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BMJ Open Respiratory Research Nov 2023Timely diagnosis of interstitial lung disease (ILD) is limited by obstacles in the current patient pathway. Misdiagnosis and delays are common and may lead to a...
INTRODUCTION
Timely diagnosis of interstitial lung disease (ILD) is limited by obstacles in the current patient pathway. Misdiagnosis and delays are common and may lead to a significant burden of diagnostic procedures and worse outcomes. This Delphi survey aimed to identify consensus on the key steps that facilitate the patient journey to an accurate ILD diagnosis and appropriate management in the US.
METHODS
A modified Delphi analysis was conducted, comprising three online surveys based on a comprehensive literature search. The surveys spanned five domains (guidelines, community screening, diagnosis, management and specialist referral) and were completed by a panel of US physicians, including primary care physicians and pulmonologists practising in community or academic settings. A priori definitions of consensus agreement were median scores of 2-3 (agree strongly/agree), with an IQR of 0-1 for questions on a 7-point Likert scale from -3 to 3, or ≥80% agreement for binary questions.
RESULTS
Forty-nine panellists completed the surveys and 62 statements reached consensus agreement. There was consensus agreement on what should be included in the primary care evaluation of patients with suspected ILD and the next steps following workup. Regarding diagnosis in community pulmonology care, consensus agreement was reached on the requisition and reporting of high-resolution CT scans and the appropriate circumstances for holding multidisciplinary discussions. Additionally, there was consensus agreement on which symptoms and comorbidities should be monitored, the frequency of consultations and the assessment of disease progression. Regarding specialist referral, consensus agreement was reached on which patients should receive priority access to ILD centres and the contents of the referral package.
CONCLUSIONS
These findings clarify the most common issues that should merit further evaluation for ILD and help define the steps for timely, accurate diagnosis and appropriate collaborative specialty management of patients with ILD.
Topics: Humans; Lung Diseases, Interstitial; Comorbidity; Surveys and Questionnaires; Diagnostic Errors; Physicians
PubMed: 38007235
DOI: 10.1136/bmjresp-2022-001594 -
Identifying asthma-related risks during hospitalization using the child asthma risk assessment tool.The Journal of Asthma : Official... Dec 2023The Child Asthma Risk Assessment Tool (CARAT) identifies risk factors for asthma morbidity. We hypothesized that CARAT-identified risk factors (using a CARAT adapted... (Review)
Review
The Child Asthma Risk Assessment Tool (CARAT) identifies risk factors for asthma morbidity. We hypothesized that CARAT-identified risk factors (using a CARAT adapted for inpatient use) would be associated with future healthcare utilization and would identify areas for intervention. We reviewed CARAT data collected during pediatric asthma admissions from 2010-2015, assessing for risk factors in environmental, medical, and social domains and providing prompts for inpatient (specialist consultation or social services engagement) and post-discharge interventions (home care visit or home environmental assessment). Confirmatory factor analysis identified groups of CARAT-identified risk factors with similar effects on healthcare utilization (latent factors). Structural equation models then evaluated relationships between latent factors and future utilization. There were 2731 unique patients admitted for asthma exacerbations; 1015 (37%) had complete CARAT assessments and were included in analyses. Those with incomplete CARAT assessments were more often younger and privately-insured. CARAT-identified risk factors across domains were common in children hospitalized for exacerbations. Risks in the environmental domain were most common. Inpatient asthma consults by pulmonologists or allergists and home care referrals were the most frequent interventions indicated (62%, 628/1015, and 50%, 510/1015, respectively). Two latent factors were positively associated with healthcare utilization in the year after index stay - social stressors and known/suspected allergies (both < 0.05). Stratified analyses analyzing data just from those children with prior healthcare utilization also indicated known/suspected allergies to be positively associated with future utilization. Inpatient interventions to address social stressors and allergic profiles may be warranted to reduce subsequent asthma morbidity.
