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Journal of the National Comprehensive... Nov 2023Immune checkpoint inhibitors (ICIs) are a first-line and perioperative treatment for lung cancer. Pneumonitis is a potentially life-threatening complication of ICI...
BACKGROUND
Immune checkpoint inhibitors (ICIs) are a first-line and perioperative treatment for lung cancer. Pneumonitis is a potentially life-threatening complication of ICI treatment in 2% to 5% of patients; however, risk factors for developing ICI pneumonitis (ICI-p) remain undefined.
METHODS
We conducted a retrospective cohort study of consecutive patients with lung cancer who received at least one dose of ICI from 2015 through 2020 at The Ohio State University. Pneumonitis cases were documented by the treating oncologist and retrospectively evaluated for agreement between an oncologist and a pulmonologist. Patient demographic and clinical characteristics were recorded and summarized between those with and without pneumonitis for the overall cohort. Univariate and multivariable survival analyses using the Fine-Gray competing risk model were used to examine the associations.
RESULTS
A total of 471 patients with lung cancer were included, of which 402 had non-small cell lung cancer and 69 had small cell lung cancer; 39 (8%) patients in the overall cohort developed ICI-p. Preexisting interstitial abnormalities and prior chest radiation were both significantly associated with ICI-p on univariate analysis (hazard ratio [HR], 8.91; 95% CI, 4.69-16.92; P<.001; and HR, 2.81; 95% CI, 1.50-5.28; P=.001). On multivariable analyses, interstitial abnormalities remained a strong independent risk factor for ICI-p when controlling for chest radiation and type of immunotherapy (HR, 9.77; 95% CI, 5.17-18.46; P<.001). Among patients with ICI-p (n=39), those with severe (grade 3-5) pneumonitis had worse overall survival compared with those with mild (grade 1 or 2) pneumonitis (P=.001). Abnormal pulmonary function test results at both 12 and 18 months prior to ICI initiation were not significantly associated with ICI-p.
CONCLUSIONS
Preexisting interstitial abnormalities on chest CT and prior chest radiation are independent risk factors that are strongly associated with ICI-p in patients with lung cancer. These findings highlight a potential need for closer observation for ICI-p among patients with these risk factors.
Topics: Humans; Lung Neoplasms; Carcinoma, Non-Small-Cell Lung; Immune Checkpoint Inhibitors; Retrospective Studies; Pneumonia
PubMed: 37935100
DOI: 10.6004/jnccn.2023.7059 -
Internal Medicine Journal Feb 2024The first dedicated tracheobronchial silicone stent was designed by the French pulmonologist Jean-Paul Dumon. The most common indications for stenting are to minimise... (Review)
Review
The first dedicated tracheobronchial silicone stent was designed by the French pulmonologist Jean-Paul Dumon. The most common indications for stenting are to minimise extrinsic airway compression from mass effect, maintain airway patency due to intrinsic obstruction or treat significant nonmalignant airway narrowing or fistulae. Silicone stents require rigid bronchoscopy for insertion; however, they are more readily repositioned and removed compared with metallic stents. Metallic stents demonstrate luminal narrowing when loads are applied to their ends, therefore stents should either be reinforced at the ends or exceed the area of stenosis by a minimum of 5 mm. Nitinol, a nickel-titanium metal alloy, is currently the preferred material used for airway stents. Airway stenting provides effective palliation for patients with severe symptomatic obstruction. Drug-eluting and three-dimensional printing of airway stents present promising solutions to the challenges of the physical and anatomical constraints of the tracheobronchial tree. Biodegradable stents could also be a solution for the treatment of nonmalignant airway obstruction.
Topics: Humans; Bronchoscopy; Airway Obstruction; Silicones; Metals; Stents; Treatment Outcome; Nickel; Titanium
PubMed: 38140778
DOI: 10.1111/imj.16304 -
AJR. American Journal of Roentgenology Apr 2024Progressive pulmonary fibrosis (PPF) and interstitial lung abnormalities (ILA) are relatively new concepts in interstitial lung disease (ILD) imaging and clinical... (Review)
Review
Progressive pulmonary fibrosis (PPF) and interstitial lung abnormalities (ILA) are relatively new concepts in interstitial lung disease (ILD) imaging and clinical management. Recognition of signs of PPF, as well as identification and classification of ILA, are important tasks during chest high-resolution CT interpretation, to optimize management of patients with ILD and those at risk of developing ILD. However, following professional society guidance, the role of imaging surveillance remains unclear in stable patients with ILD, asymptomatic patients with ILA who are at risk of progression, and asymptomatic patients at risk of developing ILD without imaging abnormalities. In this AJR Expert Panel Narrative Review, we summarize the current knowledge regarding PPF and ILA and describe the range of clinical practice with respect to imaging patients with ILD, those with ILA, and those at risk of developing ILD. In addition, we offer suggestions to help guide surveillance imaging in areas with an absence of published guidelines, where such decisions are currently driven primarily by local pulmonologists' preference.
