2f:I[703058,["/_next/static/immutable/chunks/3bhq_5eh1d760.js","/_next/static/immutable/chunks/1855-ogyi8eh4.js","/_next/static/immutable/chunks/2pfsjpn6k6zlg.js","/_next/static/immutable/chunks/26c7v3_3uwnwu.js","/_next/static/immutable/chunks/25a3ozcrvi1ff.js","/_next/static/immutable/chunks/2r4wx50nvjh_4.js","/_next/static/immutable/chunks/23fsw06hsi5b1.js","/_next/static/immutable/chunks/06_yt270h41rg.js","/_next/static/immutable/chunks/3ezsiz-7shkna.js","/_next/static/immutable/chunks/20x2y96ldu5wx.js","/_next/static/immutable/chunks/2lxntcm3pd1_h.js","/_next/static/immutable/chunks/00dgco8irq3jw.js","/_next/static/immutable/chunks/00nwe-kq0rboc.js","/_next/static/immutable/chunks/2051ffx3xzio9.js"],"PaperListPageClient"] 30:T9d4,Dipeptidyl peptidase-1 (DPP1) inhibitors prevent the activation of neutrophil serine proteases and reduce exacerbations in people with bronchiectasis. We previously identified a novel effect of DPP1 inhibitors in reducing the neutrophil pseudoenzyme azurocidin-1 (AZU1). The aim of this study was to investigate the role of AZU1 in the pathophysiology of bronchiectasis. Sputum AZU1 concentrations were analysed in multiple cohorts. These consisted of two observational cohorts of patients with bronchiectasis (EMBARC BRIDGE cohort 1 and cohort 2) and a cohort of patients with chronic obstructive pulmonary disease (COPD; TARDIS COPD cohort) to correlate AZU1 with disease severity and exacerbations. A rhinovirus challenge study was used to investigate AZU1 concentrations during experimental exacerbation in COPD, people who smoke, and controls. A post-hoc analysis of the phase 2 WILLOW trial of brensocatib versus placebo was used to assess the effect of DPP1 inhibition on airway AZU1. Higher AZU1 sputum concentration was associated with increased bronchiectasis disease severity index (p<0·0001), decreased percentage predicted forced expiratory volume in 1 second (r=-0·4662, p<0·001), and increased exacerbation frequency (p<0·0019; EMBARC cohort 1, n=197). AZU1 was associated with radiological severity (Reiff score), symptoms (quality of life bronchiectasis respiratory symptom score), and bacterial infection (sputum microbiology and 16S microbiome alpha diversity; highest levels of AZU1 were found in airway samples with Pseudomonas aeruginosa; p<0·0001; EMBARC cohort 2, n=144). Bronchiectasis patients with bacterial and viral exacerbations had increased concentrations of AZU1 (p=0·0003; n=96). These findings were extended to COPD, in which AZU1 was related to COPD severity (COPD cohort, n=101), and in patients with COPD challenged with rhinovirus A16, AZU1 was increased at day 9 post-challenge (p<0·001; n=9). In-vitro AZU1 impaired ciliary function and epithelial integrity, suggesting a mechanism by which AZU1 drives disease pathogenesis. In a post-hoc analysis of the WILLOW trial, AZU1 was the most downregulated protein with brensocatib treatment (brensocatib 10 mg, n=71; brensocatib 25 mg, n=73; and placebo, n=71). Over 24 weeks, AZU1 was significantly reduced by DPP1 inhibition (p<0·0001). AZU1 was identified as a novel marker of disease severity in bronchiectasis, associated with bacterial infection and exacerbation, and targeted by DPP1 inhibition. EMBARC3 and Insmed.25:[[["$","script","0",{"type":"application/ld+json","dangerouslySetInnerHTML":{"__html":"{\"@context\":\"https://schema.org\",\"@type\":\"CollectionPage\",\"name\":\"Bronchiectasis: condition Research & Clinical Takeaways\",\"description\":\"Explore peer-reviewed papers on Bronchiectasis (condition). Clinician-written summaries and actionable practice insights. Updated daily.\",\"url\":\"https://www.reachrx.ai/hub/tag/condition/Bronchiectasis\",\"breadcrumb\":{\"@type\":\"BreadcrumbList\",\"itemListElement\":[{\"@type\":\"ListItem\",\"position\":1,\"name\":\"Home\",\"item\":\"https://www.reachrx.ai\"},{\"@type\":\"ListItem\",\"position\":2,\"name\":\"Research Hub\",\"item\":\"https://www.reachrx.ai/hub\"},{\"@type\":\"ListItem\",\"position\":3,\"name\":\"Conditions\",\"item\":\"https://www.reachrx.ai/hub/tag/condition\"},{\"@type\":\"ListItem\",\"position\":4,\"name\":\"Bronchiectasis\",\"item\":\"https://www.reachrx.ai/hub/tag/condition/Bronchiectasis\"}]}}"}}]],["$","$L2f",null,{"title":"Bronchiectasis: Research & Clinical Takeaways","description":"Discover peer-reviewed research on Bronchiectasis with concise clinician summaries and next-step guidance.","papers":[{"doi":"10.1183/23120541.00735-2026","pmid":null,"title":"Sputum colour variability and clinical outcomes in bronchiectasis: a multicentre Canadian-UK study","journal_title":"ERJ Open Research","journal_issn":"2312-0541","published_at":"2026-08-26T00:00:00.000Z","url":"https://doi.org/10.1183/23120541.00735-2026","source_score":4,"is_retracted":false,"is_open_access":true,"raw_metadata":{"pubmed":false,"crossref":true,"unpaywall":true},"created_at":"2026-09-03T06:20:01.720Z","updated_at":"2026-09-03T06:20:01.720Z","tags":[{"paper_doi":"10.1183/23120541.00735-2026","tag_type":"age_group","value":"adult","value_numeric":null},{"paper_doi":"10.1183/23120541.00735-2026","tag_type":"category","value":"Respiratory System","value_numeric":null},{"paper_doi":"10.1183/23120541.00735-2026","tag_type":"condition","value":"Bronchiectasis","value_numeric":null},{"paper_doi":"10.1183/23120541.00735-2026","tag_type":"impact_flag","value":"practice_changing","value_numeric":null},{"paper_doi":"10.1183/23120541.00735-2026","tag_type":"level_of_evidence","value":"2","value_numeric":null},{"paper_doi":"10.1183/23120541.00735-2026","tag_type":"modality","value":"Antibiotic Therapy","value_numeric":null},{"paper_doi":"10.1183/23120541.00735-2026","tag_type":"modality","value":"Biomarker Assessment","value_numeric":null},{"paper_doi":"10.1183/23120541.00735-2026","tag_type":"sample_size","value":"n=86","value_numeric":86},{"paper_doi":"10.1183/23120541.00735-2026","tag_type":"sex","value":"mixed","value_numeric":null},{"paper_doi":"10.1183/23120541.00735-2026","tag_type":"study_design","value":"Prospective Cohort Study","value_numeric":null},{"paper_doi":"10.1183/23120541.00735-2026","tag_type":"warning","value":"Level of evidence assigned as '2' reflects prospective cohort design; no randomisation was performed.","value_numeric":null},{"paper_doi":"10.1183/23120541.00735-2026","tag_type":"warning","value":"Sample size is small (n=86 patients), which may limit the reliability of subgroup analyses such as colour variability stratification.","value_numeric":null},{"paper_doi":"10.1183/23120541.00735-2026","tag_type":"warning","value":"Study design is observational/prospective cohort — causal inferences about sputum colour driving outcomes should not be made.","value_numeric":null},{"paper_doi":"10.1183/23120541.00735-2026","tag_type":"warning","value":"The BEST score (patient-reported) was only collected in the UK cohort, creating an asymmetry between the two study populations.","value_numeric":null}],"summaries":{"what_studied":"Prospective multicentre study (Canadian Calgary Biobank and UK Liverpool POSTED cohorts) examining sputum colour stability, variability, and its relationship to disease severity and clinical outcomes in adults with non-CF bronchiectasis over up to 20 years, using both investigator-rated Murray 4-point chart and patient-reported BEST scores.","key_result":"Purulent sputum correlated with lower lung function, higher BSI scores, more exacerbations, and higher chronic *P. aeruginosa* prevalence across 1,193 samples from 86 patients. Sputum colour was stable in ~90% of samples; patients with variable colour had more severe disease than those with persistently mucoid sputum. Patient-reported colour change and BEST score shifts — but not investigator-rated objective changes — were associated with shorter time to next exacerbation and greater antibiotic use.","limitations":"Small sample size (n=86) limits statistical power and generalisability. Investigator–patient discordance was common, introducing measurement uncertainty. Only two cohorts from Canada and UK were included, limiting global applicability.","practice_takeaway":"Trust the patient: self-reported sputum colour changes and BEST score shifts are better short-term predictors of exacerbation and antibiotic need than clinician-rated colour in bronchiectasis. Use Murray colour variability alongside BSI scores to stratify long-term disease severity in clinic."}},{"doi":"10.1002/ppul.71810","pmid":"42643099","title":"Evaluation of Pulmonary and Otological Manifestations in Primary Ciliary Dyskinesia and Their Effect on Quality of Life","journal_title":"Pediatric Pulmonology","journal_issn":"8755-6863","published_at":"2026-08-26T00:00:00.000Z","url":"https://doi.org/10.22541/au.174789764.47378366/v1","source_score":2,"is_retracted":false,"is_open_access":true,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-09-06T06:22:52.237Z","updated_at":"2026-09-06T06:22:52.237Z","tags":[{"paper_doi":"10.1002/ppul.71810","tag_type":"age_group","value":"mixed","value_numeric":null},{"paper_doi":"10.1002/ppul.71810","tag_type":"category","value":"Respiratory System","value_numeric":null},{"paper_doi":"10.1002/ppul.71810","tag_type":"condition","value":"Bronchiectasis","value_numeric":null},{"paper_doi":"10.1002/ppul.71810","tag_type":"condition","value":"Chronic Rhinosinusitis","value_numeric":null},{"paper_doi":"10.1002/ppul.71810","tag_type":"condition","value":"Conductive Hearing Loss","value_numeric":null},{"paper_doi":"10.1002/ppul.71810","tag_type":"condition","value":"Otitis Media With Effusion","value_numeric":null},{"paper_doi":"10.1002/ppul.71810","tag_type":"condition","value":"Primary Ciliary Dyskinesia","value_numeric":null},{"paper_doi":"10.1002/ppul.71810","tag_type":"impact_flag","value":"limited_evidence","value_numeric":null},{"paper_doi":"10.1002/ppul.71810","tag_type":"level_of_evidence","value":"4","value_numeric":null},{"paper_doi":"10.1002/ppul.71810","tag_type":"sample_size","value":"n=56","value_numeric":56},{"paper_doi":"10.1002/ppul.71810","tag_type":"sex","value":"mixed","value_numeric":null},{"paper_doi":"10.1002/ppul.71810","tag_type":"study_design","value":"Cross-Sectional Study","value_numeric":null},{"paper_doi":"10.1002/ppul.71810","tag_type":"warning","value":"No multiple testing correction was applied; several exploratory comparisons should be interpreted cautiously.","value_numeric":null},{"paper_doi":"10.1002/ppul.71810","tag_type":"warning","value":"Speech-in-noise impairment rate (64.3%) is novel — this is the first study to apply this test in PCD — so reference ranges and clinical thresholds in this population are not yet established.","value_numeric":null},{"paper_doi":"10.1002/ppul.71810","tag_type":"warning","value":"The study is single-center with n=56; 17 of 73 eligible patients were excluded due to refusal or missed appointments, introducing potential selection bias.","value_numeric":null}],"summaries":{"what_studied":"This cross-sectional study (Jan 2023–Jan 2024) evaluated 56 PCD patients aged ≥6 years (mean age 15.2 years) for otologic, pulmonary, and sinonasal manifestations and their impact on health-related QOL (QOL-PCD), using pure-tone audiometry, DPOAE, tympanogram, speech-in-noise testing (TUR-Matrix), spirometry, and SNOT-22.","key_result":"Pathologic hearing was found in 46.4% (26/56) and abnormal speech-in-noise (TUR-Matrix) in 64.3% (36/56). Patients with pathologic hearing had significantly worse FEV1 z-scores (−1.7 vs. −0.8, p=0.01), more abnormal DPOAE (25 vs. 11, p<0.001), and worse tympanograms (26 vs. 12, p<0.001). Adults had significantly lower QOL scores in social, upper, and lower respiratory domains than children and adolescents (p<0.001, 0.02, and 0.01, respectively).","limitations":"- Single-center design with a small sample (n=56) limits generalizability.