Background: The mean age at diagnosis of sarcoidosis has been increasing worldwide, yet the clinical characteristics and treatment patterns of elderly-onset cases remain insufficiently defined, particularly in Asian populations.
Methods: We retrospectively investigated 187 consecutive Japanese patients newly diagnosed with sarcoidosis at Jichi Medical University Hospital between 2006 and 2018 who fulfilled the 2015 diagnostic criteria. Patients were classified as elderly (≥65 years, n = 49), middle-aged (45-64 years, n = 82), or younger (<45 years, n = 56). Organ involvement was assessed based on the 2023 Japan Society of Sarcoidosis and Other Granulomatous Disorders standards and the 2014 WASOG criteria. The frequency and distribution of systemic therapy (corticosteroids, methotrexate, other immunosuppressants) and treatment indications were compared across age groups.
Results: The proportion of women was significantly higher in the elderly than in the younger group (81.6% vs. 41.1%, p < 0.01), as was the frequency of cardiac involvement (12.2% vs. 3.6%, p < 0.05). Systemic therapy was initiated less often in the elderly than in the middle-aged group (10.2% vs. 25.4%, p = 0.026), and all indications involved extrapulmonary lesions (cardiac, n = 3; neurological, n = 1; ocular, n = 1). Most treated patients received systemic corticosteroids, with a median duration of 7.0 months.
Conclusion: Elderly-onset sarcoidosis in Japan was characterised by a predominance of women and a higher frequency of cardiac involvement. Systemic therapy was infrequently initiated and only for extrapulmonary disease. These findings underscore the need for systematic evaluation of extrapulmonary organs-particularly the heart-and age-adapted multidisciplinary management.
背景:结节病的平均诊断年龄在世界范围内一直在增加,但老年发病病例的临床特征和治疗模式仍然不够明确,特别是在亚洲人群中。方法:回顾性调查2006年至2018年在日一医科大学附属医院连续确诊的187例结节病患者,这些患者符合2015年诊断标准。患者分为老年(≥65岁,n = 49)、中年(45-64岁,n = 82)和年轻(结果:老年女性比例明显高于年轻组(81.6% vs. 41.1%), p结论:老年结节病在日本以女性为主,且累及心脏的频率更高。全身性治疗很少开始,仅用于肺外疾病。这些发现强调了系统评估肺外器官(尤其是心脏)和年龄适应的多学科管理的必要性。
{"title":"Clinical characteristics and systemic treatment in patients with elderly-onset sarcoidosis: A retrospective single-centre study in Japan.","authors":"Hitokazu Tsukao, Michiru Sawahata, Yoshitaka Yamanouchi, Noritaka Sakamoto, Masayuki Nakayama, Koichi Hagiwara, Makoto Maemondo","doi":"10.1016/j.resinv.2026.101381","DOIUrl":"https://doi.org/10.1016/j.resinv.2026.101381","url":null,"abstract":"<p><strong>Background: </strong>The mean age at diagnosis of sarcoidosis has been increasing worldwide, yet the clinical characteristics and treatment patterns of elderly-onset cases remain insufficiently defined, particularly in Asian populations.</p><p><strong>Methods: </strong>We retrospectively investigated 187 consecutive Japanese patients newly diagnosed with sarcoidosis at Jichi Medical University Hospital between 2006 and 2018 who fulfilled the 2015 diagnostic criteria. Patients were classified as elderly (≥65 years, n = 49), middle-aged (45-64 years, n = 82), or younger (<45 years, n = 56). Organ involvement was assessed based on the 2023 Japan Society of Sarcoidosis and Other Granulomatous Disorders standards and the 2014 WASOG criteria. The frequency and distribution of systemic therapy (corticosteroids, methotrexate, other immunosuppressants) and treatment indications were compared across age groups.</p><p><strong>Results: </strong>The proportion of women was significantly higher in the elderly than in the younger group (81.6% vs. 41.1%, p < 0.01), as was the frequency of cardiac involvement (12.2% vs. 3.6%, p < 0.05). Systemic therapy was initiated less often in the elderly than in the middle-aged group (10.2% vs. 25.4%, p = 0.026), and all indications involved extrapulmonary lesions (cardiac, n = 3; neurological, n = 1; ocular, n = 1). Most treated patients received systemic corticosteroids, with a median duration of 7.0 months.</p><p><strong>Conclusion: </strong>Elderly-onset sarcoidosis in Japan was characterised by a predominance of women and a higher frequency of cardiac involvement. Systemic therapy was infrequently initiated and only for extrapulmonary disease. These findings underscore the need for systematic evaluation of extrapulmonary organs-particularly the heart-and age-adapted multidisciplinary management.</p>","PeriodicalId":20934,"journal":{"name":"Respiratory investigation","volume":"64 2","pages":"101381"},"PeriodicalIF":2.0,"publicationDate":"2026-02-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146137698","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}
Background: Olfactory stimulation with L-menthol may reduce certain discomfort sensations, including dyspnea. Chronic obstructive pulmonary disease (COPD) and idiopathic interstitial pneumonias (IIPs) have distinct physiological characteristics and may elicit different qualitative sensations of dyspnea. Therefore, the qualitative effects of L-menthol inhalation on dyspnea may differ between these chronic lung diseases (CLDs). This study aimed to determine which aspects of dyspnea are influenced by olfactory stimulation with L-menthol in patients with CLDs.