Topics: Humans; Child; Asthma; Aftercare; Patient Discharge; Hospitalization; Risk Assessment; Hypersensitivity
PubMed: 37345884
DOI: 10.1080/02770903.2023.2228897 -
Current Hematologic Malignancy Reports Dec 2023Telomere biology disorders (TBD) are a group of genetic disorders characterized by premature shortening of telomeres, resulting in accelerated aging of somatic cells.... (Review)
Review
PURPOSE OF REVIEW
Telomere biology disorders (TBD) are a group of genetic disorders characterized by premature shortening of telomeres, resulting in accelerated aging of somatic cells. This often leads to major multisystem organ dysfunction, and TBDs have become increasingly recognized as a significant contributor to numerous disease processes within the past 10-15 years. Both research and clinical practice in this field are rapidly evolving.
RECENT FINDINGS
A subset of patients with TBD suffers from interstitial lung disease, most commonly pulmonary fibrosis. Often, the clinical presentation is indistinguishable from other forms of lung fibrosis. There are no pathognomonic radiographic or histological features, and a high level of suspicion is therefore required. Telomere evaluation is thus crucial to establishing the diagnosis. This review details the clinical presentation, objective evaluation, indicated genetic testing, and recommended management strategies for patients affected by interstitial lung disease associated with TBDs. Our goal is to empower pulmonologists and other healthcare professionals who care for these patients to provide appropriate and personalized care for this population.
PubMed: 38159192
DOI: 10.1007/s11899-023-00720-9 -
Journal of the American Society of... 2023Pulmonologists can biopsy structures below the diaphragm using the convex curvilinear ultrasound bronchoscope via the esophagus (EUS-B). The literature with respect to... (Review)
Review
INTRODUCTION
Pulmonologists can biopsy structures below the diaphragm using the convex curvilinear ultrasound bronchoscope via the esophagus (EUS-B). The literature with respect to the value of EUS-B, rapid on-site evaluation, and final diagnostic yield for structures below the diaphragm is limited. We review our institutional experience.
MATERIALS AND METHODS
Our database was queried retrospectively for EUS-B fine needle aspirations (FNAs) from 2013 to 2021. All procedures involving EUS-B-FNA of subdiaphragmatic structures were selected for analysis. The following data elements were collected for each patient: age, gender, clinical indication, sample site, on-site adequacy (OSA), preliminary and final diagnoses, and sufficiency of cell block for ancillary studies.
RESULTS
A total of 75 subdiaphragmatic sites were biopsied in 74 patients. Of which, 87% of samples subjected to rapid on-site evaluation were deemed to contain adequate material (OSA+). There were no false-positive OSAs. Six cases remained nondiagnostic at the final diagnosis. The final diagnostic yield (with cell block) was 92% (69/75 cases). Cell block was sufficient for immunohistochemistry or special stains in all applicable cases (n = 36). Molecular testing was requested for 11 cases and successful in 10 (91%). Sampling of subdiaphragmatic sites changed the stage in 67% (38/57) of lung cancer patients.
CONCLUSIONS
Pulmonologists can perform EUS-B-FNA of subdiaphragmatic sites with high OSA and final diagnostic yield when assisted by cytopathologists. Strong correlations exist between OSA, cell block adequacy, and subsequent capacity to perform ancillary testing. EUS-B below the diaphragm can make an important contribution to the diagnosis of lung cancer, nonpulmonary malignancies, and other diseases.
Topics: Humans; Retrospective Studies; Pulmonologists; Endosonography; Endoscopic Ultrasound-Guided Fine Needle Aspiration; Lung Neoplasms
PubMed: 37336683
DOI: 10.1016/j.jasc.2023.05.004 -
Revue Des Maladies Respiratoires Jan 2024Ultrasonography is an emerging tool that helps to assess diaphragmatic function. It is now widely used in ICUs to predict weaning from mechanical ventilation.... (Review)
Review
INTRODUCTION
Ultrasonography is an emerging tool that helps to assess diaphragmatic function. It is now widely used in ICUs to predict weaning from mechanical ventilation. Ultrasonography is readily available, harmless (no radiation), and repeatable with good interoperator reproducibility. Over the past few years, ultrasonography has seen increasing use in patients with chronic pulmonary pathologies.
STATE OF THE ART
The aim of this review is (1) to describe the ultrasound techniques used to assess diaphragmatic excursion and thickening, (2) to indicate the expected, normal values in healthy patients, and (3) to summarize the main findings and clinical applications in treatment of chronic respiratory disorders.