PubMed: 38656115
DOI: 10.2214/AJR.24.31125 -
The Journal of Asthma : Official... Apr 2024Mounier-Kuhn syndrome or tracheobronchomegaly, is a rare condition that consists of abnormal dilation of the trachea and main bronchi due to a pathological arrangement...
INTRODUCTION
Mounier-Kuhn syndrome or tracheobronchomegaly, is a rare condition that consists of abnormal dilation of the trachea and main bronchi due to a pathological arrangement of smooth muscle fibers in this area.
CASE REPORT
We present the case of a 46-year-old woman with poorly controlled asthma and recurrent infections, who was diagnosed with Mounier-Kuhn syndrome through a computed tomography scan revealing an unusual enlargement of the trachea with associated bronchiectasis.
RESULTS
The diagnosis of Mounier-Kuhn syndrome is radiological, involving measurement of the trachea where a diameter >25 mm in men and >21 mm in women is observed. While diagnosis is sometimes incidental, there is an association with respiratory diseases such as asthma or COPD, hence clinical suspicion is important in patients with poorly controlled underlying conditions who present with recurrent infections, inadequate secretion management, or even hemoptysis.
CONCLUSIONS
Despite its rarity, this syndrome significantly impacts patients' quality of life. Diagnosis and management involve comprehensive evaluations including computed tomography, with a multidisciplinary approach including pulmonologists and radiologists. Exploring its clinical features, associations with other respiratory diseases and treatment options is crucial in managing this rare respiratory condition.
PubMed: 38639468
DOI: 10.1080/02770903.2024.2344168 -
Journal of Pediatric Orthopedics Aug 2023Spinal conditions, such as scoliosis and spinal tumors, are prevalent in neurofibromatosis type 1 (NF1). Despite the recognized importance of their early detection and...
Consensus-Based Best Practice Guidelines for the Management of Spinal Deformity and Associated Tumors in Pediatric Neurofibromatosis Type 1: Screening and Surveillance, Surgical Intervention, and Medical Therapy.
BACKGROUND
Spinal conditions, such as scoliosis and spinal tumors, are prevalent in neurofibromatosis type 1 (NF1). Despite the recognized importance of their early detection and treatment, there remain knowledge gaps in how to approach these manifestations. The purpose of this study was to utilize the experience of a multidisciplinary committee of experts to establish consensus-based best practice guidelines (BPGs) for spinal screening and surveillance, surgical intervention, and medical therapy in pediatric patients with NF1.
METHODS
Using the results of a prior systematic review, 10 key questions that required further assessment were first identified. A committee of 20 experts across medical specialties was then chosen based on their clinical experience with spinal deformity and tumors in NF1. These were 9 orthopaedic surgeons, 4 neuro-oncologists/oncologists, 3 neurosurgeons, 2 neurologists, 1 pulmonologist, and 1 clinical geneticist. An initial online survey on current practices and opinions was conducted, followed by 2 additional surveys via a formal consensus-based modified Delphi method. The final survey involved voting on agreement or disagreement with 35 recommendations. Items reaching consensus (≥70% agreement or disagreement) were included in the final BPGs.
RESULTS
Consensus was reached for 30 total recommendations on the management of spinal deformity and tumors in NF1. These were 11 recommendations on screening and surveillance, 16 on surgical intervention, and 3 on medical therapy. Five recommendations did not achieve consensus and were excluded from the BPGs.
CONCLUSION
We present a set of consensus-based BPGs comprised of 30 recommendations for spinal screening and surveillance, surgical intervention, and medical therapy in pediatric NF1.
Topics: Child; Humans; Neurofibromatosis 1; Consensus; Scoliosis; Spine; Delphi Technique
PubMed: 37253707
DOI: 10.1097/BPO.0000000000002431 -
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 -
Diagnostics (Basel, Switzerland) Jul 2023Interstitial lung diseases (ILDs) are a group of heterogeneous diseases characterized by inflammation and/or fibrosis of the lung interstitium, leading to a wide range... (Review)
Review
BACKGROUND
Interstitial lung diseases (ILDs) are a group of heterogeneous diseases characterized by inflammation and/or fibrosis of the lung interstitium, leading to a wide range of clinical manifestations and outcomes. Over the years, the literature has demonstrated the increased diagnostic accuracy and confidence associated with a multidisciplinary approach (MDA) in assessing diseases involving lung parenchyma. This approach was recently emphasized by the latest guidelines from the American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Association for the diagnosis of ILDs.