Methods: Thirty-four patients with stable COPD or IIPs (17 each) performed a 6-min walk test (6MWT) under two conditions: wearing a surgical mask alone (control) or wearing a mask with a peppermint oil aroma sticker (0.105 ml, containing 30% L-menthol). A modified Borg scale, the Multidimensional Dyspnea Profile (MDP), and the Language of Dyspnea Questionnaire (LDQ) were used to evaluate endpoints. The 6MWT was selected to induce breathlessness with and without L-menthol.
Results: In the COPD group, L-menthol significantly improved Mental breathing effort on the MDP, and Rapid, Gasping, and Air hunger on the LDQ. In the IIPs group, L-menthol improved only the MDP Hyperpnea dimension. No significant differences were observed in the modified Borg scale before and after the 6MWT between L-menthol and control conditions in either group.
Conclusions: Olfactory stimulation with L-menthol selectively improved aspects of dyspnea in patients with COPD and IIPs, likely reflecting differences in the pathophysiology and severity of each disease.
Trial registration: Japan Registry of Clinical Trials (jRCTs031200400).
{"title":"Effects of olfactory stimulation with L-menthol on sensations of discomfort on exertion in patients with chronic lung diseases.","authors":"Soh Imamura, Takeshi Inagaki, Koichiro Tatsumi, Mitsuhiro Abe, Jun Ikari, Takeshi Kawasaki, Hideki Katsura, Yuri Suzuki, Megumi Katsumata, Takuro Imamoto, Yoshihito Ozawa, Seiichiro Sakao, Takuji Suzuki","doi":"10.1016/j.resinv.2026.101380","DOIUrl":"https://doi.org/10.1016/j.resinv.2026.101380","url":null,"abstract":"<p><strong>Background: </strong>Olfactory stimulation with L-menthol may reduce certain discomfort sensations, including dyspnea. Chronic obstructive pulmonary disease (COPD) and idiopathic interstitial pneumonias (IIPs) have distinct physiological characteristics and may elicit different qualitative sensations of dyspnea. Therefore, the qualitative effects of L-menthol inhalation on dyspnea may differ between these chronic lung diseases (CLDs). This study aimed to determine which aspects of dyspnea are influenced by olfactory stimulation with L-menthol in patients with CLDs.</p><p><strong>Methods: </strong>Thirty-four patients with stable COPD or IIPs (17 each) performed a 6-min walk test (6MWT) under two conditions: wearing a surgical mask alone (control) or wearing a mask with a peppermint oil aroma sticker (0.105 ml, containing 30% L-menthol). A modified Borg scale, the Multidimensional Dyspnea Profile (MDP), and the Language of Dyspnea Questionnaire (LDQ) were used to evaluate endpoints. The 6MWT was selected to induce breathlessness with and without L-menthol.</p><p><strong>Results: </strong>In the COPD group, L-menthol significantly improved Mental breathing effort on the MDP, and Rapid, Gasping, and Air hunger on the LDQ. In the IIPs group, L-menthol improved only the MDP Hyperpnea dimension. No significant differences were observed in the modified Borg scale before and after the 6MWT between L-menthol and control conditions in either group.</p><p><strong>Conclusions: </strong>Olfactory stimulation with L-menthol selectively improved aspects of dyspnea in patients with COPD and IIPs, likely reflecting differences in the pathophysiology and severity of each disease.</p><p><strong>Trial registration: </strong>Japan Registry of Clinical Trials (jRCTs031200400).</p>","PeriodicalId":20934,"journal":{"name":"Respiratory investigation","volume":"64 2","pages":"101380"},"PeriodicalIF":2.0,"publicationDate":"2026-02-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146137736","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}
In the diagnosis of Mycobacterium tuberculosis, collecting post-bronchoscopy sputum (PBS), as recommended by major guidelines, enhances the microbiological yield; however, evidence for PBS in nontuberculous mycobacterial pulmonary disease (NTM-PD) remains limited. Therefore, we aimed to investigate whether adding PBS increases the yield in suspected NTM-PD.
Methods
This single-center retrospective study included adults who underwent bronchoscopy for suspected NTM-PD between January 2017 and December 2023. After excluding 41 patients due to a lack of PBS submission and 14 with M. tuberculosis, 220 patients were analyzed. All patients underwent bronchial washing or bronchoalveolar lavage, and PBS was collected within 24 h. We compared culture positivity between bronchoscopic specimens and PBS and assessed the incremental yield obtained by adding PBS.
Results
NTM was successfully cultured from bronchoscopic specimens in 71/220 (32.3 %). Before bronchoscopy, sputum cultures were positive in 38/220 (17.3 %), whereas PBS cultures were positive in 72/220 (32.7 %; p < 0.01). PBS was the only positive specimen in 18/220 (8.2 %). Combining bronchoscopic specimens with PBS increased overall culture positivity to 89/220 (40.5 %). The most frequent species were Mycobacterium avium (52/220, 23.6 %) and Mycobacterium intracellulare (32/220, 14.5 %).
Conclusions
Adding PBS following bronchoscopy provided a modest yet clinically meaningful incremental yield in NTM-PD and resembled diagnostic outcomes reported in tuberculosis. PBS collection is a simple and low-burden strategy that enhances the diagnostic information from a single procedure, requiring minimal resources and no additional invasive procedures.
