ultrastructure by TEM, based on the international guidelines that classify defects into Class 1 and Class 2.</div></div><div><h3>Results</h3><div>Class 1 or 2 ciliary defects on TEM were identified in 27 patients (26.7 %); Class 1 defects (n = 19) correlated well with the genotypes in Japanese patients with PCD, most commonly caused by <em>DNAH5</em> and <em>CCDC39/40</em>. TEM proved valuable as a complementary tool, when genetic testing failed to yield a definitive diagnosis, particularly in cases where two heterozygous variants of unknown phase were identified in a single PCD-causing gene. It was especially informative in PCD patients with situs inversus. However, in cases involving homozygous <em>DRC1</em> variants, which are most frequently identified among Japanese patients with PCD, only subtle ultrastructural defects were observed, highlighting the limitations of TEM as a standalone diagnostic method. Our study also demonstrated the need for and effectiveness of standardizing the assessment of ciliary ultrastructure by TEM.</div></div><div><h3>Conclusions</h3><div>The results of this study provide a crucial foundation for establishing a better TEM-based diagnostic system in Japan.</div></div>","PeriodicalId":20934,"journal":{"name":"Respiratory investigation","volume":"63 6","pages":"Pages 1268-1273"},"PeriodicalIF":2.0,"publicationDate":"2025-10-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145313547","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pneumothorax often requires chest drain insertion, and a clamping test is sometimes performed before removal to minimize lung re–collapse. However, evidence supporting its utility remains limited. This study evaluated the utility of the clamping procedure and identified risk factors associated with lung re–collapse.
Methods
We retrospectively analyzed 440 patients with pneumothorax treated at Fukujuji Hospital from January 2016 to April 2025. The primary outcome was the frequency of lung re–collapse during the clamping test. To identify associated risk factors, patients who experienced re–collapse during the test (re–collapse group) were compared with those who completed the test successfully (success group). Patients who did not undergo clamping (nonclamping group) were also reviewed.
Results
Among 440 patients, 355 underwent clamping, including 41 (11.5 %) in the re–collapse group. Incomplete lung expansion (61.0 % vs. 29.9 %, p < 0.001), the presence of lung fluctuations (82.9 % vs. 43.0 %, p < 0.001) before clamping, and a history of smoking (85.4 % vs. 65.1 %, p = 0.008) were significantly more common in the re–collapse group than in the success group. The relapse rates were 45.2 % among patients with all risk factors, 12.7 % among those with two risk factors, 3.5 % among those with one risk factor, and 2.6 % among those with none. Similar trends were observed in analyses including the nonclamping group.
Conclusion
The clamping test helped avoid chest tube reinsertion in 11.5 % of the patients. Incomplete lung expansion, fluctuations prior to clamping, and a history of smoking were identified as risk factors for re–collapse, suggesting their potential utility in guiding the need for clamping.
{"title":"Efficacy of chest tube clamping prior to removal in patients with pneumothorax","authors":"Masafumi Shimoda, Yoshiaki Tanaka, Kozo Morimoto, Ryo Yanagawa, Kozo Yoshimori, Shoji Kudoh","doi":"10.1016/j.resinv.2025.10.004","DOIUrl":"10.1016/j.resinv.2025.10.004","url":null,"abstract":"<div><h3>Background</h3><div>Pneumothorax often requires chest drain insertion, and a clamping test is sometimes performed before removal to minimize lung re–collapse. However, evidence supporting its utility remains limited. This study evaluated the utility of the clamping procedure and identified risk factors associated with lung re–collapse.</div></div><div><h3>Methods</h3><div>We retrospectively analyzed 440 patients with pneumothorax treated at Fukujuji Hospital from January 2016 to April 2025. The primary outcome was the frequency of lung re–collapse during the clamping test. To identify associated risk factors, patients who experienced re–collapse during the test (re–collapse group) were compared with those who completed the test successfully (success group). Patients who did not undergo clamping (nonclamping group) were also reviewed.</div></div><div><h3>Results</h3><div>Among 440 patients, 355 underwent clamping, including 41 (11.5 %) in the re–collapse group. Incomplete lung expansion (61.0 % vs. 29.9 %, <em>p</em> < 0.001), the presence of lung fluctuations (82.9 % vs. 43.0 %, <em>p</em> < 0.001) before clamping, and a history of smoking (85.4 % vs. 65.1 %, <em>p</em> = 0.008) were significantly more common in the re–collapse group than in the success group. The relapse rates were 45.2 % among patients with all risk factors, 12.7 % among those with two risk factors, 3.5 % among those with one risk factor, and 2.6 % among those with none. Similar trends were observed in analyses including the nonclamping group.</div></div><div><h3>Conclusion</h3><div>The clamping test helped avoid chest tube reinsertion in 11.5 % of the patients. Incomplete lung expansion, fluctuations prior to clamping, and a history of smoking were identified as risk factors for re–collapse, suggesting their potential utility in guiding the need for clamping.</div></div>","PeriodicalId":20934,"journal":{"name":"Respiratory investigation","volume":"63 6","pages":"Pages 1262-1267"},"PeriodicalIF":2.0,"publicationDate":"2025-10-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145302927","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-10-11DOI: 10.1016/j.resinv.2025.10.005
