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Clinical characteristics and systemic treatment in patients with elderly-onset sarcoidosis: A retrospective single-centre study in Japan. 老年结节病患者的临床特征和全身治疗:日本的一项回顾性单中心研究。
IF 2 Q2 RESPIRATORY SYSTEM Pub Date : 2026-02-06 DOI: 10.1016/j.resinv.2026.101381
Hitokazu Tsukao, Michiru Sawahata, Yoshitaka Yamanouchi, Noritaka Sakamoto, Masayuki Nakayama, Koichi Hagiwara, Makoto Maemondo

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。
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引用次数: 0
Effects of olfactory stimulation with L-menthol on sensations of discomfort on exertion in patients with chronic lung diseases. l -薄荷醇嗅觉刺激对慢性肺病患者运动不应感的影响。
IF 2 Q2 RESPIRATORY SYSTEM Pub Date : 2026-02-06 DOI: 10.1016/j.resinv.2026.101380
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

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).

背景:用l -薄荷醇刺激嗅觉可以减轻某些不适感,包括呼吸困难。慢性阻塞性肺疾病(COPD)和特发性间质性肺炎(IIPs)具有不同的生理特征,可引起不同的呼吸困难的定性感觉。因此,l -薄荷醇吸入对呼吸困难的定性影响可能在这些慢性肺部疾病(CLDs)之间有所不同。本研究旨在确定CLDs患者呼吸困难的哪些方面受到l -薄荷醇嗅觉刺激的影响。方法:34例稳定期COPD或IIPs患者(各17例)在两种条件下进行6分钟步行试验(6MWT):单独佩戴外科口罩(对照组)或佩戴带有薄荷油香气贴纸(0.105 ml,含30% l -薄荷醇)的口罩。采用改进的Borg量表、多维呼吸困难量表(MDP)和呼吸困难语言问卷(LDQ)来评估终点。选择6MWT诱导有和不含l -薄荷醇的呼吸困难。结果:在COPD组中,l -薄荷醇显著改善了MDP的精神呼吸努力,显著改善了LDQ的快速、喘息和空气饥饿。在IIPs组,l -薄荷醇仅改善MDP呼吸急促维度。l -薄荷醇与对照组在6MWT前后的改良Borg量表均无显著差异。结论:l -薄荷醇的嗅觉刺激选择性地改善了COPD和IIPs患者呼吸困难的各个方面,可能反映了每种疾病的病理生理和严重程度的差异。试验注册:日本临床试验注册中心(jRCTs031200400)。
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引用次数: 0
Post-bronchoscopy sputum culture improves detection of nontuberculous mycobacterial pulmonary disease: A retrospective Study 支气管镜检查后痰培养提高非结核性分枝杆菌肺病的检出率:一项回顾性研究
IF 2 Q2 RESPIRATORY SYSTEM Pub Date : 2026-01-30 DOI: 10.1016/j.resinv.2026.101379
Kohei Yamamoto , Tatsuya Imabayashi , Toshiyuki Tanaka , Kazuki Jinno , Shunya Tanaka , Sayaka Uda , Tatsuya Yuba , Chieko Takumi