{"title":"Post-bronchoscopy sputum culture improves detection of nontuberculous mycobacterial pulmonary disease: A retrospective Study","authors":"Kohei Yamamoto , Tatsuya Imabayashi , Toshiyuki Tanaka , Kazuki Jinno , Shunya Tanaka , Sayaka Uda , Tatsuya Yuba , Chieko Takumi","doi":"10.1016/j.resinv.2026.101379","DOIUrl":"10.1016/j.resinv.2026.101379","url":null,"abstract":"<div><h3>Background</h3><div>In the diagnosis of <em>Mycobacterium tuberculosis</em>, collecting post-bronchoscopy sputum (PBS), as recommended by major guidelines, enhances the microbiological yield; however, evidence for PBS in nontuberculous mycobacterial pulmonary disease (NTM-PD) remains limited. Therefore, we aimed to investigate whether adding PBS increases the yield in suspected NTM-PD.</div></div><div><h3>Methods</h3><div>This single-center retrospective study included adults who underwent bronchoscopy for suspected NTM-PD between January 2017 and December 2023. After excluding 41 patients due to a lack of PBS submission and 14 with <em>M. tuberculosis</em>, 220 patients were analyzed. All patients underwent bronchial washing or bronchoalveolar lavage, and PBS was collected within 24 h. We compared culture positivity between bronchoscopic specimens and PBS and assessed the incremental yield obtained by adding PBS.</div></div><div><h3>Results</h3><div>NTM was successfully cultured from bronchoscopic specimens in 71/220 (32.3 %). Before bronchoscopy, sputum cultures were positive in 38/220 (17.3 %), whereas PBS cultures were positive in 72/220 (32.7 %; p < 0.01). PBS was the only positive specimen in 18/220 (8.2 %). Combining bronchoscopic specimens with PBS increased overall culture positivity to 89/220 (40.5 %). The most frequent species were <em>Mycobacterium avium</em> (52/220, 23.6 %) and <em>Mycobacterium intracellulare</em> (32/220, 14.5 %).</div></div><div><h3>Conclusions</h3><div>Adding PBS following bronchoscopy provided a modest yet clinically meaningful incremental yield in NTM-PD and resembled diagnostic outcomes reported in tuberculosis. PBS collection is a simple and low-burden strategy that enhances the diagnostic information from a single procedure, requiring minimal resources and no additional invasive procedures.</div></div>","PeriodicalId":20934,"journal":{"name":"Respiratory investigation","volume":"64 2","pages":"Article 101379"},"PeriodicalIF":2.0,"publicationDate":"2026-01-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146078955","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}
Autoimmune pulmonary alveolar proteinosis (aPAP) results from the neutralization of autoantibodies against granulocyte–macrophage colony-stimulating factor, which leads to alveolar macrophage (AM) dysfunction and surfactant accumulation. However, disease progression cannot be solely explained by surfactant overload. This study aimed to investigate whether defective efferocytosis in aPAP contributes to persistent apoptotic debris and accumulation of extracellular double-stranded DNA (dsDNA), which is a candidate biomarker of disease severity.
Methods
We analyzed bronchoalveolar lavage fluid (BALF) samples obtained from 13 patients with aPAP and 13 patients with other interstitial lung diseases (controls). Apoptotic debris was assessed cytologically, extracellular dsDNA was quantified fluorometrically with urea correction, and efferocytosis was evaluated using flow cytometry. Additionally, we analyzed correlations between BALF dsDNA levels and clinical indices.
Results
BALF samples from patients with aPAP contained abundant apoptotic debris and significantly higher dsDNA levels than those from controls. Further, AMs from patients with aPAP showed s markedly reduced uptake apoptotic cells, indicating altered efferocytosis-related processes. Corrected BALF dsDNA levels were negatively correlated with the arterial oxygen pressure to inspired oxygen fraction ratio and percent predicted diffusing capacity of the lung for carbon monoxide.
Conclusions
Altered efferocytosis-related processes in patients with aPAP may promote the accumulation of apoptotic debris and extracellular DNA in the alveolar space. Further, dsDNA levels in BALF strongly reflect impaired gas exchange and provide a biomarker of disease severity. These findings further elucidate the pathogenesis of aPAP and establish extracellular DNA as a promising tool for disease monitoring and therapeutic evaluation.
{"title":"Extracellular DNA in bronchoalveolar lavage fluid as a candidate biomarker of disease severity in autoimmune pulmonary alveolar proteinosis","authors":"Toshiki Kimura, Kosuke Imamura, Chieko Yoshida, Yusuke Tomita, Takuro Sakagami","doi":"10.1016/j.resinv.2026.101372","DOIUrl":"10.1016/j.resinv.2026.101372","url":null,"abstract":"<div><h3>Background</h3><div>Autoimmune pulmonary alveolar proteinosis (aPAP) results from the neutralization of autoantibodies against granulocyte–macrophage colony-stimulating factor, which leads to alveolar macrophage (AM) dysfunction and surfactant accumulation. However, disease progression cannot be solely explained by surfactant overload. This study aimed to investigate whether defective efferocytosis in aPAP contributes to persistent apoptotic debris and accumulation of extracellular double-stranded DNA (dsDNA), which is a candidate biomarker of disease severity.</div></div><div><h3>Methods</h3><div>We analyzed bronchoalveolar lavage fluid (BALF) samples obtained from 13 patients with aPAP and 13 patients with other interstitial lung diseases (controls). Apoptotic debris was assessed cytologically, extracellular dsDNA was quantified fluorometrically with urea correction, and efferocytosis was evaluated using flow cytometry. Additionally, we analyzed correlations between BALF dsDNA levels and clinical indices.</div></div><div><h3>Results</h3><div>BALF samples from patients with aPAP contained abundant apoptotic debris and significantly higher dsDNA levels than those from controls. Further, AMs from patients with aPAP showed s markedly reduced uptake apoptotic cells, indicating altered efferocytosis-related processes. Corrected BALF dsDNA levels were negatively correlated with the arterial oxygen pressure to inspired oxygen fraction ratio and percent predicted diffusing capacity of the lung for carbon monoxide.</div></div><div><h3>Conclusions</h3><div>Altered efferocytosis-related processes in patients with aPAP may promote the accumulation of apoptotic debris and extracellular DNA in the alveolar space. Further, dsDNA levels in BALF strongly reflect impaired gas exchange and provide a biomarker of disease severity. These findings further elucidate the pathogenesis of aPAP and establish extracellular DNA as a promising tool for disease monitoring and therapeutic evaluation.</div></div>","PeriodicalId":20934,"journal":{"name":"Respiratory investigation","volume":"64 2","pages":"Article 101372"},"PeriodicalIF":2.0,"publicationDate":"2026-01-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146078954","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}
Interstitial lung abnormalities (ILA) are CT-detected interstitial abnormalities that may represent early-stage interstitial lung disease (ILD). However, histopathologic correlations remain limited, with previous studies reporting conflicting results. In 2025, the American Thoracic Society (ATS) established criteria defining the boundary between ILA and ILD. This study aimed to investigate the histopathological spectrum of Fleischner-defined ILA, correlate findings with CT features and outcomes, and reclassify cases according to 2025 ATS criteria.