Shun Matsuura
Patient-related factors—including performance status, nutritional state, and physical function—are increasingly acknowledged as essential in guiding treatment choices and predicting prognosis for advanced lung cancer. Although the Eastern Cooperative Oncology Group Performance Status (ECOG-PS) is still the most widely used clinical measure, it is subjective by nature and often shows variation between assessors. Malnutrition and sarcopenia, which are frequently overlooked in everyday practice, have been identified as important prognostic indicators that can be measured objectively. This review examines how ECOG-PS, malnutrition, and sarcopenia interact, pointing out the shortcomings of relying solely on ECOG-PS and suggesting supplementary or alternative evaluation methods. Malnutrition, determined through a combination of serum albumin levels, inflammatory markers, and body mass index, was strongly linked to lower ECOG-PS scores and poorer survival. Sarcopenia—particularly when evaluated through muscle quality or functional measures such as grip strength and walking speed—is related to deteriorated ECOG-PS and worse treatment results. Although there is inconsistency in diagnostic standards and inherent limitations in retrospective research, the evidence indicates that integrating objective assessments of nutritional and functional status may improve personalized treatment strategies and prognostic precision. Using straightforward and easily available clinical measures could help identify physical inactivity earlier, enabling more suitable therapeutic actions. Assessing patient-related factors such as malnutrition and sarcopenia in addition to ECOG-PS before treatment may further enhance outcomes and quality of life for patients with advanced lung cancer.
{"title":"Patient-related factors in advanced lung cancer: A review of ECOG-PS, malnutrition, and sarcopenia","authors":"Shun Matsuura","doi":"10.1016/j.resinv.2025.10.005","DOIUrl":"10.1016/j.resinv.2025.10.005","url":null,"abstract":"<div><div>Patient-related factors—including performance status, nutritional state, and physical function—are increasingly acknowledged as essential in guiding treatment choices and predicting prognosis for advanced lung cancer. Although the Eastern Cooperative Oncology Group Performance Status (ECOG-PS) is still the most widely used clinical measure, it is subjective by nature and often shows variation between assessors. Malnutrition and sarcopenia, which are frequently overlooked in everyday practice, have been identified as important prognostic indicators that can be measured objectively. This review examines how ECOG-PS, malnutrition, and sarcopenia interact, pointing out the shortcomings of relying solely on ECOG-PS and suggesting supplementary or alternative evaluation methods. Malnutrition, determined through a combination of serum albumin levels, inflammatory markers, and body mass index, was strongly linked to lower ECOG-PS scores and poorer survival. Sarcopenia—particularly when evaluated through muscle quality or functional measures such as grip strength and walking speed—is related to deteriorated ECOG-PS and worse treatment results. Although there is inconsistency in diagnostic standards and inherent limitations in retrospective research, the evidence indicates that integrating objective assessments of nutritional and functional status may improve personalized treatment strategies and prognostic precision. Using straightforward and easily available clinical measures could help identify physical inactivity earlier, enabling more suitable therapeutic actions. Assessing patient-related factors such as malnutrition and sarcopenia in addition to ECOG-PS before treatment may further enhance outcomes and quality of life for patients with advanced lung cancer.</div></div>","PeriodicalId":20934,"journal":{"name":"Respiratory investigation","volume":"63 6","pages":"Pages 1250-1261"},"PeriodicalIF":2.0,"publicationDate":"2025-10-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145280973","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
This pilot study evaluated the effects of inhaled N-acetylcysteine (NAC) on sputum rheology in patients with chronic productive cough (CPC). Rheological measurements before and 30 min after inhalation were compared retrospectively in 16 outpatients receiving either NAC (n = 9) or bromhexine hydrochloride (BXH) (n = 7). NAC inhalation significantly reduced critical strain (γC), an indicator of sputum stringiness, from 2370 [1310–4390] % to 643 [389–700] % (median and interquartile range), with a significantly greater effect than BXH. This reduction was observed regardless of airway fungal colonization. In addition, two case reports, a 67-year-old man with bronchorrhea and a 79-year-old woman with refractory asthma, demonstrated improved quality-of-life scores evaluated with the Cough and Sputum Assessment Questionnaire (CASA-Q) and rheological improvement following 1–2 weeks of twice-daily NAC inhalation. These findings suggest that nebulized NAC may be a promising add-on therapy for refractory airway diseases characterized by high sputum stringiness (γC).