\n- No multiple testing correction applied despite numerous comparisons.\n- Cross-sectional design prevents causal inference between hearing loss and pulmonary decline.","practice_takeaway":"Nearly half of PCD patients have measurable hearing loss, and nearly two-thirds struggle with speech understanding in noise — both often go unreported. Routine multidisciplinary assessment including objective audiologic testing (not just symptom inquiry) should be standard in PCD care, especially in patients with lower FEV1."}},{"doi":"10.1183/23120541.00106-2026","pmid":null,"title":"Clinical heterogeneity of bronchiectasis exacerbations: a multicentre real-world study","journal_title":"ERJ Open Research","journal_issn":"2312-0541","published_at":"2026-08-20T00:00:00.000Z","url":"https://doi.org/10.1183/23120541.00106-2026","source_score":4,"is_retracted":false,"is_open_access":true,"raw_metadata":{"pubmed":false,"crossref":true,"unpaywall":true},"created_at":"2026-08-30T06:19:59.458Z","updated_at":"2026-08-30T06:19:59.458Z","tags":[{"paper_doi":"10.1183/23120541.00106-2026","tag_type":"age_group","value":"older_adult","value_numeric":null},{"paper_doi":"10.1183/23120541.00106-2026","tag_type":"category","value":"Respiratory System","value_numeric":null},{"paper_doi":"10.1183/23120541.00106-2026","tag_type":"condition","value":"Bronchiectasis","value_numeric":null},{"paper_doi":"10.1183/23120541.00106-2026","tag_type":"condition","value":"Chronic Pseudomonas aeruginosa Infection","value_numeric":null},{"paper_doi":"10.1183/23120541.00106-2026","tag_type":"condition","value":"Nontuberculous Mycobacterial Pulmonary Disease","value_numeric":null},{"paper_doi":"10.1183/23120541.00106-2026","tag_type":"impact_flag","value":"limited_evidence","value_numeric":null},{"paper_doi":"10.1183/23120541.00106-2026","tag_type":"level_of_evidence","value":"4","value_numeric":null},{"paper_doi":"10.1183/23120541.00106-2026","tag_type":"sample_size","value":"n=367","value_numeric":367},{"paper_doi":"10.1183/23120541.00106-2026","tag_type":"sex","value":"mixed","value_numeric":null},{"paper_doi":"10.1183/23120541.00106-2026","tag_type":"study_design","value":"Cross-Sectional Study","value_numeric":null},{"paper_doi":"10.1183/23120541.00106-2026","tag_type":"warning","value":"No modality or drug intervention was studied; no brand/generic or treatment tags are applicable.","value_numeric":null},{"paper_doi":"10.1183/23120541.00106-2026","tag_type":"warning","value":"Symptom data are retrospective and patient-reported (recall over 2 years), which may inflate or distort frequency estimates.","value_numeric":null},{"paper_doi":"10.1183/23120541.00106-2026","tag_type":"warning","value":"The cohort is 79% female and entirely Italian, which may not reflect global bronchiectasis populations with different sex distributions or microbiology profiles.","value_numeric":null}],"summaries":{"limitations":"Cross-sectional design relies on patient recall of exacerbations over the prior 2 years, introducing recall bias. Single-country (Italian) cohort limits generalisability. Symptom data are patient-reported without objective validation.","what_studied":"A multicentre cross-sectional study across nine Italian bronchiectasis centres evaluated the clinical heterogeneity of exacerbation symptoms in 367 consecutive adults, comparing symptom patterns by comorbidity and infection subgroups against the 2017 EMBARC/BRR consensus definition.","key_result":"Cough (87%), increased sputum volume (81%), and purulence (77%) were the most common exacerbation symptoms; 85% of typical exacerbations met EMBARC/BRR criteria, but ~1 in 7 did not — especially in patients with NTM-PD (p=0.004). Asthma/COPD comorbidity linked to more dyspnoea and wheezing; chronic *P. aeruginosa* infection linked to more haemoptysis and nocturnal dyspnoea.","practice_takeaway":"Clinicians should not rely solely on EMBARC/BRR criteria to identify exacerbations — particularly in patients with NTM-PD, where typical events frequently fall outside consensus definitions. Tailor symptom assessment to patient subgroups (e.g., screen for haemoptysis in chronic *P. aeruginosa* infection)."}},{"doi":"10.1002/ppul.71790","pmid":"42604456","title":"Physiotherapy in Primary Ciliary Dyskinesia—What Is the Evidence?: A Narrative Review and Personal Experience","journal_title":"Pediatric Pulmonology","journal_issn":"8755-6863","published_at":"2026-08-16T00:00:00.000Z","url":"https://doi.org/10.1002/ppul.71790","source_score":1,"is_retracted":false,"is_open_access":true,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-08-25T06:20:10.600Z","updated_at":"2026-08-25T06:20:10.600Z","tags":[{"paper_doi":"10.1002/ppul.71790","tag_type":"age_group","value":"mixed","value_numeric":null},{"paper_doi":"10.1002/ppul.71790","tag_type":"category","value":"Respiratory System","value_numeric":null},{"paper_doi":"10.1002/ppul.71790","tag_type":"condition","value":"Bronchiectasis","value_numeric":null},{"paper_doi":"10.1002/ppul.71790","tag_type":"condition","value":"Primary Ciliary Dyskinesia","value_numeric":null},{"paper_doi":"10.1002/ppul.71790","tag_type":"generic","value":"dornase alfa","value_numeric":null},{"paper_doi":"10.1002/ppul.71790","tag_type":"generic","value":"hypertonic saline","value_numeric":null},{"paper_doi":"10.1002/ppul.71790","tag_type":"impact_flag","value":"limited_evidence","value_numeric":null},{"paper_doi":"10.1002/ppul.71790","tag_type":"level_of_evidence","value":"5","value_numeric":null},{"paper_doi":"10.1002/ppul.71790","tag_type":"modality","value":"Airway Clearance Therapy","value_numeric":null},{"paper_doi":"10.1002/ppul.71790","tag_type":"modality","value":"Exercise Intervention","value_numeric":null},{"paper_doi":"10.1002/ppul.71790","tag_type":"modality","value":"Mucolytic Therapy","value_numeric":null},{"paper_doi":"10.1002/ppul.71790","tag_type":"study_design","value":"Narrative Review","value_numeric":null},{"paper_doi":"10.1002/ppul.71790","tag_type":"warning","value":"Dornase alfa (rhDNase) is tagged as a generic based on its mention as 'rhDNase' in the abstract; the review describes its evidence as weak or controversial specifically in PCD, distinct from its established role in cystic fibrosis.","value_numeric":null},{"paper_doi":"10.1002/ppul.71790","tag_type":"warning","value":"Level of evidence assigned as 5 (expert opinion/narrative review); no meta-analysis or systematic methodology was described.","value_numeric":null},{"paper_doi":"10.1002/ppul.71790","tag_type":"warning","value":"No sample size is applicable as this is a narrative review, not a primary study.","value_numeric":null},{"paper_doi":"10.1002/ppul.71790","tag_type":"warning","value":"The review includes 'personal experience' in the title, suggesting some recommendations may reflect author-specific practice rather than consensus.","value_numeric":null}],"summaries":{"what_studied":"This narrative review evaluates the evidence for physiotherapy—especially airway clearance therapy (ACT)—in managing Primary Ciliary Dyskinesia (PCD), covering techniques, mucolytic agents, exercise, and age-specific considerations across the lifespan.","key_result":"No ACT technique (PEP, ACBT, autogenic drainage, IPV, HFCWO, etc.) has shown clear superiority over others; evidence for mucolytics such as hypertonic saline or rhDNase is weak or controversial; exercise may improve ventilatory capacity and quality of life, but data are scarce.","limitations":"Current recommendations rely heavily on expert opinion and extrapolation from cystic fibrosis and non-CF bronchiectasis, rather than PCD-specific RCT data; randomized trials are ethically difficult when ACT is already considered standard care; practice varies widely with no standardized international guidelines.","practice_takeaway":"Start ACT early in PCD, adapt the technique to the patient's developmental stage, and prioritize adherence strategies—but recognize that the choice of specific ACT method should be individualized, as no single approach is proven superior. Mucolytics should be used cautiously given limited supporting evidence in PCD."