Background

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.
背景:在诊断结核分枝杆菌时,根据主要指南的建议,收集支气管镜检查后痰液(PBS)可提高微生物产量;然而,PBS治疗非结核性分枝杆菌肺病(NTM-PD)的证据仍然有限。因此,我们的目的是研究添加PBS是否能提高可疑NTM-PD的产量。方法该单中心回顾性研究纳入了2017年1月至2023年12月期间因疑似NTM-PD接受支气管镜检查的成年人。在排除了41名由于缺乏PBS提交的患者和14名结核分枝杆菌患者后,对220名患者进行了分析。所有患者均进行支气管清洗或支气管肺泡灌洗,并在24 h内收集PBS。我们比较支气管镜标本与PBS的培养阳性,并评估添加PBS后获得的增量产量。结果71/220(32.3%)的支气管镜标本成功培养sntm。支气管镜检查前,38/220痰培养阳性(17.3%),72/220 PBS培养阳性(32.7%;p < 0.01)。PBS是18/220中唯一阳性标本(8.2%)。将支气管镜标本与PBS结合使总体培养阳性增加到89/220(40.5%)。最常见的菌种为鸟分枝杆菌(52/220,23.6%)和胞内分枝杆菌(32/220,14.5%)。结论:支气管镜检查后添加PBS可为NTM-PD提供适度但有临床意义的增量产率,与结核病的诊断结果相似。PBS收集是一种简单且负担低的策略,可以从单个手术中增强诊断信息,所需资源最少,不需要额外的侵入性手术。
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引用次数: 0
Extracellular DNA in bronchoalveolar lavage fluid as a candidate biomarker of disease severity in autoimmune pulmonary alveolar proteinosis 支气管肺泡灌洗液中的细胞外DNA作为自身免疫性肺泡蛋白沉积症疾病严重程度的候选生物标志物
IF 2 Q2 RESPIRATORY SYSTEM Pub Date : 2026-01-30 DOI: 10.1016/j.resinv.2026.101372
Toshiki Kimura, Kosuke Imamura, Chieko Yoshida, Yusuke Tomita, Takuro Sakagami

Background

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.
自身免疫性肺泡蛋白沉积症(aPAP)是由于粒细胞-巨噬细胞集落刺激因子自身抗体的中和,导致肺泡巨噬细胞(AM)功能障碍和表面活性剂积累。然而,疾病进展不能仅仅由表面活性剂超载来解释。本研究旨在探讨aPAP中有缺陷的efferocytosis是否有助于持续的凋亡碎片和细胞外双链DNA (dsDNA)的积累,dsDNA是疾病严重程度的候选生物标志物。方法分析13例aPAP患者和13例其他间质性肺疾病患者(对照组)的支气管肺泡灌洗液(BALF)样本。凋亡碎片用细胞学方法评估,细胞外dsDNA用尿素校正荧光定量,efferocytosis用流式细胞术评估。此外,我们分析了BALF dsDNA水平与临床指标的相关性。结果aPAP患者的balf样本中含有丰富的凋亡碎片,dsDNA水平明显高于对照组。此外,来自aPAP患者的AMs显示摄取凋亡细胞明显减少,表明efferocytic相关过程发生了改变。校正后的BALF dsDNA水平与动脉氧压与吸入氧分数比和预测肺一氧化碳弥散能力的百分比呈负相关。结论aPAP患者泡沫化相关过程的改变可能促进肺泡间隙凋亡碎片和细胞外DNA的积累。此外,BALF中的dsDNA水平强烈反映了气体交换受损,并提供了疾病严重程度的生物标志物。这些发现进一步阐明了aPAP的发病机制,并建立了细胞外DNA作为疾病监测和治疗评估的有前途的工具。
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引用次数: 0
Diverse histopathological patterns in Fleischner-defined interstitial lung abnormalities: Radiologic-Pathologic correlation and reclassification using 2025 American Thoracic Society statement fleischner定义的间质性肺异常的不同组织病理学模式:放射学-病理相关性和使用2025年美国胸科学会声明的重新分类
IF 2 Q2 RESPIRATORY SYSTEM Pub Date : 2026-01-24 DOI: 10.1016/j.resinv.2026.101374
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