{"title":"N-acetylcysteine inhalation improved sputum rheology in chronic productive cough: Clinical application in two cases","authors":"Haruhiko Ogawa , Yuka Uchida , Lydia Esteban Enjuto","doi":"10.1016/j.resinv.2025.08.009","DOIUrl":"10.1016/j.resinv.2025.08.009","url":null,"abstract":"<div><div>This pilot study evaluated the effects of inhaled <em>N</em>-acetylcysteine (NAC) on sputum rheology in patients with chronic productive cough (CPC). Rheological measurements before and 30 min after inhalation were compared retrospectively in 16 outpatients receiving either NAC (<em>n</em> = 9) or bromhexine hydrochloride (BXH) (<em>n</em> = 7). NAC inhalation significantly reduced critical strain (<em>γ</em><sub>C</sub>), an indicator of sputum stringiness, from 2370 [1310–4390] % to 643 [389–700] % (median and interquartile range), with a significantly greater effect than BXH. This reduction was observed regardless of airway fungal colonization. In addition, two case reports, a 67-year-old man with bronchorrhea and a 79-year-old woman with refractory asthma, demonstrated improved quality-of-life scores evaluated with the Cough and Sputum Assessment Questionnaire (CASA-Q) and rheological improvement following 1–2 weeks of twice-daily NAC inhalation. These findings suggest that nebulized NAC may be a promising add-on therapy for refractory airway diseases characterized by high sputum stringiness (<em>γ</em><sub>C</sub>).</div></div>","PeriodicalId":20934,"journal":{"name":"Respiratory investigation","volume":"63 6","pages":"Pages 1246-1249"},"PeriodicalIF":2.0,"publicationDate":"2025-10-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145258964","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Primary ciliary dyskinesia (PCD) is a rare genetic disorder with ciliary dysfunction. The triad of situs inversus, chronic sinusitis, and bronchiectasis is termed Kartagener syndrome. We report a PCD patient presenting with Kartagener syndrome with a novel combination of DNAH11 alleles, i.e., a frameshift variant, c.2709del p.(Trp904Glyfs∗5), and a nonsense variant, c.3020T>G p.(Leu1007∗). The biallelic pathogenic variants were both rare and located close to the N-terminus. High-speed video microscopy revealed discordant cilia and diffuse-twisting wave-like movements of the airway epithelial cell populations. Researchers should note this phenomenon, and further investigation is necessary to clarify the role of DNAH11 in PCD.
{"title":"Biallelic c.2709del and c.3020T>G cause DNAH11-related primary ciliary dyskinesia presenting with Kartagener syndrome: Possible novel phenotype of diffuse-twisting wave-like movements of airway epithelial cell populations","authors":"Akira Umeda , Hidenori Kiyosawa , Kazuya Miyagawa , Yoshiyuki Ohira , Masataka Higuchi , Ho Namkoong , Takanori Asakura , Makoto Ishii , Naoki Hasegawa , Koichi Fukunaga","doi":"10.1016/j.resinv.2025.09.023","DOIUrl":"10.1016/j.resinv.2025.09.023","url":null,"abstract":"<div><div>Primary ciliary dyskinesia (PCD) is a rare genetic disorder with ciliary dysfunction. The triad of situs inversus, chronic sinusitis, and bronchiectasis is termed Kartagener syndrome. We report a PCD patient presenting with Kartagener syndrome with a novel combination of <em>DNAH11</em> alleles, i.e., a frameshift variant, c.2709del p.(Trp904Glyfs∗5), and a nonsense variant, c.3020T>G p.(Leu1007∗). The biallelic pathogenic variants were both rare and located close to the N-terminus. High-speed video microscopy revealed discordant cilia and diffuse-twisting wave-like movements of the airway epithelial cell populations. Researchers should note this phenomenon, and further investigation is necessary to clarify the role of DNAH11 in PCD.</div></div>","PeriodicalId":20934,"journal":{"name":"Respiratory investigation","volume":"63 6","pages":"Pages 1243-1245"},"PeriodicalIF":2.0,"publicationDate":"2025-10-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145258897","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
The role of single lung transplantation (SLT) in secondary pulmonary hypertension (SPH) remains controversial. This study evaluated the feasibility and long-term outcomes of SLT in patients with SPH—defined as mean pulmonary artery pressure ≥25 mmHg measured by right heart catheterization immediately before transplantation—within Japan's allocation system, characterized by protracted waiting periods.