Methods
This retrospective study analyzed 30 consecutive patients who underwent surgical lung biopsy between January 2010 and December 2021. All cases had ILA per Fleischner Society criteria and were reclassified using 2025 ATS criteria. Three pulmonary pathologists evaluated dominant and co-existing histopathological patterns. Two chest radiologists independently assessed CT findings. Overall survival was compared between usual interstitial pneumonia (UIP)-related and non-UIP-related groups using Kaplan-Meier analysis.
Results
Twenty patients (66.7 %) were men; mean age was 63.6 ± 6.5 years; 27 (90 %) had fibrotic ILA. The most common dominant pattern was UIP (43.3 %), followed by nonspecific interstitial pneumonia (NSIP) and bronchiolocentric interstitial pneumonia (BIP) (20 % each). Overall, 96.7 % of cases met 2025 ATS criteria for ILD (subclinical ILD). On CT, all UIP and NSIP cases showed reticular opacity and traction bronchiectasis, while BIP typically exhibited branching linear opacities. During median follow-up of 92.1 months, overall survival did not differ significantly between UIP-related and non-UIP-related groups (p = 0.595).
Conclusions
This study of Fleischner-defined ILA, predominantly reclassified as subclinical ILD by 2025 ATS criteria, demonstrates diverse histopathological patterns beyond UIP, including BIP, highlighting pathological heterogeneity.
{"title":"Diverse histopathological patterns in Fleischner-defined interstitial lung abnormalities: Radiologic-Pathologic correlation and reclassification using 2025 American Thoracic Society statement","authors":"Taiki Fukuda , Kaori Ishida , Tomonori Tanaka , Kensuke Kataoka , Reoto Takei , Yuki Ko , Yusei Nakamura , Mikiko Hashisako , Hiromitsu Sumikawa , Ryoko Egashira , Junya Tominaga , Hiroya Ojiri , Hiroto Hatabu , Tomoki Kimura , Yasuhiro Kondoh , Junya Fukuoka , Takeshi Johkoh","doi":"10.1016/j.resinv.2026.101374","DOIUrl":"10.1016/j.resinv.2026.101374","url":null,"abstract":"<div><h3>Background</h3><div>Interstitial lung abnormalities (ILA) are CT-detected interstitial abnormalities that may represent early-stage interstitial lung disease (ILD). However, histopathologic correlations remain limited, with previous studies reporting conflicting results. In 2025, the American Thoracic Society (ATS) established criteria defining the boundary between ILA and ILD. This study aimed to investigate the histopathological spectrum of Fleischner-defined ILA, correlate findings with CT features and outcomes, and reclassify cases according to 2025 ATS criteria.</div></div><div><h3>Methods</h3><div>This retrospective study analyzed 30 consecutive patients who underwent surgical lung biopsy between January 2010 and December 2021. All cases had ILA per Fleischner Society criteria and were reclassified using 2025 ATS criteria. Three pulmonary pathologists evaluated dominant and co-existing histopathological patterns. Two chest radiologists independently assessed CT findings. Overall survival was compared between usual interstitial pneumonia (UIP)-related and non-UIP-related groups using Kaplan-Meier analysis.</div></div><div><h3>Results</h3><div>Twenty patients (66.7 %) were men; mean age was 63.6 ± 6.5 years; 27 (90 %) had fibrotic ILA. The most common dominant pattern was UIP (43.3 %), followed by nonspecific interstitial pneumonia (NSIP) and bronchiolocentric interstitial pneumonia (BIP) (20 % each). Overall, 96.7 % of cases met 2025 ATS criteria for ILD (subclinical ILD). On CT, all UIP and NSIP cases showed reticular opacity and traction bronchiectasis, while BIP typically exhibited branching linear opacities. During median follow-up of 92.1 months, overall survival did not differ significantly between UIP-related and non-UIP-related groups (<em>p</em> = 0.595).</div></div><div><h3>Conclusions</h3><div>This study of Fleischner-defined ILA, predominantly reclassified as subclinical ILD by 2025 ATS criteria, demonstrates diverse histopathological patterns beyond UIP, including BIP, highlighting pathological heterogeneity.</div></div>","PeriodicalId":20934,"journal":{"name":"Respiratory investigation","volume":"64 2","pages":"Article 101374"},"PeriodicalIF":2.0,"publicationDate":"2026-01-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146038518","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}
Skeletal muscle dysfunction frequently accompanies chronic obstructive pulmonary disease (COPD). Handgrip strength (HGS) is a simple, reliable measure of muscle strength. This study aimed to evaluate its prognostic significance in COPD.