CONCLUSIONS
Chronic pulmonary diseases are associated with diaphragmatic dysfunction that can be assessed with ultrasound. Diaphragmatic dysfunction is primary in neuromuscular disorders and secondary to respiratory disease in other chronic pulmonary conditions (COPD, ILD). Ultrasound is correlated with the severity of the underlying disease (functional and clinical parameters).
PERSPECTIVES
The prognostic interest of diaphragm ultrasonography remains to be established, after which its utilization should become routine.
Topics: Humans; Diaphragm; Pulmonologists; Reproducibility of Results; Lung; Ultrasonography
PubMed: 37980184
DOI: 10.1016/j.rmr.2023.10.005 -
Pediatric Pulmonology Aug 2023In people with cystic fibrosis (pwCF), the impact of cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies, such as...
BACKGROUND
In people with cystic fibrosis (pwCF), the impact of cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies, such as Elexacaftor-Tezacaftor-Ivacaftor (ETI), on structural changes in the lungs is unclear.
OBJECTIVE
To determine the impact of ETI on clinical parameters and on structural lung disease as measured by the changes in the chest computed tomography (CT) scans in pwCF.
METHODS
Percent predicted forced expiratory volume in one second (ppFEV1), body mass index (BMI), and microbiologic data were collected at initiation and 3-month intervals for 1 year. Chest CT scans before starting ETI therapy (baseline) and at 1-year on ETI therapy were compared by two pulmonologists independently.
RESULTS
The sample size was 67 pwCF, 30 (44.8%) males, median age of 25 (16, 33.5) years. Significant increases in ppFEV1 and BMI observed by 3 months of ETI therapy persisted throughout 1 year of ETI therapy (p < 0.001 at all-time points for both). After 1 year on ETI, pwCF had significant reductions in Pseudomonas aeruginosa (-42%) and MRSA (-42%) positivity. None of the pwCF had worsening of chest CT parameters during 1 year of ETI therapy. Comparing chest CT findings at baseline and at 1-year follow-up, bronchiectasis was present in 65 (97%) pwCF and at 1-year follow-up decreased in 7 (11%). Bronchial wall thickening 64 (97%), decreased in 53 (79%). Mucous plugging in 63 (96%), absent in 11 (17%), and decreased in 50 (77%). Hyperinflation/air trapping in 44 (67%), decreased in 11 (18%), absent in 27 (44%) CONCLUSIONS: ETI significantly improved clinical outcomes and lung disease as documented by improvement in chest CT scans.
Topics: Male; Humans; Female; Cystic Fibrosis; Pyrazoles; Cystic Fibrosis Transmembrane Conductance Regulator; Aminophenols; Mutation; Benzodioxoles
PubMed: 37222417
DOI: 10.1002/ppul.26485 -
Respiratory Research Sep 2023Idiopathic pulmonary fibrosis (IPF) has an unknown aetiology and limited treatment options. A recent meta-analysis identified three novel causal variants in the TERT,... (Observational Study)
Observational Study
BACKGROUND
Idiopathic pulmonary fibrosis (IPF) has an unknown aetiology and limited treatment options. A recent meta-analysis identified three novel causal variants in the TERT, SPDL1, and KIF15 genes. This observational study aimed to investigate whether the aforementioned variants cause clinical phenotypes in a well-characterised IPF cohort.
METHODS
The study consisted of 138 patients with IPF who were diagnosed and treated at the Helsinki University Hospital and genotyped in the FinnGen FinnIPF study. Data on > 25 clinical parameters were collected by two pulmonologists who were blinded to the genetic data for patients with TERT loss of function and missense variants, SPDL1 and KIF15 missense variants, and a MUC5B variant commonly present in patients with IPF, or no variants were separately analysed.
RESULTS
The KIF15 missense variant is associated with the early onset of the disease, leading to progression to early-age transplantation or death. In patients with the KIF15 variant, the median age at diagnosis was 54.0 years (36.5-69.5 years) compared with 72.0 years (65.8-75.3 years) in the other patients (P = 0.023). The proportion of KIF15 variant carriers was 9- or 3.6-fold higher in patients aged < 55 or 65 years, respectively. The variants for TERT and MUC5B had similar effects on the patient's clinical course, as previously described. No distinct phenotypes were observed in patients with the SPDL1 variant.