Background

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.
背景间质性肺异常(ILA)是ct检测到的间质性异常,可能代表早期间质性肺疾病(ILD)。然而,组织病理学相关性仍然有限,先前的研究报告了相互矛盾的结果。2025年,美国胸科学会(ATS)建立了定义ILA和ILD界限的标准。本研究旨在探讨fleischner定义的ILA的组织病理学谱,将其与CT特征和结果相关联,并根据2025 ATS标准对病例进行重新分类。方法本回顾性研究分析了2010年1月至2021年12月期间连续30例接受手术肺活检的患者。根据Fleischner协会标准,所有病例均有ILA,并使用2025 ATS标准重新分类。三位肺部病理学家评估了显性和共存的组织病理学模式。两名胸部放射科医生独立评估了CT表现。采用Kaplan-Meier分析比较常规间质性肺炎(UIP)相关组和非UIP相关组的总生存率。结果男性20例(66.7%);平均年龄63.6±6.5岁;27例(90%)为纤维化性ILA。最常见的主要类型是UIP(43.3%),其次是非特异性间质性肺炎(NSIP)和细支气管中心性间质性肺炎(BIP)(各占20%)。总体而言,96.7%的病例符合2025年ATS的ILD(亚临床ILD)标准。在CT上,所有UIP和NSIP病例均表现为网状混浊和牵引性支气管扩张,而BIP典型表现为分支性线状混浊。在中位92.1个月的随访中,uip相关组和非uip相关组的总生存率无显著差异(p = 0.595)。根据2025年ATS标准,fleischner定义的ILA主要被重新归类为亚临床ILD,该研究显示了包括BIP在内的多种组织病理学模式,突出了病理异质性。
{"title":"Diverse histopathological patterns in Fleischner-defined interstitial lung abnormalities: Radiologic-Pathologic correlation and reclassification using 2025 American Thoracic Society statement","authors":"Taiki Fukuda ,&nbsp;Kaori Ishida ,&nbsp;Tomonori Tanaka ,&nbsp;Kensuke Kataoka ,&nbsp;Reoto Takei ,&nbsp;Yuki Ko ,&nbsp;Yusei Nakamura ,&nbsp;Mikiko Hashisako ,&nbsp;Hiromitsu Sumikawa ,&nbsp;Ryoko Egashira ,&nbsp;Junya Tominaga ,&nbsp;Hiroya Ojiri ,&nbsp;Hiroto Hatabu ,&nbsp;Tomoki Kimura ,&nbsp;Yasuhiro Kondoh ,&nbsp;Junya Fukuoka ,&nbsp;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}
引用次数: 0
Handgrip strength in Japanese patients with chronic obstructive pulmonary disease: a prospective cohort study 日本慢性阻塞性肺疾病患者的握力:一项前瞻性队列研究
IF 2 Q2 RESPIRATORY SYSTEM Pub Date : 2026-01-24 DOI: 10.1016/j.resinv.2026.101377
Seiichi Kobayashi, Manabu Ono, Masatsugu Ishida, Hikari Satoh, Masakazu Hanagama, Koji Okutomo, Masaru Yanai

Background

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.
背景:骨骼肌功能障碍常伴随慢性阻塞性肺疾病(COPD)。握力(HGS)是一种简单、可靠的肌肉力量测量方法。本研究旨在评价其在COPD中的预后意义。方法:我们在日本COPD患者队列中进行了一项前瞻性观察性研究,按HGS分层。在5年内评估患者的特征、恶化情况和死亡率。低HGS的定义是男性为28公斤,女性为18公斤。结果300例患者中低HGS 89例(29.7%)。与HGS正常的患者相比,这些患者的体重指数较低,肺功能较差,呼吸困难更严重,健康状况较差,体力活动减少。两组间的恶化率相似。低HGS与全因死亡风险增加相关(危险比为1.79;95%可信区间为1.03-3.13)。结论慢速HGS与日本COPD患者的不良临床结局相关。
{"title":"Handgrip strength in Japanese patients with chronic obstructive pulmonary disease: a prospective cohort study","authors":"Seiichi Kobayashi,&nbsp;Manabu Ono,&nbsp;Masatsugu Ishida,&nbsp;Hikari Satoh,&nbsp;Masakazu Hanagama,&nbsp;Koji Okutomo,&nbsp;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 &lt;28 kg in men and &lt;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}
引用次数: 0
Clinical characteristics and severity of primary ciliary dyskinesia caused by large homozygous deletion including exons 1–4 of DRC1: A multicenter retrospective cohort study 包括DRC1外显子1-4在内的大纯合缺失引起的原发性纤毛运动障碍的临床特征和严重程度:一项多中心回顾性队列研究
IF 2 Q2 RESPIRATORY SYSTEM Pub Date : 2026-01-21 DOI: 10.1016/j.resinv.2026.101376
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