Methods
We conducted a prospective observational study in a cohort of Japanese patients with COPD, stratified by HGS. Patient characteristics, exacerbations, and mortality were evaluated over 5 years. Low HGS was defined as <28 kg in men and <18 kg in women.
Results
Among 300 patients, 89 (29.7 %) had low HGS. Compared with patients with normal HGS, these patients had a lower body mass index, worse pulmonary function, more severe dyspnea, poorer health status, and reduced physical activity. Exacerbation rates were similar between the groups. Low HGS was associated with an increased risk of all-cause mortality (hazard ratio, 1.79; 95 % confidence interval, 1.03–3.13).
Conclusions
Low HGS was associated with adverse clinical outcomes in Japanese patients with COPD.
{"title":"Handgrip strength in Japanese patients with chronic obstructive pulmonary disease: a prospective cohort study","authors":"Seiichi Kobayashi, Manabu Ono, Masatsugu Ishida, Hikari Satoh, Masakazu Hanagama, Koji Okutomo, Masaru Yanai","doi":"10.1016/j.resinv.2026.101377","DOIUrl":"10.1016/j.resinv.2026.101377","url":null,"abstract":"<div><h3>Background</h3><div>Skeletal muscle dysfunction frequently accompanies chronic obstructive pulmonary disease (COPD). Handgrip strength (HGS) is a simple, reliable measure of muscle strength. This study aimed to evaluate its prognostic significance in COPD.</div></div><div><h3>Methods</h3><div>We conducted a prospective observational study in a cohort of Japanese patients with COPD, stratified by HGS. Patient characteristics, exacerbations, and mortality were evaluated over 5 years. Low HGS was defined as <28 kg in men and <18 kg in women.</div></div><div><h3>Results</h3><div>Among 300 patients, 89 (29.7 %) had low HGS. Compared with patients with normal HGS, these patients had a lower body mass index, worse pulmonary function, more severe dyspnea, poorer health status, and reduced physical activity. Exacerbation rates were similar between the groups. Low HGS was associated with an increased risk of all-cause mortality (hazard ratio, 1.79; 95 % confidence interval, 1.03–3.13).</div></div><div><h3>Conclusions</h3><div>Low HGS was associated with adverse clinical outcomes in Japanese patients with COPD.</div></div>","PeriodicalId":20934,"journal":{"name":"Respiratory investigation","volume":"64 2","pages":"Article 101377"},"PeriodicalIF":2.0,"publicationDate":"2026-01-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146038519","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}
Approximately half of primary ciliary dyskinesia (PCD) patients in Japan carry a large homozygous deletion encompassing exons 1–4 of DRC1 gene. However, the clinical manifestations of PCD patients with DRC1 variants remain poorly characterized.
Methods
We conducted a multicenter retrospective cohort study at 12 hospitals across Japan. Patients with DRC1 variants were included, and their clinical characteristics, disease severity, and radiological features were compared with those of patients with outer dynein arm (ODA) defects.
Results
A total of 43 patients with DRC1 variants and 21 with ODA defects were included. The median age at PCD diagnosis was 27 years (IQR: 17–41) for patients with DRC1 variants and 26 years (IQR: 8–31) for those with ODA defects. The median PICADAR score was significantly lower in patients with DRC1 variants than those with ODA defects (4 vs. 8, p < 0.001). The radiological severity and distribution of bronchiectasis did not differ between the two groups, while the median mucous plugging score (bronchiolitis/tree-in-bud) was significantly higher in patients with DRC1 variants (5, IQR: 4–6 vs. 3, IQR: 2–4, p = 0.044). In patients with DRC1 variants, the FEV1 z score was negatively correlated with age (r = −0.37, p = 0.028), and the modified Reiff score was positively correlated with age (r = 0.47, p = 0.010).
Conclusions
Although the sensitivity of the PICADAR score was low in these patients, most clinical and radiological features of DRC1-related PCD were relatively typical of PCD. Given that DRC1-related PCD appears to worsen with age, early diagnosis and timely intervention are crucial.
