Pub Date : 2026-03-01Epub Date: 2026-03-12DOI: 10.1016/S2212-5345(26)00035-3
{"title":"Appreciation to reviewers in 2025","authors":"","doi":"10.1016/S2212-5345(26)00035-3","DOIUrl":"10.1016/S2212-5345(26)00035-3","url":null,"abstract":"","PeriodicalId":20934,"journal":{"name":"Respiratory investigation","volume":"64 2","pages":"Article 101401"},"PeriodicalIF":2.0,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147448552","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 : 2026-03-01Epub 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":"2026-03-01","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}
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":"2026-03-01","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}
Information on the incidence and clinical characteristics of respiratory syncytial virus (RSV) infections among adults remains limited in Japan. In this study, we aimed to clarify these aspects among adult outpatients at our institution.
Methods
This single-center, retrospective observational study included outpatients aged 18 years and older who presented with fever or respiratory symptoms and underwent multiplex PCR testing of nasopharyngeal specimens between April 2021 and March 2025. We assessed the RSV positive rate, underlying comorbidities, and hospitalization rate. For those with pneumonia, we analyzed their imaging findings and sputum culture results. Clinical characteristics were also compared between hospitalized and nonhospitalized patients aged 60 years or older.
Results
The overall positive rate of RSV infection over the 4 years was 2.3 %. Among individuals aged 60 years and older, the positive rate was 2.3 %. Of the 31 hospitalized cases, 29 were aged 60 years or older, accounting for 36.3 % of all RSV-positive patients in this age group. Age significantly differed between the hospitalized and nonhospitalized groups aged 60 and older. Among 24 patients with pneumonia who underwent computed tomography scans, 11 had positive sputum cultures. Consolidation was significantly more frequent in the 11 culture-positive cases than in the remaining 13 cases.
Conclusions
RSV infection accounted for 2.3 % of adult outpatient cases, with a notably high hospitalization rate in older adults. These findings highlight RSV as an important respiratory pathogen in older adults, underscoring the need to expand diagnostic testing and preventive strategies in this population.
{"title":"Real-world clinical data on adult respiratory syncytial virus infection in a Japanese community hospital: Emphasis on positivity rate, older adults, and pneumonia cases","authors":"Hiroyuki Honda , Koji Kuronuma , Yutaro Nagano , Kanami Nagano , Kojirou Uemura , Midori Hashimoto , Kaoru Nishiyama , Hirofumi Chiba","doi":"10.1016/j.resinv.2026.101371","DOIUrl":"10.1016/j.resinv.2026.101371","url":null,"abstract":"<div><h3>Background</h3><div>Information on the incidence and clinical characteristics of respiratory syncytial virus (RSV) infections among adults remains limited in Japan. In this study, we aimed to clarify these aspects among adult outpatients at our institution.</div></div><div><h3>Methods</h3><div>This single-center, retrospective observational study included outpatients aged 18 years and older who presented with fever or respiratory symptoms and underwent multiplex PCR testing of nasopharyngeal specimens between April 2021 and March 2025. We assessed the RSV positive rate, underlying comorbidities, and hospitalization rate. For those with pneumonia, we analyzed their imaging findings and sputum culture results. Clinical characteristics were also compared between hospitalized and nonhospitalized patients aged 60 years or older.</div></div><div><h3>Results</h3><div>The overall positive rate of RSV infection over the 4 years was 2.3 %. Among individuals aged 60 years and older, the positive rate was 2.3 %. Of the 31 hospitalized cases, 29 were aged 60 years or older, accounting for 36.3 % of all RSV-positive patients in this age group. Age significantly differed between the hospitalized and nonhospitalized groups aged 60 and older. Among 24 patients with pneumonia who underwent computed tomography scans, 11 had positive sputum cultures. Consolidation was significantly more frequent in the 11 culture-positive cases than in the remaining 13 cases.</div></div><div><h3>Conclusions</h3><div>RSV infection accounted for 2.3 % of adult outpatient cases, with a notably high hospitalization rate in older adults. These findings highlight RSV as an important respiratory pathogen in older adults, underscoring the need to expand diagnostic testing and preventive strategies in this population.</div></div>","PeriodicalId":20934,"journal":{"name":"Respiratory investigation","volume":"64 2","pages":"Article 101371"},"PeriodicalIF":2.0,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145966725","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}
Patients sometimes misidentify upper respiratory tract secretions or saliva as “sputum.” The objective of this study was to investigate the prevalence of such misidentification and its clinical associations.