}},{"doi":"10.1016/j.chest.2026.06.057","pmid":"42480819","title":"Management of Adult Bronchiectasis: An American College of Chest Physicians Clinical Practice Guideline","journal_title":"CHEST","journal_issn":"0012-3692","published_at":"2026-07-21T00:00:00.000Z","url":"https://doi.org/10.1016/j.chest.2026.06.057","source_score":3,"is_retracted":false,"is_open_access":true,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-07-28T14:02:19.608Z","updated_at":"2026-07-28T14:02:19.608Z","tags":[{"paper_doi":"10.1016/j.chest.2026.06.057","tag_type":"age_group","value":"adult","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.06.057","tag_type":"category","value":"Respiratory System","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.06.057","tag_type":"condition","value":"Bronchiectasis","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.06.057","tag_type":"impact_flag","value":"limited_evidence","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.06.057","tag_type":"level_of_evidence","value":"4","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.06.057","tag_type":"modality","value":"Airway Clearance","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.06.057","tag_type":"modality","value":"Antibiotic Therapy","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.06.057","tag_type":"modality","value":"Anti-Inflammatory Therapy","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.06.057","tag_type":"modality","value":"Surgical Resection","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.06.057","tag_type":"sample_size","value":"n=47","value_numeric":47},{"paper_doi":"10.1016/j.chest.2026.06.057","tag_type":"study_design","value":"Clinical Practice Guideline","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.06.057","tag_type":"warning","value":"All 13 recommendations are conditional with low-certainty evidence; clinicians should interpret guidance as expert consensus rather than high-quality RCT-backed directives.","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.06.057","tag_type":"warning","value":"Study design classified as Clinical Practice Guideline based on GRADE methodology and Delphi consensus process; it is not a primary research study.","value_numeric":null},{"paper_doi":"10.1016/j.chest.2026.06.057","tag_type":"warning","value":"The sample_size tag reflects the number of included studies (n=47), not individual patients — total patient enrollment is not reported in the abstract.","value_numeric":null}],"summaries":{"what_studied":"An expert panel evaluated 8 PICO questions on adult bronchiectasis management — covering antibiotic therapy for acute exacerbations, long-term suppressive antibiotics, anti-inflammatory therapies, airway clearance, hemoptysis management, and surgical resection — using a systematic review of 47 studies and the GRADE approach.","key_result":"Thirteen evidence-based recommendations were produced across all key management domains; all 13 are conditional recommendations based primarily on low-certainty evidence.","limitations":"All recommendations are conditional and based on low-certainty evidence, limiting the strength of guidance. Accurate phenotyping and endotyping to guide treatment decisions remain underdeveloped. Variability in clinical practice and research gaps persist across most PICO domains.","practice_takeaway":"Clinicians should apply shared decision-making and a multidisciplinary approach when managing bronchiectasis, as no recommendation carries high-certainty backing. Treatment burden — including cost and quality-of-life impact — must be weighed in every therapeutic decision."}},{"doi":"10.1183/23120541.01397-2025","pmid":"42445230","title":"The association between chronic rhinosinusitis, bronchiectasis and type 2 inflammation: an EMBARC registry analysis","journal_title":"ERJ Open Research","journal_issn":"2312-0541","published_at":"2026-07-13T00:00:00.000Z","url":"https://doi.org/10.1183/23120541.01397-2025","source_score":6,"is_retracted":false,"is_open_access":true,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-07-18T13:39:56.711Z","updated_at":"2026-07-18T13:39:56.711Z","tags":[{"paper_doi":"10.1183/23120541.01397-2025","tag_type":"age_group","value":"adult","value_numeric":null},{"paper_doi":"10.1183/23120541.01397-2025","tag_type":"category","value":"Respiratory System","value_numeric":null},{"paper_doi":"10.1183/23120541.01397-2025","tag_type":"condition","value":"Bronchiectasis","value_numeric":null},{"paper_doi":"10.1183/23120541.01397-2025","tag_type":"condition","value":"Chronic Rhinosinusitis","value_numeric":null},{"paper_doi":"10.1183/23120541.01397-2025","tag_type":"condition","value":"Immunodeficiency","value_numeric":null},{"paper_doi":"10.1183/23120541.01397-2025","tag_type":"condition","value":"Nasal Polyposis","value_numeric":null},{"paper_doi":"10.1183/23120541.01397-2025","tag_type":"condition","value":"Primary Ciliary Dyskinesia","value_numeric":null},{"paper_doi":"10.1183/23120541.01397-2025","tag_type":"impact_flag","value":"practice_changing","value_numeric":null},{"paper_doi":"10.1183/23120541.01397-2025","tag_type":"level_of_evidence","value":"3","value_numeric":null},{"paper_doi":"10.1183/23120541.01397-2025","tag_type":"modality","value":"Biologic / Monoclonal Antibody (Type 2-Directed)","value_numeric":null},{"paper_doi":"10.1183/23120541.01397-2025","tag_type":"sample_size","value":"n=16640","value_numeric":16640},{"paper_doi":"10.1183/23120541.01397-2025","tag_type":"sex","value":"mixed","value_numeric":null},{"paper_doi":"10.1183/23120541.01397-2025","tag_type":"study_design","value":"Registry-Based Observational Study","value_numeric":null},{"paper_doi":"10.1183/23120541.01397-2025","tag_type":"warning","value":"CRS and nasal polyp status were recorded by site investigators without formal EPOS criteria validation, which may introduce differential ascertainment across the 30+ countries in the registry.","value_numeric":null},{"paper_doi":"10.1183/23120541.01397-2025","tag_type":"warning","value":"'Practice_changing' flag applied cautiously — the study identifies a biomarker-enriched phenotype relevant to trial design, but no interventional data for biologics in this specific population are yet available.","value_numeric":null},{"paper_doi":"10.1183/23120541.01397-2025","tag_type":"warning","value":"The chief editor of the publishing journal (ERJ Open Research) is a co-author (J.D. Chalmers), which is a potential conflict of interest noted in the paper itself.","value_numeric":null},{"paper_doi":"10.1183/23120541.01397-2025","tag_type":"warning","value":"The level of evidence is assigned as 3 (prospective cohort/registry); it is not a randomised trial and causality cannot be inferred.","value_numeric":null}],"summaries":{"what_studied":"This EMBARC registry analysis examined the prevalence and impact of chronic rhinosinusitis with (CRSwNP) and without nasal polyps (CRSnNP) in 16,640 adults with bronchiectasis, and whether comorbid CRS is linked to type 2 inflammatory biomarkers (elevated blood eosinophil count or total IgE ≥150 IU/mL) across different bronchiectasis aetiologies (idiopathic, post-infective, PCD, immune deficiency, and others).","key_result":"CRSnNP was present in 20.9% and CRSwNP in 7.3% of patients. Both subtypes were linked to worse respiratory symptoms, more exacerbations, but **lower hospitalisation rates and mortality** compared to no CRS. CRSwNP independently predicted elevated type 2 biomarkers (OR ~2.1 for elevated eosinophils or IgE), but this association held only in idiopathic and post-infective bronchiectasis — not in PCD or immune deficiency.","limitations":"- CRS and nasal polyp diagnoses were investigator-reported from a registry field, not validated against established EPOS criteria, introducing potential misclassification.\n- Blood eosinophils and total IgE were not available for all patients (~46% had eosinophil data), and more granular type 2 markers (sputum eosinophils, fractional exhaled NO, specific IgE) were absent.\n- Immunodeficiency was grouped as a single category despite heterogeneous underlying mechanisms.","practice_takeaway":"In bronchiectasis patients with nasal polyps, actively check for elevated eosinophils and IgE — this phenotype may benefit from type 2-directed biologics. Conversely, CRS in PCD or immune deficiency is unlikely to signal type 2 inflammation, so routine biomarker screening in those groups is less warranted."}},{"doi":"10.1093/ajrccm/aamag297","pmid":"42348458","title":"The frequent exacerbator phenotype in bronchiectasis revisited: Data from EMBARC registry","journal_title":"American Journal of Respiratory and Critical Care Medicine","journal_issn":"1073-449X","published_at":"2026-06-25T00:00:00.000Z","url":"https://doi.org/10.1093/ajrccm/aamag297","source_score":13,"is_retracted":false,"is_open_access":true,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-07-01T13:27:17.820Z","updated_at":"2026-07-01T13:27:17.820Z","tags":[{"paper_doi":"10.1093/ajrccm/aamag297","tag_type":"age_group","value":"adult","value_numeric":null},{"paper_doi":"10.1093/ajrccm/aamag297","tag_type":"category","value":"Respiratory System","value_numeric":null},{"paper_doi":"10.1093/ajrccm/aamag297","tag_type":"condition","value":"Bronchiectasis","value_numeric":null},{"paper_doi":"10.1093/ajrccm/aamag297","tag_type":"impact_flag","value":"practice_changing","value_numeric":null},{"paper_doi":"10.1093/ajrccm/aamag297","tag_type":"level_of_evidence","value":"2","value_numeric":null},{"paper_doi":"10.1093/ajrccm/aamag297","tag_type":"sample_size","value":"n=19324","value_numeric":19324},{"paper_doi":"10.1093/ajrccm/aamag297","tag_type":"sex","value":"mixed","value_numeric":null},{"paper_doi":"10.1093/ajrccm/aamag297","tag_type":"study_design","value":"Prospective Cohort Study (Registry-Based)","value_numeric":null},{"paper_doi":"10.1093/ajrccm/aamag297","tag_type":"warning","value":"The 'frequent exacerbator' threshold of ≥3/year is challenged but not yet replaced by an official updated definition; clinicians should interpret the continuous-risk framing cautiously pending guideline revision.","value_numeric":null},{"paper_doi":"10.1093/ajrccm/aamag297","tag_type":"warning","value":"The paper is published June 25, 2026 — very recent; clinical guideline uptake of these findings has not yet occurred.","value_numeric":null},{"paper_doi":"10.1093/ajrccm/aamag297","tag_type":"warning","value":"The study design is observational (registry), so causality cannot be established despite the large sample size.","value_numeric":null}],"summaries":{"what_studied":"This study used the EMBARC registry (30 countries, Europe and Asia) to quantify how each prior exacerbation predicts future exacerbations and hospitalizations in 19,324 bronchiectasis patients over up to 5 years, and whether the pattern varies by etiology or region.","key_result":"Each additional prior exacerbation raised future exacerbation risk incrementally: IRR 1.45 for 1 prior, 1.84 for 2, 2.50 for 3, and 3.56 for ≥4. One prior severe (hospitalized) exacerbation raised future severe exacerbation risk nearly 4-fold (IRR 3.96, 95% CI 3.71–4.22). The pattern was consistent across etiologies and regions, with no clear threshold separating high vs. low risk.","limitations":"Observational registry design limits causal inference. Exacerbation history was captured at baseline, so recall or recording bias is possible. The analysis cannot fully account for unmeasured confounders (e.g., treatment intensity, comorbidities) that may drive both prior and future exacerbations.","practice_takeaway":"Treat every exacerbation in bronchiectasis as a meaningful risk marker — not just the third one. Patients with even one prior hospitalization warrant especially close follow-up, given nearly a 4-fold higher risk of future severe exacerbations."