Methods
We retrospectively reviewed 83 deceased-donor lung transplantations, categorized into four groups: SLT without SPH (n = 14), SLT with SPH (n = 19), bilateral lung transplantation (BLT) without SPH (n = 12), and BLT with SPH (n = 38). Outcomes analyzed included 5-year survival, CLAD-free survival, and exercise tolerance.
Results
At transplantation, 68.6 % of recipients exhibited SPH, a significant increase compared to registration. Among SLT recipients, the SPH group demonstrated 5-year survival (83.2 %) and CLAD-free survival (65.9 %) not inferior to those of the non-SPH group (50.0 % and 56.8 %, respectively). Perfusion ratios and 6-min walk distances remained comparable between SPH and non-SPH groups for up to five years. Additionally, 5-year survival (83.2 % vs. 81.1 %) and CLAD-free survival (65.9 % vs. 64.5 %) were similar between SLT and BLT. High preoperative oxygen requirements were associated with poorer early graft function in SPH patients receiving SLT. No definitive predictors of CLAD or long-term survival were identified.
Conclusions
SLT is a viable option for patients with mild-to-moderate SPH, providing survival and functional outcomes comparable to BLT. Careful recipient and donor selection remains essential, particularly given the risk of SPH progression during extended waiting periods.
背景：单肺移植（SLT）在继发性肺动脉高压（SPH）中的作用仍有争议。本研究评估了sph患者SLT的可行性和长期结果，sph的定义是在移植前立即通过右心导管测量平均肺动脉压≥25 mmHg，在日本的分配系统中，其特点是等待时间长。方法：回顾性分析83例死亡供体肺移植病例，将其分为4组：无SPH的SLT （n = 14）、无SPH的SLT （n = 19）、无SPH的双侧肺移植（BLT） （n = 12）和双侧肺移植合并SPH （n = 38）。结果分析包括5年生存率、无clad生存率和运动耐量。结果：移植时，68.6%的受者表现出SPH，与登记时相比显著增加。在SLT接受者中，SPH组的5年生存率（83.2%）和无clad生存率（65.9%）不低于非SPH组（分别为50.0%和56.8%）。灌注比和6分钟步行距离在SPH组和非SPH组之间保持可比性长达5年。此外，SLT和BLT的5年生存率（83.2% vs. 81.1%）和无clad生存率（65.9% vs. 64.5%）相似。接受SLT的SPH患者术前高氧需要量与较差的早期移植物功能相关。没有确定明确的预测因子或长期生存。结论：SLT是轻度至中度SPH患者的可行选择，提供与BLT相当的生存和功能结果。仔细选择受体和供体仍然是必要的，特别是考虑到延长等待期SPH进展的风险。
{"title":"Survival and functional outcomes of single lung transplantation in secondary pulmonary hypertension","authors":"Dai Shimizu , Kentaroh Miyoshi , Haruchika Yamamoto , Shin Tanaka , Seiichiro Sugimoto , Mikio Okazaki , Shinichi Toyooka","doi":"10.1016/j.resinv.2025.09.022","DOIUrl":"10.1016/j.resinv.2025.09.022","url":null,"abstract":"<div><h3>Background</h3><div>The role of single lung transplantation (SLT) in secondary pulmonary hypertension (SPH) remains controversial. This study evaluated the feasibility and long-term outcomes of SLT in patients with SPH—defined as mean pulmonary artery pressure ≥25 mmHg measured by right heart catheterization immediately before transplantation—within Japan's allocation system, characterized by protracted waiting periods.</div></div><div><h3>Methods</h3><div>We retrospectively reviewed 83 deceased-donor lung transplantations, categorized into four groups: SLT without SPH (n = 14), SLT with SPH (n = 19), bilateral lung transplantation (BLT) without SPH (n = 12), and BLT with SPH (n = 38). Outcomes analyzed included 5-year survival, CLAD-free survival, and exercise tolerance.</div></div><div><h3>Results</h3><div>At transplantation, 68.6 % of recipients exhibited SPH, a significant increase compared to registration. Among SLT recipients, the SPH group demonstrated 5-year survival (83.2 %) and CLAD-free survival (65.9 %) not inferior to those of the non-SPH group (50.0 % and 56.8 %, respectively). Perfusion ratios and 6-min walk distances remained comparable between SPH and non-SPH groups for up to five years. Additionally, 5-year survival (83.2 % vs. 81.1 %) and CLAD-free survival (65.9 % vs. 64.5 %) were similar between SLT and BLT. High preoperative oxygen requirements were associated with poorer early graft function in SPH patients receiving SLT. No definitive predictors of CLAD or long-term survival were identified.</div></div><div><h3>Conclusions</h3><div>SLT is a viable option for patients with mild-to-moderate SPH, providing survival and functional outcomes comparable to BLT. Careful recipient and donor selection remains essential, particularly given the risk of SPH progression during extended waiting periods.</div></div>","PeriodicalId":20934,"journal":{"name":"Respiratory investigation","volume":"63 6","pages":"Pages 1235-1242"},"PeriodicalIF":2.0,"publicationDate":"2025-10-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145252501","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Acute exacerbations of idiopathic interstitial pneumonias (AE-IIPs) are life-threatening events. However, comparative prognostic data across IIP subtypes during AE are limited. This study aimed to evaluate in-hospital mortality differences among major AE-IIP subtypes using a nationwide database in Japan.