METHODS
In this review, we will discuss the role, composition, and timing of multidisciplinary diagnosis (MDD) concerning idiopathic pulmonary fibrosis, connective tissue disease associated with ILDs, hypersensitive pneumonia, and idiopathic pneumonia with autoimmune features, based on the latest recommendations for their diagnosis.
RESULTS
The integration of clinical, radiological, histopathological, and, often, serological data is crucial in the early identification and management of ILDs, improving patient outcomes. Based on the recent endorsement of transbronchial cryo-biopsy in idiopathic pulmonary fibrosis guidelines, an MDA helps guide the choice of the sampling technique, obtaining the maximum diagnostic performance, and avoiding the execution of more invasive procedures such as a surgical lung biopsy. A multidisciplinary team should include pulmonologists, radiologists, pathologists, and, often, rheumatologists, being assembled regularly to achieve a consensus diagnosis and to review cases in light of new features.
CONCLUSIONS
The literature highlighted that an MDA is essential to improve the accuracy and reliability of ILD diagnosis, allowing for the early optimization of therapy and reducing the need for invasive procedures. The multidisciplinary diagnosis of ILDs is an ongoing and dynamic process, often referred to as a "working diagnosis", involving the progressive integration and re-evaluation of clinical, radiological, and histological features.
PubMed: 37510180
DOI: 10.3390/diagnostics13142437 -
BMJ Supportive & Palliative Care Sep 2023Occupational exposure to silica has been seldom implicated to cause systemic sclerosis. Erasmus syndrome is a rare condition where there is development of systemic...
Occupational exposure to silica has been seldom implicated to cause systemic sclerosis. Erasmus syndrome is a rare condition where there is development of systemic sclerosis following silicosis. Early diagnosis is essential for appropriate treatment and symptom control. We describe a 42-year-old stone cutter with silicosis who was subsequently diagnosed to have diffuse pattern of systemic sclerosis. A multidisciplinary team including pulmonologists, specialist palliative care physicians, rheumatologists and radiologists were involved in diagnosis and management. Timely diagnosis and a multidisciplinary team management with pharmacological and non-pharmacological measures was essential in holistic care provision. This is the first report of specialist palliative care team facilitating diagnosis of this rare syndrome and promoting integration in the overall care and management.
PubMed: 37758299
DOI: 10.1136/spcare-2023-004588 -
Pediatric Pulmonology May 2024Recent evidence suggests that alexithymic deficits in emotional processing may also affect physical health, and alexithymia may also be associated with organic...
OBJECTIVE
Recent evidence suggests that alexithymic deficits in emotional processing may also affect physical health, and alexithymia may also be associated with organic disorders. The emotional well-being of patients with primary ciliary dyskinesia (PCD) is often negatively affected by uncertainty about the prognosis, lack of ongoing medical care, and lack of symptom control. This study aims to evaluate the frequency of alexithymia and its possible impact on the management of childhood PCD.
MATERIALS AND METHODS
Subjects were recruited from patients with PCD and healthy volunteers aged 8-18 years. The questionnaire included sociodemographic characteristics and self-report scales. Data were compared between patient and control groups.
RESULTS
In the >14 years of age group, the total Toronto Alexithymia Scale (TAS-20) score was significantly higher in the patients (56.60 ± 13.01) compared to the control group (46.47 ± 7.50) (p = .007). There were 6 (30) patients with a TAS-20 score ≥61. There was a significant correlation between TAS-20 and Pediatric Quality of Life (PedsQL) score (child), but no correlation between TAS-20 and Kovacs Children Depression Inventory (CDI) and PedsQL score (parents). The TAS-20 score was significantly higher in patients with bronchiectasis (p = .035), nasal polyps (p = .045), and siblings with PCD (p = .001). Furthermore, the TAS-20 score had a significant negative correlation with pulmonary function tests.
CONCLUSION
Although this study is based on limited data from a single center and cannot be generalized to all PCD patient communities, our results show that PCD patients are more likely to have alexithymia compared to healthy controls and highlight the need to evaluate for alexithymia in patients with PCD. It is important for pediatric pulmonologists to have a thorough knowledge of the alexithymic features associated with PCD and to refer patients to pediatric psychiatry when necessary, especially in patients who are noncompliant with treatment protocols.
PubMed: 38695576
DOI: 10.1002/ppul.27038