}},{"doi":"10.1007/s10815-026-03930-1","pmid":"42321501","title":"Structural and functional characterization of DNAH5 variants in a Portuguese family with primary ciliary dyskinesia","journal_title":"Journal of Assisted Reproduction and Genetics","journal_issn":"1058-0468","published_at":"2026-06-19T00:00:00.000Z","url":"https://doi.org/10.1007/s10815-026-03930-1","source_score":1,"is_retracted":false,"is_open_access":true,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-06-25T13:25:17.591Z","updated_at":"2026-06-25T13:25:17.591Z","tags":[{"paper_doi":"10.1007/s10815-026-03930-1","tag_type":"age_group","value":"adult","value_numeric":null},{"paper_doi":"10.1007/s10815-026-03930-1","tag_type":"category","value":"Obstetrics & Gynecology","value_numeric":null},{"paper_doi":"10.1007/s10815-026-03930-1","tag_type":"condition","value":"Bronchiectasis","value_numeric":null},{"paper_doi":"10.1007/s10815-026-03930-1","tag_type":"condition","value":"Oligoasthenoteratozoospermia","value_numeric":null},{"paper_doi":"10.1007/s10815-026-03930-1","tag_type":"condition","value":"Primary Ciliary Dyskinesia","value_numeric":null},{"paper_doi":"10.1007/s10815-026-03930-1","tag_type":"generic","value":"DNAH5","value_numeric":null},{"paper_doi":"10.1007/s10815-026-03930-1","tag_type":"impact_flag","value":"limited_evidence","value_numeric":null},{"paper_doi":"10.1007/s10815-026-03930-1","tag_type":"level_of_evidence","value":"5","value_numeric":null},{"paper_doi":"10.1007/s10815-026-03930-1","tag_type":"modality","value":"Genetic Variant Characterization","value_numeric":null},{"paper_doi":"10.1007/s10815-026-03930-1","tag_type":"modality","value":"Immunofluorescence","value_numeric":null},{"paper_doi":"10.1007/s10815-026-03930-1","tag_type":"modality","value":"Transmission Electron Microscopy","value_numeric":null},{"paper_doi":"10.1007/s10815-026-03930-1","tag_type":"sample_size","value":"n=4","value_numeric":4},{"paper_doi":"10.1007/s10815-026-03930-1","tag_type":"sex","value":"mixed","value_numeric":null},{"paper_doi":"10.1007/s10815-026-03930-1","tag_type":"study_design","value":"Case Report / Family Study","value_numeric":null},{"paper_doi":"10.1007/s10815-026-03930-1","tag_type":"warning","value":"DNAH5 is tagged as 'generic' as it is a gene/protein target rather than a conventional drug; no pharmacologic therapy was evaluated in this study.","value_numeric":null},{"paper_doi":"10.1007/s10815-026-03930-1","tag_type":"warning","value":"Phenotypic variability between brothers (fertile vs. infertile) with identical genotypes raises the possibility of modifier gene effects, which remain unresolved.","value_numeric":null},{"paper_doi":"10.1007/s10815-026-03930-1","tag_type":"warning","value":"Sample size is very small (2 affected brothers, 2 carrier parents); conclusions about variant reclassification are supported by converging evidence but lack functional rescue validation.","value_numeric":null},{"paper_doi":"10.1007/s10815-026-03930-1","tag_type":"warning","value":"The study_design tag 'Case Report / Family Study' is used as this is a single-family case report with multi-modal characterization — not a traditional cohort or trial.","value_numeric":null}],"summaries":{"what_studied":"A Portuguese family was evaluated to characterize two compound heterozygous DNAH5 variants — a missense variant of uncertain significance (VUS) c.5290T>C p.(Ser1764Pro) and a pathogenic truncating variant c.4237C>T p.(Gln1413*) — in two brothers diagnosed with primary ciliary dyskinesia (PCD), using nasal nitric oxide, high-speed videomicroscopy (HSVM), transmission electron microscopy (TEM), whole-exome sequencing, immunofluorescence, and protein structural modeling.","key_result":"Both brothers carried compound heterozygous DNAH5 variants in trans; the proband (diagnosed at age 44) showed nasal nitric oxide of 30 nL/min, ciliary beat frequency of 0.66 Hz, 62.5% total ciliary immotility, class-1 ODA defects on TEM, and markedly reduced DNAH5 expression on immunofluorescence (p<0.0001). The brother (diagnosed at 48) had a similar phenotype including oligoasthenoteratozoospermia. Protein structural modeling showed the Ser1764Pro substitution destabilizes the Link3–4 β-sheet of the DNAH5 linker domain, supporting reclassification of c.5290T>C p.(Ser1764Pro) from VUS to pathogenic.","limitations":"- Very small family cohort (2 affected brothers + 2 carrier parents); findings may not generalize broadly.\n- TEM and detailed semen analysis were not performed in the brother or parents, limiting full phenotypic comparison.\n- Protein structural analysis is predictive (in silico/structural models) rather than confirmed by functional rescue experiments.","practice_takeaway":"When a DNAH5 VUS co-segregates in trans with a known pathogenic variant and both brothers show reduced DNAH5 immunofluorescence and classic PCD findings, integrate structural modeling and family immunofluorescence to support reclassification — this can enable inclusion in diagnostic panels and guide counseling. Consider PCD even with a low PICADAR score (≤5) and adult-onset presentation, as diagnosis may be delayed by decades."}},{"doi":"10.1038/s41584-026-01394-2","pmid":"42315602","title":"Lung disease in rheumatoid arthritis","journal_title":"Nature Reviews Rheumatology","journal_issn":"1759-4790","published_at":"2026-06-18T00:00:00.000Z","url":"https://doi.org/10.1038/s41584-026-01394-2","source_score":9,"is_retracted":false,"is_open_access":false,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-06-20T13:24:51.988Z","updated_at":"2026-06-20T13:24:51.988Z","tags":[{"paper_doi":"10.1038/s41584-026-01394-2","tag_type":"age_group","value":"adult","value_numeric":null},{"paper_doi":"10.1038/s41584-026-01394-2","tag_type":"category","value":"Rheumatology","value_numeric":null},{"paper_doi":"10.1038/s41584-026-01394-2","tag_type":"condition","value":"Bronchiectasis","value_numeric":null},{"paper_doi":"10.1038/s41584-026-01394-2","tag_type":"condition","value":"Bronchiolitis","value_numeric":null},{"paper_doi":"10.1038/s41584-026-01394-2","tag_type":"condition","value":"Chronic Obstructive Pulmonary Disease","value_numeric":null},{"paper_doi":"10.1038/s41584-026-01394-2","tag_type":"condition","value":"Interstitial Lung Disease","value_numeric":null},{"paper_doi":"10.1038/s41584-026-01394-2","tag_type":"condition","value":"Lung Cancer","value_numeric":null},{"paper_doi":"10.1038/s41584-026-01394-2","tag_type":"condition","value":"Rheumatoid Arthritis","value_numeric":null},{"paper_doi":"10.1038/s41584-026-01394-2","tag_type":"generic","value":"baricitinib","value_numeric":null},{"paper_doi":"10.1038/s41584-026-01394-2","tag_type":"generic","value":"brensocatib","value_numeric":null},{"paper_doi":"10.1038/s41584-026-01394-2","tag_type":"generic","value":"methotrexate","value_numeric":null},{"paper_doi":"10.1038/s41584-026-01394-2","tag_type":"generic","value":"nintedanib","value_numeric":null},{"paper_doi":"10.1038/s41584-026-01394-2","tag_type":"generic","value":"pirfenidone","value_numeric":null},{"paper_doi":"10.1038/s41584-026-01394-2","tag_type":"generic","value":"rituximab","value_numeric":null},{"paper_doi":"10.1038/s41584-026-01394-2","tag_type":"generic","value":"tocilizumab","value_numeric":null},{"paper_doi":"10.1038/s41584-026-01394-2","tag_type":"impact_flag","value":"practice_changing","value_numeric":null},{"paper_doi":"10.1038/s41584-026-01394-2","tag_type":"level_of_evidence","value":"5","value_numeric":null},{"paper_doi":"10.1038/s41584-026-01394-2","tag_type":"modality","value":"Antifibrotic","value_numeric":null},{"paper_doi":"10.1038/s41584-026-01394-2","tag_type":"modality","value":"DPP-1 Inhibitor","value_numeric":null},{"paper_doi":"10.1038/s41584-026-01394-2","tag_type":"modality","value":"Immunosuppressant","value_numeric":null},{"paper_doi":"10.1038/s41584-026-01394-2","tag_type":"sex","value":"mixed","value_numeric":null},{"paper_doi":"10.1038/s41584-026-01394-2","tag_type":"study_design","value":"Narrative Review","value_numeric":null},{"paper_doi":"10.1038/s41584-026-01394-2","tag_type":"warning","value":"Full text of the article was not accessible; summary and tags are derived from the abstract, key points, glossary, and reference list only — specific prevalence figures, hazard ratios, and detailed management algorithms could not be verified.","value_numeric":null},{"paper_doi":"10.1038/s41584-026-01394-2","tag_type":"warning","value":"Study design is classified as 'Narrative Review' rather than 'Systematic Review' based on the absence of reported search methodology or PRISMA-style data synthesis.","value_numeric":null},{"paper_doi":"10.1038/s41584-026-01394-2","tag_type":"warning","value":"The 'practice_changing' impact flag reflects the review's synthesis of evolving screening and antifibrotic treatment recommendations, not a single landmark trial result.","value_numeric":null}],"summaries":{"what_studied":"This narrative review evaluates the full spectrum of pulmonary manifestations in rheumatoid arthritis (RA), including ILD, COPD, bronchiectasis, bronchiolitis, pleural disease, and lung cancer, with a focus on pathophysiology, screening strategies, and management approaches.","key_result":"RA-ILD (especially usual interstitial pneumonia pattern) carries the worst prognosis among RA lung manifestations. Key risk factors for RA-ILD include male sex, older age, ACPA seropositivity, smoking, and high disease activity. Evidence-based treatments remain limited, but antifibrotics (nintedanib, pirfenidone) can be considered for progressive fibrotic phenotypes. COPD and bronchiectasis significantly raise infection risk and mortality in RA and are underrecognized.","limitations":"- Full text was not accessible; summary is based on the abstract, key points, and reference list only, limiting depth of evidence appraisal.\n- As a narrative review, it does not perform systematic pooled analysis, so estimates of effect size or prevalence are not consistently reported.\n- Evidence-based treatment recommendations for RA-ILD remain sparse, reflecting the limited RCT data in this population.","practice_takeaway":"Screen RA patients for ILD using HRCT when risk factors (male sex, older age, ACPA positivity, smoking, high disease activity) are present. For progressive fibrosing RA-ILD, consider antifibrotics (nintedanib or pirfenidone) alongside a multidisciplinary approach that includes smoking cessation, vaccinations, and pulmonary rehabilitation."