Methods
We retrospectively analysed patients with AE of idiopathic pulmonary fibrosis (IPF), nonspecific interstitial pneumonia (NSIP), cryptogenic organising pneumonia (COP), or acute interstitial pneumonia (AIP) who received high-dose methylprednisolone between 1 July, 2010 and 31 March, 2023. Data were extracted from the Japanese Diagnosis Procedure Combination database. The primary outcome was all-cause in-hospital mortality. Secondary outcomes included 14- and 28-day mortality. Multivariable logistic regression analysis with generalised estimating equations was employed to adjust for potential confounders, incorporating multiple imputation to address missing data.
Results
A total of 6645 patients were included (IPF, n = 2092; NSIP, n = 581; COP, n = 871; AIP, n = 3101). Unadjusted in-hospital mortality rates were 53.9 % for IPF, 40.1 % for NSIP, 17.6 % for COP, and 49.3 % for AIP. After adjustment, in-hospital mortality was significantly higher for IPF (odds ratio [OR], 3.92; 95 % confidence interval [95 % CI], 3.05–5.04; p < 0.001), NSIP (OR, 2.80; 95 % CI, 2.10–3.73; p < 0.001), and AIP (OR, 3.07; 95 % CI, 2.43–3.89; p < 0.001), compared with COP. Similar trends were observed for both secondary outcomes.
Conclusions
Among patients with AE-IIPs, those with IPF, NSIP, and AIP exhibited significantly higher in-hospital mortality compared with COP. These findings underscore the inferior prognosis associated with AE-IPF and AIP.
背景：特发性间质性肺炎（AE-IIPs）急性加重是危及生命的事件。然而，AE期间IIP亚型预后的比较数据有限。本研究旨在利用日本全国数据库评估AE-IIP主要亚型的住院死亡率差异。方法：回顾性分析2010年7月1日至2023年3月31日期间接受大剂量甲基强的松龙治疗的特发性肺纤维化（IPF）、非特异性间质性肺炎（NSIP）、隐源性组织性肺炎（COP）或急性间质性肺炎（AIP） AE患者。数据取自日本诊断程序组合数据库。主要结局为全因住院死亡率。次要结局包括14天和28天死亡率。采用广义估计方程的多变量逻辑回归分析来调整潜在的混杂因素，并结合多重输入来解决缺失数据。结果：共纳入6645例患者（IPF, n = 2092； NSIP, n = 581； COP, n = 871； AIP, n = 3101）。未经调整的住院死亡率为：IPF 53.9%, NSIP 40.1%, COP 17.6%, AIP 49.3%。调整后，IPF的住院死亡率显著高于COP（优势比[OR]， 3.92； 95%可信区间[95% CI]， 3.05-5.04; p）结论：在ae - iip患者中，IPF、NSIP和AIP患者的住院死亡率显著高于COP。这些发现强调AE-IPF和AIP的预后较差。
{"title":"Prognostic comparison of acute exacerbations across idiopathic interstitial pneumonia subtypes: A nationwide observational study","authors":"Nobuyasu Awano , Shotaro Aso , Takehiro Izumo , Hiroki Matsui , Kiyohide Fushimi , Hideo Yasunaga","doi":"10.1016/j.resinv.2025.10.001","DOIUrl":"10.1016/j.resinv.2025.10.001","url":null,"abstract":"<div><h3>Background</h3><div>Acute exacerbations of idiopathic interstitial pneumonias (AE-IIPs) are life-threatening events. However, comparative prognostic data across IIP subtypes during AE are limited. This study aimed to evaluate in-hospital mortality differences among major AE-IIP subtypes using a nationwide database in Japan.</div></div><div><h3>Methods</h3><div>We retrospectively analysed patients with AE of idiopathic pulmonary fibrosis (IPF), nonspecific interstitial pneumonia (NSIP), cryptogenic organising pneumonia (COP), or acute interstitial pneumonia (AIP) who received high-dose methylprednisolone between 1 July, 2010 and 31 March, 2023. Data were extracted from the Japanese Diagnosis Procedure Combination database. The primary outcome was all-cause in-hospital mortality. Secondary outcomes included 14- and 28-day mortality. Multivariable logistic regression analysis with generalised estimating equations was employed to adjust for potential confounders, incorporating multiple imputation to address missing data.