Methods
We conducted a cross-sectional study of adults with cough and/or sputum symptoms, examining how often patients referred to upper respiratory tract secretions or saliva as “sputum,” and explored related clinical features.
Results
Of 72 patients with sputum symptoms, 32% and 13% referred to upper respiratory tract secretions and saliva, respectively, as “sputum.” Patients who misidentified upper respiratory secretions more often reported post-nasal drip (41.2% vs. 6.1%) and rhinitis (64.7% vs. 26.5%) than those who did not, which remained significant after adjustment for covariates (p < 0.05). Saliva misidentification was more common in older patients (p = 0.03).
Conclusions
32% of the patients with sputum production referred to upper respiratory tract secretions as “sputum,” highlighting a potential source of miscommunication in clinical practice.
{"title":"The prevalence of patients who mistakenly referred to upper respiratory tract secretions as sputum","authors":"Tadao Nagasaki , Masato Muraki , Soichiro Hanada , Ken Shirahase , Yoshiyuki Kawabata , Masamichi Iwai , Akiko Sano , Osamu Nishiyama , Takashi Iwanaga , Hiroyuki Sano , Ryuta Haraguchi , Yuji Tohda , Hisako Matsumoto","doi":"10.1016/j.resinv.2026.101378","DOIUrl":"10.1016/j.resinv.2026.101378","url":null,"abstract":"<div><h3>Background</h3><div>Patients sometimes misidentify upper respiratory tract secretions or saliva as “sputum.” The objective of this study was to investigate the prevalence of such misidentification and its clinical associations.</div></div><div><h3>Methods</h3><div>We conducted a cross-sectional study of adults with cough and/or sputum symptoms, examining how often patients referred to upper respiratory tract secretions or saliva as “sputum,” and explored related clinical features.</div></div><div><h3>Results</h3><div>Of 72 patients with sputum symptoms, 32% and 13% referred to upper respiratory tract secretions and saliva, respectively, as “sputum.” Patients who misidentified upper respiratory secretions more often reported post-nasal drip (41.2% vs. 6.1%) and rhinitis (64.7% vs. 26.5%) than those who did not, which remained significant after adjustment for covariates (<em>p</em> < 0.05). Saliva misidentification was more common in older patients (<em>p</em> = 0.03).</div></div><div><h3>Conclusions</h3><div>32% of the patients with sputum production referred to upper respiratory tract secretions as “sputum,” highlighting a potential source of miscommunication in clinical practice.</div></div>","PeriodicalId":20934,"journal":{"name":"Respiratory investigation","volume":"64 2","pages":"Article 101378"},"PeriodicalIF":2.0,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146158129","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":"2026-03-01","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}
Pub Date : 2026-03-01Epub Date: 2026-02-17DOI: 10.1016/j.resinv.2026.101386
Yasuo Shimizu
Mycobacterial infections and sarcoidosis have similar clinical presentations and require careful differential diagnosis, but the etiology differs between these diseases. It has been demonstrated that circulating interferon-γ levels decrease during infection by mycobacteria but increase in sarcoidosis. The involvement of innate-like lymphoid T cells in host immunity against these diseases has also become increasingly evident. Mucosal-associated invariant T (MAIT) cells are involved in innate immunity and are activated when antigens are presented to the T cell receptor through major histocompatibility complex class I-like molecules, namely MR1, by antigen-presenting cells. Notably, MAIT cells produce cytokines, such as interferon-γ, and antigen-derived ligands loaded onto MR1 can act as agonists or antagonists on MAIT cells. Therefore, MAIT cells may discriminate against ligands derived from the causative antigens of mycobacteria and sarcoidosis, potentially contributing to the ridge of the host immune response between these diseases.