}},{"doi":"10.1002/ppul.71705","pmid":"42313717","title":"The Relationship Between Structural and Functional Pulmonary Status and Quality of Life in Children With Primary Ciliary Dyskinesia","journal_title":"Pediatric Pulmonology","journal_issn":"8755-6863","published_at":"2026-06-18T00:00:00.000Z","url":"https://pmc.ncbi.nlm.nih.gov/articles/PMC13278357/","source_score":2,"is_retracted":false,"is_open_access":true,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-06-22T13:24:01.200Z","updated_at":"2026-06-22T13:24:01.200Z","tags":[{"paper_doi":"10.1002/ppul.71705","tag_type":"age_group","value":"pediatric","value_numeric":null},{"paper_doi":"10.1002/ppul.71705","tag_type":"category","value":"Respiratory System","value_numeric":null},{"paper_doi":"10.1002/ppul.71705","tag_type":"condition","value":"Bronchiectasis","value_numeric":null},{"paper_doi":"10.1002/ppul.71705","tag_type":"condition","value":"Primary Ciliary Dyskinesia","value_numeric":null},{"paper_doi":"10.1002/ppul.71705","tag_type":"impact_flag","value":"limited_evidence","value_numeric":null},{"paper_doi":"10.1002/ppul.71705","tag_type":"level_of_evidence","value":"4","value_numeric":null},{"paper_doi":"10.1002/ppul.71705","tag_type":"modality","value":"High-Resolution Computed Tomography","value_numeric":null},{"paper_doi":"10.1002/ppul.71705","tag_type":"modality","value":"Pulmonary Function Testing","value_numeric":null},{"paper_doi":"10.1002/ppul.71705","tag_type":"modality","value":"Quality of Life Assessment","value_numeric":null},{"paper_doi":"10.1002/ppul.71705","tag_type":"sample_size","value":"n=30","value_numeric":30},{"paper_doi":"10.1002/ppul.71705","tag_type":"sex","value":"mixed","value_numeric":null},{"paper_doi":"10.1002/ppul.71705","tag_type":"study_design","value":"Cross-Sectional Study","value_numeric":null},{"paper_doi":"10.1002/ppul.71705","tag_type":"warning","value":"Genetic confirmation was the sole diagnostic criterion (nasal NO and electron microscopy not routinely available), which may introduce selection bias toward specific genotypes.","value_numeric":null},{"paper_doi":"10.1002/ppul.71705","tag_type":"warning","value":"SGRQ was used off-label in a pediatric PCD population — it was originally developed for COPD adults and lacks PCD-specific validation in children; results should be interpreted with caution.","value_numeric":null},{"paper_doi":"10.1002/ppul.71705","tag_type":"warning","value":"Study is single-center, retrospective, and small (n=30); some PFT subtests were missing for up to 8 patients, which may affect correlation reliability.","value_numeric":null}],"summaries":{"what_studied":"This retrospective cross-sectional study examined the relationships among lung function (spirometry, plethysmography, DLCO), structural lung damage (modified Bhalla CT score), and quality of life (SGRQ) in 30 genetically confirmed PCD children (median age 11.6 years) evaluated during clinically stable periods over a 1-year window.","key_result":"SGRQ total scores correlated negatively with FEV1/FVC (r = –0.502, p = 0.025) and FEF25-75 (r = –0.519, p = 0.016), and positively with exacerbation frequency. Modified Bhalla scores correlated with FEV1 (r = –0.637, p = 0.008), FVC, FEF25-75, and FEV1/FVC, but **not** with SGRQ scores. Mild obstruction was present in 48%, moderate in 24%, and severe in 20%; 80% had mild and 20% moderate structural damage on HRCT.","limitations":"- Small sample (n = 30) from a single tertiary center limits generalizability.\n- SGRQ was not designed or validated for pediatric PCD; QOL-PCD was intentionally excluded.\n- Cross-sectional design prevents causal or temporal inferences about the sequence of structural, functional, and QoL changes.","practice_takeaway":"In children with PCD, patient-reported symptom burden (SGRQ) tracks with spirometric decline and exacerbation frequency, but not with CT structural scores — so PFT, imaging, and QoL tools each capture distinct information and should be used together. Clinicians should not rely on spirometry alone to gauge disease progression, as structural damage may precede functional change."}},{"doi":"10.1038/s41533-026-00528-z","pmid":"42259823","title":"The European respiratory society guideline for adult bronchiectasis 2025: summary and implementation guide for primary care","journal_title":"npj Primary Care Respiratory Medicine","journal_issn":"2055-1010","published_at":"2026-06-08T00:00:00.000Z","url":"https://doi.org/10.1038/s41533-026-00528-z","source_score":5,"is_retracted":false,"is_open_access":true,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-06-15T13:21:58.604Z","updated_at":"2026-06-15T13:21:58.604Z","tags":[{"paper_doi":"10.1038/s41533-026-00528-z","tag_type":"age_group","value":"adult","value_numeric":null},{"paper_doi":"10.1038/s41533-026-00528-z","tag_type":"category","value":"Primary Health Care","value_numeric":null},{"paper_doi":"10.1038/s41533-026-00528-z","tag_type":"category","value":"Respiratory System","value_numeric":null},{"paper_doi":"10.1038/s41533-026-00528-z","tag_type":"condition","value":"Allergic Bronchopulmonary Aspergillosis","value_numeric":null},{"paper_doi":"10.1038/s41533-026-00528-z","tag_type":"condition","value":"Bronchiectasis","value_numeric":null},{"paper_doi":"10.1038/s41533-026-00528-z","tag_type":"condition","value":"Non-Tuberculous Mycobacterial Infection","value_numeric":null},{"paper_doi":"10.1038/s41533-026-00528-z","tag_type":"impact_flag","value":"practice_changing","value_numeric":null},{"paper_doi":"10.1038/s41533-026-00528-z","tag_type":"level_of_evidence","value":"1","value_numeric":null},{"paper_doi":"10.1038/s41533-026-00528-z","tag_type":"modality","value":"Airway Clearance Techniques","value_numeric":null},{"paper_doi":"10.1038/s41533-026-00528-z","tag_type":"modality","value":"Inhaled Corticosteroid","value_numeric":null},{"paper_doi":"10.1038/s41533-026-00528-z","tag_type":"modality","value":"Long-Term Antibiotic Therapy","value_numeric":null},{"paper_doi":"10.1038/s41533-026-00528-z","tag_type":"modality","value":"Pulmonary Rehabilitation","value_numeric":null},{"paper_doi":"10.1038/s41533-026-00528-z","tag_type":"study_design","value":"Clinical Practice Guideline Summary","value_numeric":null},{"paper_doi":"10.1038/s41533-026-00528-z","tag_type":"warning","value":"Study design tag 'Clinical Practice Guideline Summary' used as this is a primary care implementation guide for an ERS guideline, not a classic systematic review or RCT.","value_numeric":null},{"paper_doi":"10.1038/s41533-026-00528-z","tag_type":"warning","value":"The article was published in 2026 but references the December 2025 ERS guideline; specific recommendation grades from the underlying guideline are not reproduced here.","value_numeric":null},{"paper_doi":"10.1038/s41533-026-00528-z","tag_type":"warning","value":"This is a guideline implementation summary, not an original research study — no primary data or sample size applies. Level of evidence reflects the ERS guideline source documents, not this article independently.","value_numeric":null}],"summaries":{"what_studied":"This article summarises the 2025 European Respiratory Society (ERS) guidelines for adult bronchiectasis, with a focus on translating specialist recommendations into primary care practice — covering diagnosis, investigation, management, and exacerbation care.","key_result":"Key priorities include reducing diagnostic delay, standardising investigations (sputum microbiology, immunoglobulins, ABPA serology, NTM testing), and applying evidence-based management (airway clearance, pulmonary rehabilitation, judicious antibiotic use); inhaled corticosteroids have a limited role in bronchiectasis, unlike in asthma or COPD.","limitations":"This is a guideline summary/implementation guide rather than an original research study, so it does not generate new clinical data. The recommendations reflect evidence quality from the underlying ERS guideline, which is not independently assessed here.","practice_takeaway":"Primary care clinicians should consider bronchiectasis early in patients with chronic productive cough, initiate standardised blood and sputum investigations, and refer promptly when poor prognostic features are present. Long-term antibiotic therapy (e.g., for *Pseudomonas aeruginosa* infection) and airway clearance should be coordinated with specialist input, and inhaled corticosteroids should not be routinely used."}},{"doi":"10.1002/resp.70268","pmid":"42240484","title":"Walking With Huffing: A Practical New Option for Airway Clearance During Bronchiectasis Exacerbations?","journal_title":"Respirology","journal_issn":"1323-7799","published_at":"2026-06-04T00:00:00.000Z","url":"https://doi.org/10.1002/resp.70268","source_score":1,"is_retracted":false,"is_open_access":false,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-06-10T14:23:57.276Z","updated_at":"2026-06-10T14:23:57.276Z","tags":[{"paper_doi":"10.1002/resp.70268","tag_type":"category","value":"Respiratory System","value_numeric":null},{"paper_doi":"10.1002/resp.70268","tag_type":"condition","value":"Bronchiectasis","value_numeric":null},{"paper_doi":"10.1002/resp.70268","tag_type":"modality","value":"Airway Clearance Technique","value_numeric":null},{"paper_doi":"10.1002/resp.70268","tag_type":"warning","value":"Only the paper title, DOI, journal, and publication date were provided — no abstract, methods, results, or conclusions were available. All summary fields requiring specific data (key result, limitations, practice takeaway) have been set to null as they cannot be verified from the available text. Full-text access is needed for a complete structured summary.","value_numeric":null}],"summaries":{"what_studied":"This paper evaluates walking combined with huffing as an airway clearance technique during bronchiectasis exacerbations, likely compared to standard airway clearance methods."