背景:在日本,大约一半的原发性纤毛运动障碍(PCD)患者携带DRC1基因外显子1-4的大量纯合缺失。然而,伴有DRC1变异的PCD患者的临床表现仍不清楚。方法:我们在日本12家医院进行了一项多中心回顾性队列研究。纳入DRC1变异患者,并将其临床特征、疾病严重程度和影像学特征与外动力蛋白臂(ODA)缺陷患者进行比较。结果:共纳入43例DRC1变异患者和21例ODA缺陷患者。DRC1变异患者诊断PCD时的中位年龄为27岁(IQR: 17-41), ODA缺陷患者诊断PCD时的中位年龄为26岁(IQR: 8-31)。DRC1变异患者PICADAR评分中位数明显低于ODA缺陷患者(4比8,p 1 z评分与年龄呈负相关(r = -0.37, p = 0.028),修改后的Reiff评分与年龄呈正相关(r = 0.47, p = 0.010)。结论:尽管PICADAR评分在这些患者中敏感性较低,但drc1相关PCD的大多数临床和影像学特征都是相对典型的PCD。鉴于drc1相关PCD似乎随着年龄的增长而恶化,早期诊断和及时干预至关重要。
{"title":"Clinical characteristics and severity of primary ciliary dyskinesia caused by large homozygous deletion including exons 1–4 of DRC1: A multicenter retrospective cohort study","authors":"Masashi Ito , Atsuko Nakano , Yukiko Arimoto , Mitsuko Kondo , Yusuke Matsuda , Miki Abo , Takashi Kido , Masashi Morishita , Takeshige Honma , Hisashi Nishimori , Yuzaburo Inoue , Keisuke Iwamoto , Yuichiro Hashida , Kazuhiko Takeuchi , Miyabayashi Akiko , Keiko Wakabayashi , Hiroyuki Yamada , Minako Hijikata , Naoto Keicho , Kozo Morimoto","doi":"10.1016/j.resinv.2026.101376","DOIUrl":"10.1016/j.resinv.2026.101376","url":null,"abstract":"<div><h3>Background</h3><div>Approximately half of primary ciliary dyskinesia (PCD) patients in Japan carry a large homozygous deletion encompassing exons 1–4 of <em>DRC1</em> gene. However, the clinical manifestations of PCD patients with <em>DRC1</em> variants remain poorly characterized.</div></div><div><h3>Methods</h3><div>We conducted a multicenter retrospective cohort study at 12 hospitals across Japan. Patients with <em>DRC1</em> variants were included, and their clinical characteristics, disease severity, and radiological features were compared with those of patients with outer dynein arm (ODA) defects.</div></div><div><h3>Results</h3><div>A total of 43 patients with <em>DRC1</em> variants and 21 with ODA defects were included. The median age at PCD diagnosis was 27 years (IQR: 17–41) for patients with <em>DRC1</em> variants and 26 years (IQR: 8–31) for those with ODA defects. The median PICADAR score was significantly lower in patients with <em>DRC1</em> variants than those with ODA defects (4 vs. 8, p < 0.001). The radiological severity and distribution of bronchiectasis did not differ between the two groups, while the median mucous plugging score (bronchiolitis/tree-in-bud) was significantly higher in patients with <em>DRC1</em> variants (5, IQR: 4–6 vs. 3, IQR: 2–4, p = 0.044). In patients with <em>DRC1</em> variants, the FEV<sub>1</sub> z score was negatively correlated with age (r = −0.37, p = 0.028), and the modified Reiff score was positively correlated with age (r = 0.47, p = 0.010).</div></div><div><h3>Conclusions</h3><div>Although the sensitivity of the PICADAR score was low in these patients, most clinical and radiological features of <em>DRC1</em>-related PCD were relatively typical of PCD. Given that <em>DRC1</em>-related PCD appears to worsen with age, early diagnosis and timely intervention are crucial.</div></div>","PeriodicalId":20934,"journal":{"name":"Respiratory investigation","volume":"64 2","pages":"Article 101376"},"PeriodicalIF":2.0,"publicationDate":"2026-01-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146030642","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 : 2026-01-20DOI: 10.1016/j.resinv.2026.101373
A. Taha , M.S. Muneer , A. Kalra , M. Muelly , J. Reicher
Background
Several limitations hinder the effectiveness of human-based lung cancer screening (LCS): high false-positive rates leading to unnecessary follow-up imaging, procedures, and surgeries; inter-reader variability; inconsistent Lung-RADS adherence; and fatigue-related diagnostic errors. Additionally, most artificial intelligence (AI) models address only one task (nodule detection or risk stratification) and require manual image processing, which is time-consuming and costly. We developed Bronchosolve, a closed-loop, fully-automated software that processes scans without manual input, aiming to improve consistency, accuracy, and throughput in LCS.
Methods
The software integrates pre-processing, analysis, and result generation, using a deep-learning convolutional neural network (CNN) for pulmonary nodule triaging. Inputs were full chest CT scans in DICOM format, without clinical or demographic data. Automated steps included: 1) optimal CT series selection, 2) normalization and preprocessing, 3) AI-based detection and classification of suspicious nodules, and 4) report generation. The model was trained on a multi-center high-prevalence set of 2358 cases (malignant and benign nodules). Validation used a U.S.-based, multi-site cohort (n = 184; 8 sites). Positive cases were biopsy-confirmed within 1 year; negatives had biopsy or ≥2-year follow-up.
Results
All cases completed automatically (100 % success). Median age was 62.5 years (IQR 58.5–66.5); 45 % former smokers, 55 % current smokers, and 40 % female. The model achieved an AUC of 0.898 [0.851–0.940], outperforming Lung-RADS (pAUC 0.669) and the Brock model (AUC 0.783). Sensitivity was 83.6 %; specificity was 86.3 %. Performance remained consistent across scanner types and slice thicknesses.
Conclusions
Bronchosolve enables accurate, fully-automated risk classification of lung nodules and may enhance non-invasive diagnostic workflows.