CONCLUSIONS
Our study indicated the potential of KIF15 to be used in the genetic diagnostics of IPF. Further studies are needed to elucidate the biological mechanisms of KIF15 in IPF.
Topics: Humans; Middle Aged; Idiopathic Pulmonary Fibrosis; Genotype; Phenotype; Mucin-5B; Kinesins
PubMed: 37777755
DOI: 10.1186/s12931-023-02540-0 -
Journal of Cardiothoracic Surgery Jul 2023COVID-19 Patients may be at risk for involving with spontaneous pneumothorax. However, clinical data are lacking in this regard. In this study, we aimed to investigate...
INTRODUCTION
COVID-19 Patients may be at risk for involving with spontaneous pneumothorax. However, clinical data are lacking in this regard. In this study, we aimed to investigate the demographic, clinical, and radiological characteristics and survival predictors in COVID-19 patients with pneumothorax.
METHODS
This is a retrospectivestudy conducted on COVID-19 patients with pneumothorax that had been hospitalized at hospital. l from December 2021 to March 2022. The chest computed tomography (CT) scan of all patients was reviewed by an experienced pulmonologist in search of pulmonary pneumothorax. Survival analysis was conducted to identify the predictors of survival in patients with COVID-19 and pneumothorax.
RESULTS
A total of 67 patients with COVID-19 and pneumothorax were identified. Of these, 40.7% were located in the left lung, 40.7% were in the right lung, and 18.6% were found bilaterally. The most common symptoms in the patient with pneumothorax were dyspnea (65.7%), increased cough severity (53.7%), chest pain (25.4%), and hemoptysis (16.4%). The frequency of pulmonary left and right bullae, pleural effusion, andfungus ball were 22.4%, 22.4%, 22.4%, and 7.5%, respectively. Pneumothorax was managed with chest drain (80.6%), chest drain and surgery (6%), and conservatively (13.4%). The 50-day mortality rate was 52.2% (35 patients). The average survival time for deceased patients was 10.06 (2.17) days.
CONCLUSIONS
Our results demonstrated that those with pleural effusion or pulmonary bullae have a lower survival rate. Further studies are required to investigate the incidence and causality relation between COVID-19 and pneumothorax.
Topics: Humans; Pneumothorax; Blister; COVID-19; Pleural Effusion; Survival Analysis
PubMed: 37403072
DOI: 10.1186/s13019-023-02331-0 -
Journal of Bronchology & Interventional... Oct 2023
Topics: Humans; Pulmonologists; Bronchoscopy
PubMed: 37784236
DOI: 10.1097/LBR.0000000000000939 -
Seminars in Respiratory and Critical... Jun 2024Antisynthetase syndrome (ASyS) is now a widely recognized entity within the spectrum of idiopathic inflammatory myopathies. Initially described in patients with a triad... (Review)
Review
Antisynthetase syndrome (ASyS) is now a widely recognized entity within the spectrum of idiopathic inflammatory myopathies. Initially described in patients with a triad of myositis, arthritis, and interstitial lung disease (ILD), its presentation can be diverse. Additional common symptoms experienced by patients with ASyS include Raynaud's phenomenon, mechanic's hand, and fever. Although there is a significant overlap with polymyositis and dermatomyositis, the key distinction lies in the presence of antisynthetase antibodies (ASAs). Up to 10 ASAs have been identified to correlate with a presentation of ASyS, each having manifestations that may slightly differ from others. Despite the proposal of three classification criteria to aid diagnosis, the heterogeneous nature of patient presentations poses challenges. ILD confers a significant burden in patients with ASyS, sometimes manifesting in isolation. Notably, ILD is also often the initial presentation of ASyS, requiring pulmonologists to remain vigilant for an accurate diagnosis. This article will comprehensively review the various aspects of ASyS, including disease presentation, diagnosis, management, and clinical course, with a primary focus on its pulmonary manifestations.
Topics: Humans; Myositis; Lung Diseases, Interstitial; Autoantibodies; Diagnosis, Differential
PubMed: 38710221
DOI: 10.1055/s-0044-1785536