{"title":"Are there differences in antigen recognition by mucosal-associated invariant T cells against mycobacterial infection and sarcoidosis?","authors":"Yasuo Shimizu","doi":"10.1016/j.resinv.2026.101386","DOIUrl":"10.1016/j.resinv.2026.101386","url":null,"abstract":"<div><div>Mycobacterial infections and sarcoidosis have similar clinical presentations and require careful differential diagnosis, but the etiology differs between these diseases. It has been demonstrated that circulating interferon-γ levels decrease during infection by mycobacteria but increase in sarcoidosis. The involvement of innate-like lymphoid T cells in host immunity against these diseases has also become increasingly evident. Mucosal-associated invariant T (MAIT) cells are involved in innate immunity and are activated when antigens are presented to the T cell receptor through major histocompatibility complex class I-like molecules, namely MR1, by antigen-presenting cells. Notably, MAIT cells produce cytokines, such as interferon-γ, and antigen-derived ligands loaded onto MR1 can act as agonists or antagonists on MAIT cells. Therefore, MAIT cells may discriminate against ligands derived from the causative antigens of mycobacteria and sarcoidosis, potentially contributing to the ridge of the host immune response between these diseases.</div></div>","PeriodicalId":20934,"journal":{"name":"Respiratory investigation","volume":"64 2","pages":"Article 101386"},"PeriodicalIF":2.0,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146220500","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 disease (ILD) comprises a wide range of pulmonary disorders associated with high morbidity and mortality. Although idiopathic pulmonary fibrosis (IPF) has been the focus of much prior research, population-level data covering the full spectrum of ILD in Japan remain limited.
Methods
This retrospective cohort study used the National Database of Health Insurance Claims (NDB) in Japan to identify patients diagnosed with ILD from January 2015 to December 2023. We analyzed annual prevalence, incidence, patient demographics, and survival outcomes overall and by ILD subtypes.
Results
A total of 2,758,542 patients with ILD were identified during the study period. ILD prevalence nearly doubled, from 656 per 100,000 population in 2015 to 1301 per 100,000 in 2023, while incidence remained relatively stable (192 per 100,000 in 2015 to 212 per 100,000 in 2023), with a notable decline observed in 2020 (178 per 100,000). Males consistently had higher prevalence and incidence than females. Age and sex distributions differed substantially across ILD subtypes. The overall 5-year survival rate was 59.9 %, with IPF demonstrating the poorest survival (32.1 %).
Conclusions
This nationwide study provides the most comprehensive epidemiological overview of ILD in Japan to date. The insights gained can support evidence-based healthcare planning and help guide priorities for future research and clinical improvements.
{"title":"Epidemiology of interstitial lung diseases in Japan: A nationwide database analysis","authors":"Koichi Miyashita , Yusuke Inoue , Hideki Yasui , Yuzo Suzuki , Masato Karayama , Hironao Hozumi , Kazuki Furuhashi , Noriyuki Enomoto , Tomoyuki Fujisawa , Eiji Nakatani , Naoki Inui , Toshiyuki Ojima , Takafumi Suda","doi":"10.1016/j.resinv.2026.101367","DOIUrl":"10.1016/j.resinv.2026.101367","url":null,"abstract":"<div><h3>Background</h3><div>Interstitial lung disease (ILD) comprises a wide range of pulmonary disorders associated with high morbidity and mortality. Although idiopathic pulmonary fibrosis (IPF) has been the focus of much prior research, population-level data covering the full spectrum of ILD in Japan remain limited.</div></div><div><h3>Methods</h3><div>This retrospective cohort study used the National Database of Health Insurance Claims (NDB) in Japan to identify patients diagnosed with ILD from January 2015 to December 2023. We analyzed annual prevalence, incidence, patient demographics, and survival outcomes overall and by ILD subtypes.</div></div><div><h3>Results</h3><div>A total of 2,758,542 patients with ILD were identified during the study period. ILD prevalence nearly doubled, from 656 per 100,000 population in 2015 to 1301 per 100,000 in 2023, while incidence remained relatively stable (192 per 100,000 in 2015 to 212 per 100,000 in 2023), with a notable decline observed in 2020 (178 per 100,000). Males consistently had higher prevalence and incidence than females. Age and sex distributions differed substantially across ILD subtypes. The overall 5-year survival rate was 59.9 %, with IPF demonstrating the poorest survival (32.1 %).</div></div><div><h3>Conclusions</h3><div>This nationwide study provides the most comprehensive epidemiological overview of ILD in Japan to date. The insights gained can support evidence-based healthcare planning and help guide priorities for future research and clinical improvements.</div></div>","PeriodicalId":20934,"journal":{"name":"Respiratory investigation","volume":"64 2","pages":"Article 101367"},"PeriodicalIF":2.0,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145941427","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}
Adherence to peak expiratory flow monitoring in asthma management is low because of the burden of data recording. We developed a smartphone application that helps record and monitor patients’ daily PEF.