}},{"doi":"10.1183/23120541.00388-2026","pmid":null,"title":"Initiation of inhaled antibiotic therapy in chronic airway diseases: the REPAIR registry","journal_title":"ERJ Open Research","journal_issn":"2312-0541","published_at":"2026-05-28T00:00:00.000Z","url":"https://doi.org/10.1183/23120541.00388-2026","source_score":1,"is_retracted":false,"is_open_access":true,"raw_metadata":{"pubmed":false,"crossref":true,"unpaywall":true},"created_at":"2026-06-06T13:22:58.613Z","updated_at":"2026-06-06T13:22:58.613Z","tags":[{"paper_doi":"10.1183/23120541.00388-2026","tag_type":"age_group","value":"older_adult","value_numeric":null},{"paper_doi":"10.1183/23120541.00388-2026","tag_type":"category","value":"Respiratory System","value_numeric":null},{"paper_doi":"10.1183/23120541.00388-2026","tag_type":"condition","value":"Bronchiectasis","value_numeric":null},{"paper_doi":"10.1183/23120541.00388-2026","tag_type":"condition","value":"Chronic Airway Disease","value_numeric":null},{"paper_doi":"10.1183/23120541.00388-2026","tag_type":"condition","value":"Chronic Bronchial Infection","value_numeric":null},{"paper_doi":"10.1183/23120541.00388-2026","tag_type":"generic","value":"colistimethate","value_numeric":null},{"paper_doi":"10.1183/23120541.00388-2026","tag_type":"impact_flag","value":"limited_evidence","value_numeric":null},{"paper_doi":"10.1183/23120541.00388-2026","tag_type":"level_of_evidence","value":"3","value_numeric":null},{"paper_doi":"10.1183/23120541.00388-2026","tag_type":"modality","value":"Inhaled Antibiotic","value_numeric":null},{"paper_doi":"10.1183/23120541.00388-2026","tag_type":"sample_size","value":"n=411","value_numeric":411},{"paper_doi":"10.1183/23120541.00388-2026","tag_type":"sex","value":"mixed","value_numeric":null},{"paper_doi":"10.1183/23120541.00388-2026","tag_type":"study_design","value":"Prospective Registry (Cross-Sectional Baseline Analysis)","value_numeric":null},{"paper_doi":"10.1183/23120541.00388-2026","tag_type":"warning","value":"Level of evidence set to 3 (observational registry); no comparator arm or randomisation present.","value_numeric":null},{"paper_doi":"10.1183/23120541.00388-2026","tag_type":"warning","value":"The registry is described as 'national' but the country is not explicitly named in the abstract — findings may not be generalisable internationally.","value_numeric":null},{"paper_doi":"10.1183/23120541.00388-2026","tag_type":"warning","value":"The study is a cross-sectional baseline analysis of a registry — longitudinal outcomes (e.g., exacerbation reduction, eradication rates) are not yet reported and should not be inferred.","value_numeric":null},{"paper_doi":"10.1183/23120541.00388-2026","tag_type":"warning","value":"Underlying airway disease phenotypes (e.g., bronchiectasis, COPD, others) are referenced but not individually enumerated in the abstract — condition tags reflect the dominant stated phenotypes.","value_numeric":null}],"summaries":{"what_studied":"A prospective, multicentre national registry (REPAIR) enrolled 411 adult patients with chronic airway diseases (mean age 67.9 years; 57.2% women) at the moment of inhaled antibiotic therapy (IAT) initiation, describing clinical, microbiological, and prescribing characteristics in routine practice.","key_result":"*Pseudomonas aeruginosa* was isolated in 79.8% of patients; colistimethate was the most prescribed IAT (78.6%), strongly tied to *P. aeruginosa* isolation. IAT was initiated during exacerbation or early convalescence in 44.3% of cases. Lung function, exacerbation burden, and chronic bronchial infection (CBI) status did not significantly drive antibiotic class selection — microbiology did.","limitations":"Cross-sectional baseline analysis only — no outcomes data yet reported. Single-country (national) registry limits generalisability. Prescribing patterns reflect real-world variation without a control group or randomisation.","practice_takeaway":"In routine practice, IAT initiation is primarily microbiology-driven (especially *P. aeruginosa*), not guided by lung function or exacerbation burden — clinicians should be aware that nearly half of patients start IAT during acute illness or early recovery, which may affect tolerability and adherence assessment."}},{"doi":"10.1183/13993003.00012-2026","pmid":"42167772","title":"Mucoactive therapies and the European Respiratory Society guideline for adult bronchiectasis: what now after the CLEAR trial?","journal_title":"European Respiratory Journal","journal_issn":"0903-1936","published_at":"2026-05-21T00:00:00.000Z","url":"https://doi.org/10.1183/13993003.00012-2026","source_score":4,"is_retracted":false,"is_open_access":false,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-05-28T13:24:28.032Z","updated_at":"2026-05-28T13:24:28.032Z","tags":[{"paper_doi":"10.1183/13993003.00012-2026","tag_type":"age_group","value":"adult","value_numeric":null},{"paper_doi":"10.1183/13993003.00012-2026","tag_type":"category","value":"Respiratory System","value_numeric":null},{"paper_doi":"10.1183/13993003.00012-2026","tag_type":"condition","value":"Bronchiectasis","value_numeric":null},{"paper_doi":"10.1183/13993003.00012-2026","tag_type":"impact_flag","value":"practice_changing","value_numeric":null},{"paper_doi":"10.1183/13993003.00012-2026","tag_type":"level_of_evidence","value":"7","value_numeric":null},{"paper_doi":"10.1183/13993003.00012-2026","tag_type":"modality","value":"Mucoactive Therapy","value_numeric":null},{"paper_doi":"10.1183/13993003.00012-2026","tag_type":"study_design","value":"Narrative Review / Editorial","value_numeric":null},{"paper_doi":"10.1183/13993003.00012-2026","tag_type":"warning","value":"Full text of this article was not available for analysis — only title, DOI, journal, and publication date were provided. All summary content is inferred from metadata only. Key results, sample sizes, and specific recommendations could not be verified. Users should consult the full publication before making clinical decisions.","value_numeric":null},{"paper_doi":"10.1183/13993003.00012-2026","tag_type":"warning","value":"Study design tagged as 'Narrative Review / Editorial' based on the commentary framing in the title; actual design (e.g., systematic review, guideline commentary) is unconfirmed.","value_numeric":null},{"paper_doi":"10.1183/13993003.00012-2026","tag_type":"warning","value":"The 'practice_changing' impact flag is tentative — the paper appears to contextualize a major trial against existing guidelines, which is typically high clinical relevance, but this cannot be confirmed without full text.","value_numeric":null}],"summaries":{"what_studied":"This paper examines mucoactive therapies (e.g., inhaled hyperosmolar agents, mucolytics) in adult bronchiectasis in the context of the European Respiratory Society (ERS) guidelines and the recently published CLEAR trial, evaluating how the trial's findings should inform clinical practice.","key_result":"The paper discusses how the CLEAR trial results interact with existing ERS guideline recommendations for mucoactive therapies in adult bronchiectasis, but specific quantitative outcomes are not extractable from the abstract/metadata alone.","limitations":"Full text not available for analysis; summary is based on title, journal, and DOI metadata only — specific findings, sample sizes, and quantitative results cannot be verified.","practice_takeaway":"Clinicians managing adult bronchiectasis should reassess their use of mucoactive therapies in light of both the CLEAR trial data and updated ERS guidance, though specific actionable changes require review of the full publication."}},{"doi":"10.1152/ajplung.00321.2025","pmid":"42166638","title":"Impaired mucociliary clearance in bronchiectasis: the result of a differentiation-delayed airway epithelium?","journal_title":"American Journal of Physiology-Lung Cellular and Molecular Physiology","journal_issn":"1040-0605","published_at":"2026-05-21T00:00:00.000Z","url":"https://doi.org/10.1152/ajplung.00321.2025","source_score":2,"is_retracted":false,"is_open_access":true,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-06-29T13:22:46.917Z","updated_at":"2026-06-29T13:22:46.917Z","tags":[{"paper_doi":"10.1152/ajplung.00321.2025","tag_type":"age_group","value":"adult","value_numeric":null},{"paper_doi":"10.1152/ajplung.00321.2025","tag_type":"category","value":"Respiratory System","value_numeric":null},{"paper_doi":"10.1152/ajplung.00321.2025","tag_type":"condition","value":"Bronchiectasis","value_numeric":null},{"paper_doi":"10.1152/ajplung.00321.2025","tag_type":"impact_flag","value":"limited_evidence","value_numeric":null},{"paper_doi":"10.1152/ajplung.00321.2025","tag_type":"level_of_evidence","value":"5","value_numeric":null},{"paper_doi":"10.1152/ajplung.00321.2025","tag_type":"modality","value":"Mucociliary Clearance Assessment","value_numeric":null},{"paper_doi":"10.1152/ajplung.00321.2025","tag_type":"study_design","value":"In Vitro Experimental Study","value_numeric":null},{"paper_doi":"10.1152/ajplung.00321.2025","tag_type":"warning","value":"Sample size (number of donors) is not reported in the abstract; total N could not be tagged.","value_numeric":null},{"paper_doi":"10.1152/ajplung.00321.2025","tag_type":"warning","value":"Sex of donors is not reported in the abstract.","value_numeric":null},{"paper_doi":"10.1152/ajplung.00321.2025","tag_type":"warning","value":"Study design is in vitro only—findings on differentiation delay have not been confirmed in vivo or in clinical cohorts.","value_numeric":null},{"paper_doi":"10.1152/ajplung.00321.2025","tag_type":"warning","value":"The NewCAM scoring system is novel and not yet externally validated; results depend on this unvalidated tool.","value_numeric":null}],"summaries":{"what_studied":"This study examined whether bronchial epithelial cells from bronchiectasis donors show impaired mucociliary clearance (MCC) compared to healthy donors, using a novel four-parameter scoring system (NewCAM) and single-cell RNA sequencing in air-liquid interface (ALI) cultures over 28 days.","key_result":"Bronchiectasis donor cultures had significantly lower active ciliated area (P < 0.05) and apical particle velocity (P < 0.05) vs. healthy donors; scRNA-seq showed more immature basal cell types and fewer terminally differentiated ciliated and secretory cells, consistent with a differentiation delay.","limitations":"Results are from in vitro ALI cultures, which may not fully replicate the in vivo airway environment; the study does not establish causality for the differentiation delay; sample size is not reported in the abstract.","practice_takeaway":"Bronchiectasis airways may have an intrinsic epithelial differentiation defect that impairs MCC—this points to epithelial maturation as a potential therapeutic target, though clinical translation requires further in vivo validation."