</div></div><div><h3>Results</h3><div>A total of 6645 patients were included (IPF, <em>n</em> = 2092; NSIP, <em>n</em> = 581; COP, <em>n</em> = 871; AIP, <em>n</em> = 3101). Unadjusted in-hospital mortality rates were 53.9 % for IPF, 40.1 % for NSIP, 17.6 % for COP, and 49.3 % for AIP. After adjustment, in-hospital mortality was significantly higher for IPF (odds ratio [OR], 3.92; 95 % confidence interval [95 % CI], 3.05–5.04; <em>p</em> < 0.001), NSIP (OR, 2.80; 95 % CI, 2.10–3.73; <em>p</em> < 0.001), and AIP (OR, 3.07; 95 % CI, 2.43–3.89; <em>p</em> < 0.001), compared with COP. Similar trends were observed for both secondary outcomes.</div></div><div><h3>Conclusions</h3><div>Among patients with AE-IIPs, those with IPF, NSIP, and AIP exhibited significantly higher in-hospital mortality compared with COP. These findings underscore the inferior prognosis associated with AE-IPF and AIP.</div></div>","PeriodicalId":20934,"journal":{"name":"Respiratory investigation","volume":"63 6","pages":"Pages 1229-1234"},"PeriodicalIF":2.0,"publicationDate":"2025-10-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145244941","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-10-03DOI: 10.1016/j.resinv.2025.09.006
Serena Ferraiuolo , Farah Bou Nasser Eddine , Deborah Ferrante , Andrea Dal Corso , Lorenzo Querin , Marco Rigamonti , PierNatale Brusasca , Alfredo Mento , Antonio Savinelli , Karolina Zytko , Massimo Panizzo , Elisa Ghezzi , Clara Rossini , Chiara Mauro , Andrea Pighini , Claudia Zierold , Fabrizio Bonelli , Paolo Ingallinella
Background
Legionella pneumophila is a leading cause of Legionnaires’ disease, with Legionella pneumophila serogroup 1 (Lp1) being the most commonly detected strain. Current urinary antigen tests (UAT) primarily target Lp1, leading to underdiagnosis of non-Lp1 and other Legionella species. A broad-spectrum, fully automated UAT is needed to improve diagnostic accuracy and patient outcomes.
Methods
A novel chemiluminescence immunoassay-based UAT was developed targeting both Legionella peptidoglycan-associated lipoprotein (PAL) and soluble lipopolysaccharide antigen to enable broader detection. Monoclonal antibodies were generated against PAL and integrated into an automated immunoassay platform. The assay's analytical performance was evaluated using recombinant PAL antigens, urine samples spiked with Legionella strains, and clinical specimens. Sensitivity, specificity, cross-reactivity, interference, and precision were assessed.
Results
The LIAISON® Legionella Urinary Ag assay demonstrated a 97.5 % positive agreement and 100 % negative agreement with the Binax™ UAT in detecting Legionella infections. Importantly, the new test also identified multiple non-Lp1 Legionella species, which the comparator assay failed to detect. The assay showed low imprecision, no cross-reactivity or interference.
Conclusions
The LIAISON® Legionella Urinary Ag Assay, a fully automated chemiluminescence-based UAT, provides highly sensitive and specific detection of Legionella infections, including non-Lp1 strains, addressing a major limitation of existing diagnostics. This assay has the potential to improve early detection, guide targeted antibiotic therapy, and enhance public health surveillance of Legionnaires’ disease.