{"title":"Performance validation of a closed loop fully automated AI model for lung nodule stratification in screening cases","authors":"A. Taha , M.S. Muneer , A. Kalra , M. Muelly , J. Reicher","doi":"10.1016/j.resinv.2026.101373","DOIUrl":"10.1016/j.resinv.2026.101373","url":null,"abstract":"<div><h3>Background</h3><div>Several limitations hinder the effectiveness of human-based lung cancer screening (LCS): high false-positive rates leading to unnecessary follow-up imaging, procedures, and surgeries; inter-reader variability; inconsistent Lung-RADS adherence; and fatigue-related diagnostic errors. Additionally, most artificial intelligence (AI) models address only one task (nodule detection or risk stratification) and require manual image processing, which is time-consuming and costly. We developed Bronchosolve, a closed-loop, fully-automated software that processes scans without manual input, aiming to improve consistency, accuracy, and throughput in LCS.</div></div><div><h3>Methods</h3><div>The software integrates pre-processing, analysis, and result generation, using a deep-learning convolutional neural network (CNN) for pulmonary nodule triaging. Inputs were full chest CT scans in DICOM format, without clinical or demographic data. Automated steps included: 1) optimal CT series selection, 2) normalization and preprocessing, 3) AI-based detection and classification of suspicious nodules, and 4) report generation. The model was trained on a multi-center high-prevalence set of 2358 cases (malignant and benign nodules). Validation used a U.S.-based, multi-site cohort (n = 184; 8 sites). Positive cases were biopsy-confirmed within 1 year; negatives had biopsy or ≥2-year follow-up.</div></div><div><h3>Results</h3><div>All cases completed automatically (100 % success). Median age was 62.5 years (IQR 58.5–66.5); 45 % former smokers, 55 % current smokers, and 40 % female. The model achieved an AUC of 0.898 [0.851–0.940], outperforming Lung-RADS (pAUC 0.669) and the Brock model (AUC 0.783). Sensitivity was 83.6 %; specificity was 86.3 %. Performance remained consistent across scanner types and slice thicknesses.</div></div><div><h3>Conclusions</h3><div>Bronchosolve enables accurate, fully-automated risk classification of lung nodules and may enhance non-invasive diagnostic workflows.</div></div>","PeriodicalId":20934,"journal":{"name":"Respiratory investigation","volume":"64 2","pages":"Article 101373"},"PeriodicalIF":2.0,"publicationDate":"2026-01-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146019445","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 : 2026-01-20DOI: 10.1016/j.resinv.2026.101375
Doppo Fukui , Daisuke Morinaga , Jun Sakakibara-Konishi , Yukiko Yoshida , Masahiro Kashima , Shotaro Ito , Megumi Furuta , Yuta Takashima , Zenichi Tanei , Satoshi Konno
A comprehensive pathological evaluation is useful for diagnosing synchronous multiple primary lung cancer (sMPLC). However, a consensus regarding treatment for sMPLC with different driver mutations is lacking. We present a case of sMPLC harboring an epidermal growth factor receptor (EGFR) mutation and anaplastic lymphoma kinase (ALK) fusion gene. For the advanced EGFR-positive tumor, osimertinib plus chemotherapy was initiated, the latter also covering ALK-positive tumor. A marked response and slight reduction occurred in the EGFR-positive and ALK-positive tumors, respectively. Surgical resection of the ALK-positive tumor achieved negative margins. Targeted therapy with chemotherapy may effectively treat sMPLC with different driver mutations.
{"title":"A case of synchronous multiple primary lung cancers each harboring an EGFR mutation or an ALK fusion gene alone that responded to osimertinib with chemotherapy","authors":"Doppo Fukui , Daisuke Morinaga , Jun Sakakibara-Konishi , Yukiko Yoshida , Masahiro Kashima , Shotaro Ito , Megumi Furuta , Yuta Takashima , Zenichi Tanei , Satoshi Konno","doi":"10.1016/j.resinv.2026.101375","DOIUrl":"10.1016/j.resinv.2026.101375","url":null,"abstract":"<div><div>A comprehensive pathological evaluation is useful for diagnosing synchronous multiple primary lung cancer (sMPLC). However, a consensus regarding treatment for sMPLC with different driver mutations is lacking. We present a case of sMPLC harboring an epidermal growth factor receptor (EGFR) mutation and anaplastic lymphoma kinase (ALK) fusion gene. For the advanced EGFR-positive tumor, osimertinib plus chemotherapy was initiated, the latter also covering ALK-positive tumor. A marked response and slight reduction occurred in the EGFR-positive and ALK-positive tumors, respectively. Surgical resection of the ALK-positive tumor achieved negative margins. Targeted therapy with chemotherapy may effectively treat sMPLC with different driver mutations.</div></div>","PeriodicalId":20934,"journal":{"name":"Respiratory investigation","volume":"64 2","pages":"Article 101375"},"PeriodicalIF":2.0,"publicationDate":"2026-01-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146019476","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 exacerbation (AE) is a critical prognostic event in interstitial lung diseases (ILDs), initially recognized in idiopathic pulmonary fibrosis (IPF). Current AE diagnostic criteria do not account for oxygenation deterioration, and evidence of new computed tomography (CT) findings alone is insufficient to diagnose AE-ILD. We investigated a CT-based criterion for distinguishing AE from acute deterioration (AD)-ILD and assessed the prognostic significance of oxygenation deterioration in AE-ILD.
Methods
Patients with AD-ILD, defined by worsening shortness of breath and new lung abnormalities within 1 month, were prospectively enrolled. AD-ILD included both AE-ILD and other acute conditions, non-AE-AD-ILD. Oxygenation deterioration was assessed using modified Blancal criteria. CT-based AE criteria were developed through receiver operating characteristic (ROC) curve and multivariate logistic regression analyses based on CT findings suggestive of AE.