Methods
PEF recordings and application usage were assessed over 4 weeks. Respiratory function was measured in the laboratory. We analyzed the peak flow recording rates, variability, and correlations between the collected data.
Results
The recording rates for asthma and non-asthma groups were 83.9 % and 73.2 %, respectively, twice daily. Participants with asthma found the application more user-friendly than paper diaries, and 90 % wanted to continue using it. The maximum PEF recorded with the application and the PEF collected through spirometry were correlated.
Conclusion
Our application can be a useful tool for evaluating asthma. Future studies involving larger patient cohorts are needed to confirm the usefulness of this smartphone application.
{"title":"Feasibility testing of a smartphone application for visualizing and sharing peak flow monitoring data in asthma evaluation","authors":"Kotaro Sasahara, Yuma Sakai, Katsunori Masaki, Saki Tomiyasu, Ryuta Onozato, Shiori Mori, Kaoru Koyama, Yo Otsu, Momoko Kurihara, Emiko Matsuyama, Reina Nakamura, Keeya Sunata, Masato Asaoka, Yuto Akiyama, Misato Irie, Hiroki Kabata, Jun Miyata, Koichi Fukunaga","doi":"10.1016/j.resinv.2025.101364","DOIUrl":"10.1016/j.resinv.2025.101364","url":null,"abstract":"<div><h3>Background</h3><div>Adherence to peak expiratory flow monitoring in asthma management is low because of the burden of data recording. We developed a smartphone application that helps record and monitor patients’ daily PEF.</div></div><div><h3>Methods</h3><div>PEF recordings and application usage were assessed over 4 weeks. Respiratory function was measured in the laboratory. We analyzed the peak flow recording rates, variability, and correlations between the collected data.</div></div><div><h3>Results</h3><div>The recording rates for asthma and non-asthma groups were 83.9 % and 73.2 %, respectively, twice daily. Participants with asthma found the application more user-friendly than paper diaries, and 90 % wanted to continue using it. The maximum PEF recorded with the application and the PEF collected through spirometry were correlated.</div></div><div><h3>Conclusion</h3><div>Our application can be a useful tool for evaluating asthma. Future studies involving larger patient cohorts are needed to confirm the usefulness of this smartphone application.</div></div>","PeriodicalId":20934,"journal":{"name":"Respiratory investigation","volume":"64 2","pages":"Article 101364"},"PeriodicalIF":2.0,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145998938","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 : 2026-03-01Epub Date: 2026-02-10DOI: 10.1016/j.resinv.2026.101383
Tatsuya Hirano , Ichidai Tanaka , Masahiro Morise , Junji Koyama , Takahiko Hashimoto , Kazumi Hori , Reiko Matsuzawa , Sho Hori , Kennosuke Karube , Takayasu Ito , Koji Sakamoto , Yuichiro Shindo , Mitsuo Sato , Toshihiko Yokoyama , Tomoki Kimura , Yasuhiro Kondoh , Makoto Ishii
Background
Non-small cell lung cancer (NSCLC) is diagnosed using small biopsy samples obtained by bronchoscopy or transthoracic lung core biopsy, and treated with immune checkpoint inhibitors, such as programmed cell death protein-1 (PD-1) and programmed cell death protein ligand-1 (PD-L1) inhibitors, as the first-line therapy. However, the clinical benefits of first-line PD-1/PD-L1 inhibitors in non-small cell carcinoma-not otherwise specified (NSCC–NOS) remain unclear. This study aimed to clarify the clinical efficacy of first-line PD-1/PD-L1 inhibitors in patients with NSCC-NOS.