{"title":"The LIAISON® Legionella Urinary Ag assay: A novel high-throughput, fully automated dual-antigen detection method with improved sensitivity and expanded Legionella species and serogroup coverage","authors":"Serena Ferraiuolo , Farah Bou Nasser Eddine , Deborah Ferrante , Andrea Dal Corso , Lorenzo Querin , Marco Rigamonti , PierNatale Brusasca , Alfredo Mento , Antonio Savinelli , Karolina Zytko , Massimo Panizzo , Elisa Ghezzi , Clara Rossini , Chiara Mauro , Andrea Pighini , Claudia Zierold , Fabrizio Bonelli , Paolo Ingallinella","doi":"10.1016/j.resinv.2025.09.006","DOIUrl":"10.1016/j.resinv.2025.09.006","url":null,"abstract":"<div><h3>Background</h3><div><em>Legionella pneumophila</em> is a leading cause of Legionnaires’ disease, with <em>Legionella pneumophila</em> serogroup 1 (<em>Lp</em>1) being the most commonly detected strain. Current urinary antigen tests (UAT) primarily target <em>Lp</em>1, leading to underdiagnosis of non-<em>Lp</em>1 and other <em>Legionella</em> species. A broad-spectrum, fully automated UAT is needed to improve diagnostic accuracy and patient outcomes.</div></div><div><h3>Methods</h3><div>A novel chemiluminescence immunoassay-based UAT was developed targeting both <em>Legionella</em> peptidoglycan-associated lipoprotein (PAL) and soluble lipopolysaccharide antigen to enable broader detection. Monoclonal antibodies were generated against PAL and integrated into an automated immunoassay platform. The assay's analytical performance was evaluated using recombinant PAL antigens, urine samples spiked with <em>Legionella</em> strains, and clinical specimens. Sensitivity, specificity, cross-reactivity, interference, and precision were assessed.</div></div><div><h3>Results</h3><div>The LIAISON® <em>Legionella</em> Urinary Ag assay demonstrated a 97.5 % positive agreement and 100 % negative agreement with the Binax™ UAT in detecting <em>Legionella</em> infections. Importantly, the new test also identified multiple non-<em>Lp</em>1 <em>Legionella</em> species, which the comparator assay failed to detect. The assay showed low imprecision, no cross-reactivity or interference.</div></div><div><h3>Conclusions</h3><div>The LIAISON® <em>Legionella</em> Urinary Ag Assay, a fully automated chemiluminescence-based UAT, provides highly sensitive and specific detection of <em>Legionella</em> infections, including non-<em>Lp</em>1 strains, addressing a major limitation of existing diagnostics. This assay has the potential to improve early detection, guide targeted antibiotic therapy, and enhance public health surveillance of Legionnaires’ disease.</div></div>","PeriodicalId":20934,"journal":{"name":"Respiratory investigation","volume":"63 6","pages":"Pages 1221-1228"},"PeriodicalIF":2.0,"publicationDate":"2025-10-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145219745","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Studies on the association between chest computed tomography (CT) findings of extensive pulmonary involvement and long-term pulmonary and extrapulmonary coronavirus disease 2019 (COVID-19) sequelae are lacking. This study aimed to investigate the relationship between the severity of pneumonia on admission and residual pulmonary and extrapulmonary complications at three months post-hospitalization.
Methods
Using data from the Japan COVID-19 Task Force database, we conducted quantitative analysis of CT scans of 164 patients obtained at admission and three months later. The parameters included pneumonia volume, total lung volume, and area and density of the pectoralis muscle (PM), subcutaneous and epicardial adipose tissue, and vertebral bone density.
Results
Patients with extensive pneumonia on admission had high residual pneumonia volumes, reduced lung volumes, and decreased area and density of PM at three months. No significant differences were observed in the adipose tissue or bone parameters. The severity of pneumonia at admission was independently associated with PM atrophy.
Conclusions
CT-based quantification of pneumonia extent during the acute phase of COVID-19 may be useful in predicting long-term pulmonary sequelae and muscle wasting. This approach may allow the objective evaluation of Long COVID and facilitate the identification of potential therapeutic targets.