Background

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 ,&nbsp;Atsuko Nakano ,&nbsp;Yukiko Arimoto ,&nbsp;Mitsuko Kondo ,&nbsp;Yusuke Matsuda ,&nbsp;Miki Abo ,&nbsp;Takashi Kido ,&nbsp;Masashi Morishita ,&nbsp;Takeshige Honma ,&nbsp;Hisashi Nishimori ,&nbsp;Yuzaburo Inoue ,&nbsp;Keisuke Iwamoto ,&nbsp;Yuichiro Hashida ,&nbsp;Kazuhiko Takeuchi ,&nbsp;Miyabayashi Akiko ,&nbsp;Keiko Wakabayashi ,&nbsp;Hiroyuki Yamada ,&nbsp;Minako Hijikata ,&nbsp;Naoto Keicho ,&nbsp;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 &lt; 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}
引用次数: 0
Performance validation of a closed loop fully automated AI model for lung nodule stratification in screening cases 筛选病例中肺结节分层的闭环全自动AI模型的性能验证。
IF 2 Q2 RESPIRATORY SYSTEM Pub Date : 2026-01-20 DOI: 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.
背景:一些局限性阻碍了基于人的肺癌筛查(LCS)的有效性:高假阳性率导致不必要的随访成像、程序和手术;inter-reader可变性;肺- rads依从性不一致;以及与疲劳相关的诊断错误。此外,大多数人工智能(AI)模型只解决一个任务(结节检测或风险分层),并且需要手动图像处理,这既耗时又昂贵。我们开发了Bronchosolve,这是一种闭环,全自动软件,无需人工输入即可处理扫描,旨在提高LCS的一致性,准确性和吞吐量。方法:该软件集成了预处理、分析和结果生成,采用深度学习卷积神经网络(CNN)进行肺结节分类。输入是DICOM格式的全胸部CT扫描,没有临床或人口统计学数据。自动化步骤包括:1)最佳CT序列选择,2)归一化和预处理,3)基于人工智能的可疑结节检测和分类,4)报告生成。该模型在2358例(恶性和良性结节)的多中心高患病率集上进行训练。验证采用基于美国的多站点队列(n = 184; 8个站点)。阳性病例在1年内活检确诊;阴性患者行活检或随访≥2年。结果:所有病例均自动完成,成功率100%。中位年龄62.5岁(IQR 58.5-66.5);45%曾经吸烟,55%现在吸烟,40%是女性。该模型的AUC为0.898[0.851-0.940],优于Lung-RADS模型(AUC 0.669)和Brock模型(AUC 0.783)。灵敏度为83.6%;特异性为86.3%。不同扫描仪类型和切片厚度的性能保持一致。结论:Bronchosolve能够实现准确、全自动的肺结节风险分类,并可能增强非侵入性诊断工作流程。
{"title":"Performance validation of a closed loop fully automated AI model for lung nodule stratification in screening cases","authors":"A. Taha ,&nbsp;M.S. Muneer ,&nbsp;A. Kalra ,&nbsp;M. Muelly ,&nbsp;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}
引用次数: 0
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 同步多发原发肺癌病例,每一个都有EGFR突变或单独的ALK融合基因,对奥西替尼化疗有反应。
IF 2 Q2 RESPIRATORY SYSTEM Pub Date : 2026-01-20 DOI: 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.
综合病理评价对同步多发原发肺癌(sMPLC)的诊断有重要意义。然而,对于具有不同驱动突变的sMPLC的治疗缺乏共识。我们报告一例sMPLC携带表皮生长因子受体(EGFR)突变和间变性淋巴瘤激酶(ALK)融合基因。对于晚期egfr阳性肿瘤,采用奥西替尼加化疗,后者也适用于alk阳性肿瘤。egfr阳性和alk阳性肿瘤分别有明显的反应和轻微的减少。手术切除alk阳性肿瘤获得阴性边缘。靶向化疗可有效治疗不同驱动突变的sMPLC。
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引用次数: 0
Oxygenation deterioration and computed tomography findings in acute exacerbation of interstitial lung diseases: a prospective observational study 肺间质性疾病急性加重期的氧合恶化和计算机断层扫描表现:一项前瞻性观察研究
IF 2 Q2 RESPIRATORY SYSTEM Pub Date : 2026-01-19 DOI: 10.1016/j.resinv.2025.101361
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

Background

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)。
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引用次数: 0
期刊
Respiratory investigation
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