}},{"doi":"10.1016/s2213-2600(26)00057-3","pmid":"42155496","title":"Comorbid diabetes disease severity and microbial changes in patients with bronchiectasis: a combined analysis of data from the EMBARC, EMBARC-India, Australian, and BE-China registries","journal_title":"The Lancet Respiratory Medicine","journal_issn":"2213-2600","published_at":"2026-05-19T00:00:00.000Z","url":"https://doi.org/10.1016/s2213-2600(26)00057-3","source_score":7,"is_retracted":false,"is_open_access":true,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-05-23T13:24:49.230Z","updated_at":"2026-05-23T13:24:49.230Z","tags":[{"paper_doi":"10.1016/s2213-2600(26)00057-3","tag_type":"age_group","value":"adult","value_numeric":null},{"paper_doi":"10.1016/s2213-2600(26)00057-3","tag_type":"age_group","value":"older_adult","value_numeric":null},{"paper_doi":"10.1016/s2213-2600(26)00057-3","tag_type":"category","value":"Respiratory System","value_numeric":null},{"paper_doi":"10.1016/s2213-2600(26)00057-3","tag_type":"condition","value":"Bronchiectasis","value_numeric":null},{"paper_doi":"10.1016/s2213-2600(26)00057-3","tag_type":"condition","value":"Type 1 Diabetes Mellitus","value_numeric":null},{"paper_doi":"10.1016/s2213-2600(26)00057-3","tag_type":"condition","value":"Type 2 Diabetes Mellitus","value_numeric":null},{"paper_doi":"10.1016/s2213-2600(26)00057-3","tag_type":"generic","value":"metformin","value_numeric":null},{"paper_doi":"10.1016/s2213-2600(26)00057-3","tag_type":"impact_flag","value":"practice_changing","value_numeric":null},{"paper_doi":"10.1016/s2213-2600(26)00057-3","tag_type":"level_of_evidence","value":"2","value_numeric":null},{"paper_doi":"10.1016/s2213-2600(26)00057-3","tag_type":"modality","value":"Microbiome Analysis","value_numeric":null},{"paper_doi":"10.1016/s2213-2600(26)00057-3","tag_type":"modality","value":"Serum Proteomics","value_numeric":null},{"paper_doi":"10.1016/s2213-2600(26)00057-3","tag_type":"sample_size","value":"n=30263","value_numeric":30263},{"paper_doi":"10.1016/s2213-2600(26)00057-3","tag_type":"sex","value":"mixed","value_numeric":null},{"paper_doi":"10.1016/s2213-2600(26)00057-3","tag_type":"study_design","value":"Prospective Observational Registry Cohort Study","value_numeric":null},{"paper_doi":"10.1016/s2213-2600(26)00057-3","tag_type":"warning","value":"Cause of death was not recorded, so the mortality excess attributed to diabetes may include non-respiratory causes.","value_numeric":null},{"paper_doi":"10.1016/s2213-2600(26)00057-3","tag_type":"warning","value":"Diabetes type (type 1 vs type 2) was not recorded in BE-China, limiting subgroup generalisability; 92.5% of typed cases were type 2.","value_numeric":null},{"paper_doi":"10.1016/s2213-2600(26)00057-3","tag_type":"warning","value":"Metformin analysis was observational only—no randomised data exist to confirm or refute a treatment effect in bronchiectasis.","value_numeric":null},{"paper_doi":"10.1016/s2213-2600(26)00057-3","tag_type":"warning","value":"Microbiome and Olink proteomic analyses were conducted in a small EMBARC-BRIDGE sub-cohort (n=433 and n=479 respectively), which may limit statistical power and generalisability.","value_numeric":null}],"summaries":{"what_studied":"This registry-based cohort study evaluated the impact of comorbid diabetes on clinical outcomes, microbiome, and inflammatory profiles in 30,263 adults with CT-confirmed non-cystic fibrosis bronchiectasis across 33 countries (EMBARC, EMBARC-India, BE-China, ABR registries), with long-term follow-up available in 11,153 patients from EMBARC and EMBARC-India.","key_result":"8.2% (2,487/30,263) of patients had diabetes. After adjustment for confounders, diabetes was associated with more frequent exacerbations (IRR 1.18, 95% CI 1.09–1.28), more hospital admissions (IRR 1.57, 1.40–1.76), and 80% higher 5-year all-cause mortality (HR 1.80, 95% CI 1.53–2.12; all p<0.0001). Patients with diabetes also had significantly more cardiovascular comorbidities (53.5% vs 21.8%), higher BSI scores (median 8 vs 7), worse dyspnoea, lower FEV1% predicted (67.1% vs 73.7%), and increased sputum isolation of Enterobacteriaceae, Moraxella catarrhalis, and Haemophilus influenzae.","limitations":"Observational design limits causal inference; missing data in sputum cultures, treatment records, and diabetes type varied by registry; HbA1c and glycaemic control were not recorded; cause of death was not captured; microbiome and proteomic sub-cohorts were small (n=433 and n=479).","practice_takeaway":"Clinicians should treat diabetes as a high-risk comorbidity in bronchiectasis and include it in individualised risk stratification—consistent with the 2025 ERS bronchiectasis guidelines—given its independent association with 80% higher 5-year mortality and 57% more hospitalisations. Vigilance for Enterobacteriaceae and Haemophilus influenzae infections is warranted in patients with both conditions."}},{"doi":"10.1002/resp.70265","pmid":"42155584","title":"Active Cycle of Breathing Technique Versus Oscillating Positive Expiratory Pressure Therapy Versus Exercise With Huffing During an Exacerbation of Bronchiectasis: A Randomised, Controlled Trial","journal_title":"Respirology","journal_issn":"1323-7799","published_at":"2026-05-19T00:00:00.000Z","url":"https://doi.org/10.1002/resp.70265","source_score":2,"is_retracted":false,"is_open_access":true,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-05-22T13:22:18.610Z","updated_at":"2026-05-22T13:22:18.610Z","tags":[{"paper_doi":"10.1002/resp.70265","tag_type":"age_group","value":"adult","value_numeric":null},{"paper_doi":"10.1002/resp.70265","tag_type":"category","value":"Respiratory System","value_numeric":null},{"paper_doi":"10.1002/resp.70265","tag_type":"condition","value":"Bronchiectasis","value_numeric":null},{"paper_doi":"10.1002/resp.70265","tag_type":"impact_flag","value":"confirms_prior","value_numeric":null},{"paper_doi":"10.1002/resp.70265","tag_type":"level_of_evidence","value":"2","value_numeric":null},{"paper_doi":"10.1002/resp.70265","tag_type":"modality","value":"Airway Clearance Technique","value_numeric":null},{"paper_doi":"10.1002/resp.70265","tag_type":"modality","value":"Oscillating Positive Expiratory Pressure Therapy","value_numeric":null},{"paper_doi":"10.1002/resp.70265","tag_type":"sample_size","value":"n=55","value_numeric":55},{"paper_doi":"10.1002/resp.70265","tag_type":"sex","value":"mixed","value_numeric":null},{"paper_doi":"10.1002/resp.70265","tag_type":"study_design","value":"Randomized Controlled Trial","value_numeric":null},{"paper_doi":"10.1002/resp.70265","tag_type":"warning","value":"No brand names for OPEP devices are specified; findings may not generalise across all OPEP device types.","value_numeric":null},{"paper_doi":"10.1002/resp.70265","tag_type":"warning","value":"The time-to-first-exacerbation analysis at 6 months is exploratory and affected by substantial loss to follow-up; treat those findings as hypothesis-generating only.","value_numeric":null},{"paper_doi":"10.1002/resp.70265","tag_type":"warning","value":"The trial was registered in 2021 but published in 2026 — a long gap that may reflect recruitment or publication delays; no protocol deviations are noted in the abstract.","value_numeric":null}],"summaries":{"what_studied":"This RCT compared three airway clearance techniques (ACTs) — ACBT, oscillating positive expiratory pressure (OPEP) therapy, and walking with huffing — in 55 adults hospitalised with an acute exacerbation of bronchiectasis, assessing sputum expectoration, health-related quality of life (HRQoL), and time to first re-exacerbation over 6 months post-discharge.","key_result":"ACBT and walking with huffing both significantly improved HRQoL (LCQ total score and multiple QoL-B domains) by discharge; OPEP produced minimal HRQoL changes. Sputum wet weights were largely similar across groups, except ACBT yielded 2.58 g less than walking with huffing 1-hour post-session on discharge day. ACBT had the longest median time to re-exacerbation (158 days, 95% CI 139–177) vs. walking with huffing (118 days, 95% CI 83–154) and OPEP (89 days, 95% CI 69–111).","limitations":"- Small sample (n=55) limits statistical power for between-group comparisons.\n- Time-to-exacerbation data should be interpreted with caution due to significant loss to follow-up at 6 months.\n- Blinding of participants and therapists was not feasible given the nature of the interventions.","practice_takeaway":"Walking with huffing is a simple, equipment-free alternative to ACBT that delivers comparable sputum clearance and HRQoL gains during a bronchiectasis exacerbation — clinicians can confidently offer it when ACBT is not tolerated or feasible. OPEP therapy showed less benefit on HRQoL in this acute inpatient setting and may warrant reassessment as first-line during exacerbations."}},{"doi":"10.1002/ppul.71670","pmid":"42153676","title":"A Novel PCD Phenotype: Inherent Mucus Hypersecretion by Patient‐Derived Airway Epithelial Cells","journal_title":"Pediatric Pulmonology","journal_issn":"8755-6863","published_at":"2026-05-19T00:00:00.000Z","url":"https://doi.org/10.1002/ppul.71670","source_score":2,"is_retracted":false,"is_open_access":true,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-05-27T15:42:58.410Z","updated_at":"2026-05-27T15:42:58.410Z","tags":[{"paper_doi":"10.1002/ppul.71670","tag_type":"age_group","value":"adolescent","value_numeric":null},{"paper_doi":"10.1002/ppul.71670","tag_type":"brand","value":"Dupixent","value_numeric":null},{"paper_doi":"10.1002/ppul.71670","tag_type":"category","value":"Respiratory System","value_numeric":null},{"paper_doi":"10.1002/ppul.71670","tag_type":"condition","value":"Bronchiectasis","value_numeric":null},{"paper_doi":"10.1002/ppul.71670","tag_type":"condition","value":"Mucus Hypersecretion","value_numeric":null},{"paper_doi":"10.1002/ppul.71670","tag_type":"condition","value":"Primary Ciliary Dyskinesia","value_numeric":null},{"paper_doi":"10.1002/ppul.71670","tag_type":"generic","value":"dupilumab","value_numeric":null},{"paper_doi":"10.1002/ppul.71670","tag_type":"impact_flag","value":"limited_evidence","value_numeric":null},{"paper_doi":"10.1002/ppul.71670","tag_type":"level_of_evidence","value":"5","value_numeric":null},{"paper_doi":"10.1002/ppul.71670","tag_type":"modality","value":"Biologic Agent","value_numeric":null},{"paper_doi":"10.1002/ppul.71670","tag_type":"sample_size","value":"n=1","value_numeric":1},{"paper_doi":"10.1002/ppul.71670","tag_type":"sex","value":"female","value_numeric":null},{"paper_doi":"10.1002/ppul.71670","tag_type":"study_design","value":"Case Report","value_numeric":null},{"paper_doi":"10.1002/ppul.71670","tag_type":"warning","value":"Age group tagged as 'adolescent' though the patient is 19 years old; may be considered a young adult depending on classification system used.","value_numeric":null},{"paper_doi":"10.1002/ppul.71670","tag_type":"warning","value":"Dupilumab is mentioned only as a