{"title":"Extent of pulmonary involvement on admission predicts long-term pulmonary and muscular sequelae of COVID-19: A longitudinal computed tomography study","authors":"Takashi Shimada , Naoya Tanabe , Shotaro Chubachi , Takanori Asakura , Ho Namkoong , Hiromu Tanaka , Shuhei Azekawa , Shiro Otake , Kensuke Nakagawara , Takahiro Fukushima , Mayuko Watase , Tomoki Maetani , Yusuke Shiraishi , Hideki Terai , Mamoru Sasaki , Soichiro Ueda , Yukari Kato , Norihiro Harada , Shoji Suzuki , Shuichi Yoshida , Koichi Fukunaga","doi":"10.1016/j.resinv.2025.09.014","DOIUrl":"10.1016/j.resinv.2025.09.014","url":null,"abstract":"<div><h3>Background</h3><div>Studies on the association between chest computed tomography (CT) findings of extensive pulmonary involvement and long-term pulmonary and extrapulmonary coronavirus disease 2019 (COVID-19) sequelae are lacking. This study aimed to investigate the relationship between the severity of pneumonia on admission and residual pulmonary and extrapulmonary complications at three months post-hospitalization.</div></div><div><h3>Methods</h3><div>Using data from the Japan COVID-19 Task Force database, we conducted quantitative analysis of CT scans of 164 patients obtained at admission and three months later. The parameters included pneumonia volume, total lung volume, and area and density of the pectoralis muscle (PM), subcutaneous and epicardial adipose tissue, and vertebral bone density.</div></div><div><h3>Results</h3><div>Patients with extensive pneumonia on admission had high residual pneumonia volumes, reduced lung volumes, and decreased area and density of PM at three months. No significant differences were observed in the adipose tissue or bone parameters. The severity of pneumonia at admission was independently associated with PM atrophy.</div></div><div><h3>Conclusions</h3><div>CT-based quantification of pneumonia extent during the acute phase of COVID-19 may be useful in predicting long-term pulmonary sequelae and muscle wasting. This approach may allow the objective evaluation of Long COVID and facilitate the identification of potential therapeutic targets.</div></div>","PeriodicalId":20934,"journal":{"name":"Respiratory investigation","volume":"63 6","pages":"Pages 1215-1220"},"PeriodicalIF":2.0,"publicationDate":"2025-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145207264","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
During the winter and spring of 2024–2025, severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) XEC subvariant became the main cause of the epidemic, and at the same time, major influenza epidemics were also observed. To clarify the differences between COVID-19 and influenza, we conducted a comparative study of patients who required hospitalization during the same period.
Methods
We compared 193 patients with COVID-19 Omicron XEC subvariant and 98 patients with influenza H1N1 pdm09 who required hospitalization.
Results
The following characteristics were significantly more common in the COVID-19 group than in the influenza group: 1) males, 2) patients with chronic kidney disease, malignant tumors, autoimmune diseases, and patients using immunosuppressants, 3) patients with multiple underlying diseases, 4) healthcare-associated pneumonia, 5) pure viral pneumonia, 6) aspiration pneumonia, 7) cases in which antivirals had not been used since the diagnosis of infection, 8) patients who required invasive mechanical ventilation management or intensive care unit admission, and 9) deaths. On the other hand, the following characteristics were significantly more frequently observed in the influenza group than in the COVID-19 group: 1) patients without underlying diseases, 2) patients who had been vaccinated within the past year, 3) community-acquired pneumonia, and 4) mixed bacterial pneumonia.
Conclusions
There were many differences between the COVID-19 group and the influenza group that required hospitalization. The rates of severe illness and mortality in the elderly remain high in the COVID-19 group.
{"title":"Differences between influenza and COVID-19 patients who required hospitalization: A study of the 2024–2025 season","authors":"Naoyuki Miyashita , Yasushi Nakamori , Makoto Ogata , Naoki Fukuda , Akihisa Yamura , Tomoki Ito","doi":"10.1016/j.resinv.2025.09.012","DOIUrl":"10.1016/j.resinv.2025.09.012","url":null,"abstract":"<div><h3>Background</h3><div>During the winter and spring of 2024–2025, severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) XEC subvariant became the main cause of the epidemic, and at the same time, major influenza epidemics were also observed. To clarify the differences between COVID-19 and influenza, we conducted a comparative study of patients who required hospitalization during the same period.</div></div><div><h3>Methods</h3><div>We compared 193 patients with COVID-19 Omicron XEC subvariant and 98 patients with influenza H1N1 pdm09 who required hospitalization.</div></div><div><h3>Results</h3><div>The following characteristics were significantly more common in the COVID-19 group than in the influenza group: 1) males, 2) patients with chronic kidney disease, malignant tumors, autoimmune diseases, and patients using immunosuppressants, 3) patients with multiple underlying diseases, 4) healthcare-associated pneumonia, 5) pure viral pneumonia, 6) aspiration pneumonia, 7) cases in which antivirals had not been used since the diagnosis of infection, 8) patients who required invasive mechanical ventilation management or intensive care unit admission, and 9) deaths. On the other hand, the following characteristics were significantly more frequently observed in the influenza group than in the COVID-19 group: 1) patients without underlying diseases, 2) patients who had been vaccinated within the past year, 3) community-acquired pneumonia, and 4) mixed bacterial pneumonia.</div></div><div><h3>Conclusions</h3><div>There were many differences between the COVID-19 group and the influenza group that required hospitalization. The rates of severe illness and mortality in the elderly remain high in the COVID-19 group.</div></div>","PeriodicalId":20934,"journal":{"name":"Respiratory investigation","volume":"63 6","pages":"Pages 1210-1214"},"PeriodicalIF":2.0,"publicationDate":"2025-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145207297","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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