Methods
We retrospectively enrolled patients with recurrent or unresectable advanced NSCLC treated with first-line PD-1/PD-L1 inhibitors, with or without chemotherapy, at three medical institutions. We adjusted for patient characteristics using propensity score matching (PSM) and analyzed the impact of the histological subtype, NSCC-NOS, on survival outcomes in patients with NSCLC treated with immunotherapy.
Results
Of the 312 eligible patients, 42 (13.5%) had a histological subtype of NSCC-NOS. Significantly more patients had PD-L1 ≥ 50% in the NSCC-NOS group than in the Non NSCC-NOS group (P = 0.01). After PSM, 41 patients with NSCC-NOS and 123 with other histological subtypes (Non NSCC-NOS) were analyzed. The median progression-free survival (PFS) and the median overall survival (OS) of patients with NSCC-NOS were significantly longer than those of patients with Non NSCC-NOS, respectively (median PFS: 14.8 vs. 6.1 months, P < 0.01; median OS: 33.4 vs. 15.1 months, P = 0.04, log-rank test).
Conclusions
NSCC-NOS is a histological subtype that is highly responsive to first-line PD-1/PD-L1 inhibitors with or without chemotherapy.
{"title":"Favorable clinical impact of histological subtype with non-small cell carcinoma-not otherwise specified in patients with non-small cell lung cancer receiving immune checkpoint inhibitors","authors":"Tatsuya Hirano , Ichidai Tanaka , Masahiro Morise , Junji Koyama , Takahiko Hashimoto , Kazumi Hori , Reiko Matsuzawa , Sho Hori , Kennosuke Karube , Takayasu Ito , Koji Sakamoto , Yuichiro Shindo , Mitsuo Sato , Toshihiko Yokoyama , Tomoki Kimura , Yasuhiro Kondoh , Makoto Ishii","doi":"10.1016/j.resinv.2026.101383","DOIUrl":"10.1016/j.resinv.2026.101383","url":null,"abstract":"<div><h3>Background</h3><div>Non-small cell lung cancer (NSCLC) is diagnosed using small biopsy samples obtained by bronchoscopy or transthoracic lung core biopsy, and treated with immune checkpoint inhibitors, such as programmed cell death protein-1 (PD-1) and programmed cell death protein ligand-1 (PD-L1) inhibitors, as the first-line therapy. However, the clinical benefits of first-line PD-1/PD-L1 inhibitors in non-small cell carcinoma-not otherwise specified (NSCC–NOS) remain unclear. This study aimed to clarify the clinical efficacy of first-line PD-1/PD-L1 inhibitors in patients with NSCC-NOS.</div></div><div><h3>Methods</h3><div>We retrospectively enrolled patients with recurrent or unresectable advanced NSCLC treated with first-line PD-1/PD-L1 inhibitors, with or without chemotherapy, at three medical institutions. We adjusted for patient characteristics using propensity score matching (PSM) and analyzed the impact of the histological subtype, NSCC-NOS, on survival outcomes in patients with NSCLC treated with immunotherapy.</div></div><div><h3>Results</h3><div>Of the 312 eligible patients, 42 (13.5%) had a histological subtype of NSCC-NOS. Significantly more patients had PD-L1 ≥ 50% in the NSCC-NOS group than in the Non NSCC-NOS group (<em>P</em> = 0.01). After PSM, 41 patients with NSCC-NOS and 123 with other histological subtypes (Non NSCC-NOS) were analyzed. The median progression-free survival (PFS) and the median overall survival (OS) of patients with NSCC-NOS were significantly longer than those of patients with Non NSCC-NOS, respectively (median PFS: 14.8 vs. 6.1 months, <em>P</em> < 0.01; median OS: 33.4 vs. 15.1 months, <em>P</em> = 0.04, log-rank test).</div></div><div><h3>Conclusions</h3><div>NSCC-NOS is a histological subtype that is highly responsive to first-line PD-1/PD-L1 inhibitors with or without chemotherapy.</div></div>","PeriodicalId":20934,"journal":{"name":"Respiratory investigation","volume":"64 2","pages":"Article 101383"},"PeriodicalIF":2.0,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146166533","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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