speculative clinical implication, not as a treatment administered in this case.","value_numeric":null},{"paper_doi":"10.1002/ppul.71670","tag_type":"warning","value":"Single-patient case report; findings are hypothesis-generating and cannot establish causality between RSPH4A genotype and goblet-cell predominance.","value_numeric":null},{"paper_doi":"10.1002/ppul.71670","tag_type":"warning","value":"The patient's prior chronic infections and BAL procedures may have epigenetically altered epithelial differentiation, making it difficult to attribute in vitro findings solely to intrinsic genetic effects.","value_numeric":null}],"summaries":{"what_studied":"This case report describes a 19-year-old woman with PCD (RSPH4A mutations) whose clinical course featured mucus hypersecretion disproportionate to her bronchiectasis burden; nasal-brushing-derived airway basal cells were differentiated at air–liquid interface (ALI) for 35 days and analyzed by confocal imaging to correlate in vitro epithelial phenotype with clinical history.","key_result":"ALI cultures from the patient showed profound ciliogenesis defect with very few multiciliated cells, goblet cell predominance, reduced epithelial height, disorganized actin, and nuclear accumulation of RSPH4A (absent ciliary localization) — all more severe than her sibling with the same genotype and healthy controls; FEV1 declined to 35% predicted during MAI infection and recovered to 82% with treatment.","limitations":"Single patient case report limits generalizability; prior chronic infection and inflammation in vivo may have epigenetically reprogrammed the epithelial cells, confounding interpretation of intrinsic genotype effects; sibling comparison is limited to one individual with a partially different genotype (additional DNAI1 variant).","practice_takeaway":"PCD patients with RSPH4A mutations may harbor an inherent goblet-cell/mucus-hypersecretion phenotype beyond ciliary dysfunction; clinicians should consider this when managing refractory airway secretions, and biologic agents such as dupilumab targeting mucus hypersecretion and airway inflammation may warrant evaluation in this subset."}},{"doi":"10.1016/s2213-2600(25)00334-0","pmid":"42044645","title":"Azurocidin-1 as a mediator of bronchiectasis severity, epithelial defence, and target of dipeptidyl peptidase-1 inhibition: an international, multicohort study","journal_title":"The Lancet Respiratory Medicine","journal_issn":"2213-2600","published_at":"2026-04-28T00:00:00.000Z","url":"https://doi.org/10.1016/s2213-2600(25)00334-0","source_score":20,"is_retracted":false,"is_open_access":true,"raw_metadata":{"ooir":{"day":"2026-04-30","doi":"10.1016/s2213-2600(25)00334-0","days":"14","issn":"2213-2600","rank":"1","field":"Clinical Medicine","score":"20","category":"Respiratory System","categories":["Respiratory System"],"_categories":["Respiratory System"]},"pubmed":{"doi":"10.1016/S2213-2600(25)00334-0","title":"Azurocidin-1 as a mediator of bronchiectasis severity, epithelial defence, and target of dipeptidyl peptidase-1 inhibition: an international, multicohort study.","journal":"The Lancet. 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reflects the identification of a novel, measurable biomarker and its therapeutic targetability, though clinical adoption depends on validation of brensocatib in phase 3 trials (ASPEN trial results pending at time of publication).","value_numeric":null},{"paper_doi":"10.1016/s2213-2600(25)00334-0","tag_type":"warning","value":"The total sample size of 762 is an approximation summed across distinct cohorts (EMBARC cohort 1 n=197, EMBARC cohort 2 n=144, COPD cohort n=101, rhinovirus challenge n=9, WILLOW trial n=215); cohorts were not pooled for a single analysis. The rhinovirus challenge sub-study is extremely small (n=9) and findings should be interpreted with caution. Study design tag reflects a hybrid of observational and post-hoc RCT analysis; level of evidence 3 assigned conservatively given the post-hoc nature of the trial data.","value_numeric":null}],"summaries":{"what_studied":"This multicohort international study investigated the role of sputum azurocidin-1 (AZU1) — a neutrophil pseudoenzyme — as a marker of bronchiectasis severity, its behavior during exacerbations (bacterial and viral), its effect on airway epithelium in vitro, and whether DPP1 inhibition with brensocatib reduces AZU1 (post-hoc analysis of the phase 2 WILLOW trial, up to 24 weeks).","key_result":"Higher sputum AZU1 correlated with worse bronchiectasis disease severity index (p<0.0001), lower FEV1% predicted (r=−0.47, p<0.001), more frequent exacerbations (p=0.0019), and highest AZU1 levels in airways infected with *Pseudomonas aeruginosa* (p<0.0001). In vitro, AZU1 impaired ciliary function and epithelial integrity. Brensocatib (10 mg and 25 mg) was the most potent reducer of AZU1 among all proteins measured over 24 weeks (p<0.0001).","limitations":"- WILLOW trial AZU1 analysis was post-hoc and not pre-specified, limiting causal inference.\n- Rhinovirus challenge cohort was very small (n=9 COPD patients), constraining generalizability of viral exacerbation findings.\n- In vitro epithelial data cannot directly confirm the same mechanism operates in human airways in vivo.","practice_takeaway":"AZU1 may serve as a clinically useful sputum biomarker to identify bronchiectasis patients with greater severity and infection burden, particularly *Pseudomonas* infection. DPP1 inhibition with brensocatib substantially reduces AZU1, supporting its potential as a therapeutic target in neutrophilic airway disease."}},{"doi":"10.1016/j.mucimm.2026.03.012","pmid":"41921915","title":"Single-cell profiling of BAL in preschool cystic fibrosis reveals macrophage dysregulation and ivacaftor-modified inflammatory programs in the early life lung","journal_title":"Mucosal Immunology","journal_issn":"1933-0219","published_at":"2026-03-31T00:00:00.000Z","url":"https://doi.org/10.1016/j.mucimm.2026.03.012","source_score":30,"is_retracted":false,"is_open_access":false,"raw_metadata":{"pubmed":true,"crossref":true,"unpaywall":true},"created_at":"2026-06-15T13:21:55.512Z","updated_at":"2026-06-15T13:21:55.512Z","tags":[{"paper_doi":"10.1016/j.mucimm.2026.03.012","tag_type":"age_group","value":"child","value_numeric":null},{"paper_doi":"10.1016/j.mucimm.2026.03.012","tag_type":"age_group","value":"pediatric","value_numeric":null},{"paper_doi":"10.1016/j.mucimm.2026.03.012","tag_type":"brand","value":"Kalydeco","value_numeric":null},{"paper_doi":"10.1016/j.mucimm.2026.03.012","tag_type":"brand","value":"Orkambi","value_numeric":null},{"paper_doi":"10.1016/j.mucimm.2026.03.012","tag_type":"category","value":"Immunology","value_numeric":null},{"paper_doi":"10.1016/j.mucimm.2026.03.012","tag_type":"condition","value":"Bronchiectasis","value_numeric":null},{"paper_doi":"10.1016/j.mucimm.2026.03.012","tag_type":"condition","value":"Cystic Fibrosis","value_numeric":null},{"paper_doi":"10.1016/j.mucimm.2026.03.012","tag_type":"generic","value":"ivacaftor","value_numeric":null},{"paper_doi":"10.1016/j.mucimm.2026.03.012","tag_type":"generic","value":"lumacaftor/ivacaftor","value_numeric":null},{"paper_doi":"10.1016/j.mucimm.2026.03.012","tag_type":"impact_flag","value":"practice_changing","value_numeric":null},{"paper_doi":"10.1016/j.mucimm.2026.03.012","tag_type":"level_of_evidence","value":"4","value_numeric":null},{"paper_doi":"10.1016/j.mucimm.2026.03.012","tag_type":"modality","value":"CFTR Modulator","value_numeric":null},{"paper_doi":"10.1016/j.mucimm.2026.03.012","tag_type":"sample_size","value":"n=45","value_numeric":45},{"paper_doi":"10.1016/j.mucimm.2026.03.012","tag_type":"sex","value":"mixed","value_numeric":null},{"paper_doi":"10.1016/j.mucimm.2026.03.012","tag_type":"study_design","value":"Cross-Sectional Single-Cell Multi-Omic Study","value_numeric":null},{"paper_doi":"10.1016/j.mucimm.2026.03.012","tag_type":"warning","value":"Sample size is 45 BAL samples from preschool children — conclusions, especially the null finding for lumacaftor/ivacaftor, may be underpowered; interpret with caution.","value_numeric":null},{"paper_doi":"10.1016/j.mucimm.2026.03.012","tag_type":"warning","value":"Sex breakdown across the cohort was not explicitly reported in the abstract; the 'mixed' sex tag is assumed based on typical CF trial enrollment.","value_numeric":null},{"paper_doi":"10.1016/j.mucimm.2026.03.012","tag_type":"warning","value":"The 'practice_changing' impact flag reflects the novelty of the multi-omic atlas and the ivacaftor immune finding, but clinical translation of single-cell transcriptomic data requires further validation.","value_numeric":null}],"summaries":{"what_studied":"Single-cell RNA sequencing (scRNA-seq) combined with multiplexed surface protein profiling of bronchoalveolar lavage (BAL) from preschool children with cystic fibrosis (CF) to characterize immune and epithelial cell populations and assess the effect of CFTR modulators (ivacaftor; lumacaftor/ivacaftor) on airway inflammation.","key_result":"A lower airway atlas of >190,000 cells from 45 BAL samples (43 cell populations) revealed widespread macrophage gene expression dysregulation in preschool CF lungs — including TNF/IFN signalling, cholesterol homeostasis, and pulmonary fibrosis pathways — that were worsened by early bronchiectasis. Ivacaftor (but not lumacaftor/ivacaftor) partially restored macrophage functional programs and reduced neutrophil infiltration-associated pathway expression.","limitations":"Small sample size limits generalizability; lumacaftor/ivacaftor findings may reflect limited power rather than true absence of effect; cross-sectional design precludes causal inference about bronchiectasis and inflammatory changes.","practice_takeaway":"Even with CFTR modulator therapy, macrophage-driven inflammation persists in preschool CF lungs, supporting the case for adjunctive anti-inflammatory strategies. Ivacaftor appears more effective than lumacaftor/ivacaftor at modifying the CF airway immune environment, though this needs confirmation in larger studies."}}],"initialOffset":21,"fetchConfig":{"kind":"tags","tags":[{"tag_type":"condition","value":"Bronchiectasis"}]},"breadcrumbItems":[{"name":"Home","href":"/"},{"name":"Research Hub","href":"/hub"},{"name":"Conditions","href":"/hub/tag/condition"},{"name":"Bronchiectasis"}],"hrefConfig":{"basePath":"/hub/papers","webViewQueryParams":{"hub_ctx":"tag","hub_tag_type":"condition","hub_tag_value":"Bronchiectasis"}}}]]

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