Results
Overall, 394 patients were analyzed, including 303 diagnosed with AE and 91 with non-AE-AD-ILD. The underlying ILDs included IPF (n = 177) and non-IPF ILDs (n = 217). The presence of at least two of three CT-based parameters, including bilateral shadows, ground-glass opacity predominance, and diffuse pattern, was identified as predictive of AE diagnosis in AD-ILDs using logistic and ROC analyses. Ninety-day survival was significantly lower in patients with AE-ILD with oxygenation deterioration than in those without deterioration and in patients with non-AE-AD-ILDs. Oxygenation deterioration independently predicted poor survival in multivariate Cox proportional hazard regression analysis.
Conclusions
The developed CT-based AE criteria effectively predicted AE and oxygenation deterioration in AD-ILD. Additionally, oxygenation deterioration predicted worse survival in AE-ILD.
This study was registered with the University Hospital Medical Information Network (UMIN000027844; registered 1 July 2017).
背景:急性加重(AE)是间质性肺疾病(ILDs)的一个关键预后事件,最初在特发性肺纤维化(IPF)中得到认可。目前的AE诊断标准没有考虑到氧合恶化,仅凭新的计算机断层扫描(CT)发现的证据不足以诊断AE- ild。我们研究了一种基于ct的区分AE与急性恶化(AD)-ILD的标准,并评估了AE-ILD中氧合恶化的预后意义。方法:前瞻性纳入AD-ILD患者,其定义为1个月内呼吸短促加重和新的肺部异常。AD-ILD包括AE-ILD和其他急性疾病,非ae -AD-ILD。采用改良Blancal标准评估氧合恶化。根据提示AE的CT表现,通过受试者工作特征(ROC)曲线和多因素logistic回归分析,制定基于CT的AE标准。结果:总共分析了394例患者,其中303例诊断为AE, 91例诊断为非AE- ad - ild。基础ild包括IPF (n = 177)和非IPF (n = 217)。通过logistic和ROC分析,三种基于ct的参数中至少两种的存在,包括双侧阴影、磨玻璃不透明优势和弥漫性模式,被确定为ad - ild声发射诊断的预测指标。伴氧合恶化的AE-ILD患者的90天生存率明显低于无氧合恶化患者和非ae - ad - ild患者。在多变量Cox比例风险回归分析中,氧合恶化独立预测生存不良。结论:建立的基于ct的AE标准可有效预测AD-ILD的AE和氧合恶化。此外,氧合恶化预示着AE-ILD患者更差的生存。本研究已在大学医院医疗信息网络注册(UMIN000027844,注册日期为2017年7月1日)。
{"title":"Oxygenation deterioration and computed tomography findings in acute exacerbation of interstitial lung diseases: a prospective observational study","authors":"Toru Arai , Junji Otsuka , Shinobu Akagawa , Yoriyuki Murata , Hidetoshi Yanai , Ryoji Ito , Takuo Shibayama , Toshihiko Ii , Toshiyuki Kita , Yoshifusa Koreeda , Takayasu Watanabe , Isoko Owan , Atsuko Hara , Kentaro Wakamatsu , Akihiro Yoshii , Shiro Ohshima , Shin Sasaki , Susumu Oguri , Naoki Arai , Eiji Yatsuyanagi , Hiromitsu Sumikawa","doi":"10.1016/j.resinv.2025.101361","DOIUrl":"10.1016/j.resinv.2025.101361","url":null,"abstract":"<div><h3>Background</h3><div>Acute exacerbation (AE) is a critical prognostic event in interstitial lung diseases (ILDs), initially recognized in idiopathic pulmonary fibrosis (IPF). Current AE diagnostic criteria do not account for oxygenation deterioration, and evidence of new computed tomography (CT) findings alone is insufficient to diagnose AE-ILD. We investigated a CT-based criterion for distinguishing AE from acute deterioration (AD)-ILD and assessed the prognostic significance of oxygenation deterioration in AE-ILD.</div></div><div><h3>Methods</h3><div>Patients with AD-ILD, defined by worsening shortness of breath and new lung abnormalities within 1 month, were prospectively enrolled. AD-ILD included both AE-ILD and other acute conditions, non-AE-AD-ILD. Oxygenation deterioration was assessed using modified Blancal criteria. CT-based AE criteria were developed through receiver operating characteristic (ROC) curve and multivariate logistic regression analyses based on CT findings suggestive of AE.</div></div><div><h3>Results</h3><div>Overall, 394 patients were analyzed, including 303 diagnosed with AE and 91 with non-AE-AD-ILD. The underlying ILDs included IPF (n = 177) and non-IPF ILDs (n = 217). The presence of at least two of three CT-based parameters, including bilateral shadows, ground-glass opacity predominance, and diffuse pattern, was identified as predictive of AE diagnosis in AD-ILDs using logistic and ROC analyses. Ninety-day survival was significantly lower in patients with AE-ILD with oxygenation deterioration than in those without deterioration and in patients with non-AE-AD-ILDs. Oxygenation deterioration independently predicted poor survival in multivariate Cox proportional hazard regression analysis.</div></div><div><h3>Conclusions</h3><div>The developed CT-based AE criteria effectively predicted AE and oxygenation deterioration in AD-ILD. Additionally, oxygenation deterioration predicted worse survival in AE-ILD.</div><div>This study was registered with the University Hospital Medical Information Network (UMIN000027844; registered 1 July 2017).</div></div>","PeriodicalId":20934,"journal":{"name":"Respiratory investigation","volume":"64 2","pages":"Article 101361"},"PeriodicalIF":2.0,"publicationDate":"2026-01-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146011672","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}