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PURPOSE: This study aimed to evaluate the hypothesis that active smoking impacts upon mediators and abundance of circulating fibrocyte cells in smoking-related disease characterised by fibrosis. METHODS: Flow cytometry and enzyme-linked immunosorbent assays were used to investigate blood from five patient groups: healthy never-smokers, healthy current smokers, stable chronic obstructive pulmonary disease (COPD) active smokers, idiopathic pulmonary fibrosis (IPF) never-smokers, and IPF active smokers. RESULTS: A significant inverse dose-response relationship was observed in healthy smokers among cumulative smoking burden (pack-years) and fibrocyte abundance (p = 0.006, r = -0.86). Among serum profibrotic fibrocyte chemokines measured, CCL18 rose significantly alongside fibrocyte numbers in all five subject groups, while having an inverse dose-response relationship with pack-year burden in healthy smokers (p = 0.003, r = -0.89). In IPF, CCL2 rose in direct proportion to fibrocyte abundance irrespective of smoking status but had lower serum levels in those currently smoking (p = < 0.001). For the study population, CXCL12 was decreased in pooled current smokers versus never-smokers (p = 0.03). CONCLUSION: The suppressive effect of current, as distinct from former, chronic smoking on circulating fibrocyte abundance in healthy smokers, and modulation of regulatory chemokine levels by active smoking may have implications for future studies of fibrocytes in smoking-related lung diseases as a potential confounding variable.
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Quimiocina CCL2 , Quimiocina CXCL12 , Quimiocinas CC , Fibrosis Pulmonar Idiopática , Enfermedad Pulmonar Obstructiva Crónica , Humanos , Fibrosis Pulmonar Idiopática/sangre , Fibrosis Pulmonar Idiopática/patología , Masculino , Enfermedad Pulmonar Obstructiva Crónica/sangre , Enfermedad Pulmonar Obstructiva Crónica/patología , Persona de Mediana Edad , Quimiocina CXCL12/sangre , Femenino , Quimiocina CCL2/sangre , Anciano , Quimiocinas CC/sangre , Estudios de Casos y Controles , Adulto , Fumar Cigarrillos/efectos adversos , Fumar Cigarrillos/sangre , Fumadores , No Fumadores/estadística & datos numéricos , Fumar/efectos adversos , Fumar/sangre , Ensayo de Inmunoadsorción Enzimática , Citometría de FlujoRESUMEN
AIMS: Patients with idiopathic pulmonary fibrosis (IPF) receiving antifibrotic medication and patients with non-IPF fibrosing lung disease often demonstrate rates of annualised forced vital capacity (FVC) decline within the range of measurement variation (5.0%-9.9%). We examined whether change in visual CT variables could help confirm whether marginal FVC declines represented genuine clinical deterioration rather than measurement noise. METHODS: In two IPF cohorts (cohort 1: n=103, cohort 2: n=108), separate pairs of radiologists scored paired volumetric CTs (acquired between 6 and 24 months from baseline). Change in interstitial lung disease, honeycombing, reticulation, ground-glass opacity extents and traction bronchiectasis severity was evaluated using a 5-point scale, with mortality prediction analysed using univariable and multivariable Cox regression analyses. Both IPF populations were then combined to determine whether change in CT variables could predict mortality in patients with marginal FVC declines. RESULTS: On univariate analysis, change in all CT variables except ground-glass opacity predicted mortality in both cohorts. On multivariate analysis adjusted for patient age, gender, antifibrotic use and baseline disease severity (diffusing capacity for carbon monoxide), change in traction bronchiectasis severity predicted mortality independent of FVC decline. Change in traction bronchiectasis severity demonstrated good interobserver agreement among both scorer pairs. Across all study patients with marginal FVC declines, change in traction bronchiectasis severity independently predicted mortality and identified more patients with deterioration than change in honeycombing extent. CONCLUSIONS: Change in traction bronchiectasis severity is a measure of disease progression that could be used to help resolve the clinical importance of marginal FVC declines.
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Fibrosis Pulmonar Idiopática/diagnóstico por imagen , Fibrosis Pulmonar Idiopática/fisiopatología , Capacidad Vital/fisiología , Anciano , Estudios de Cohortes , Progresión de la Enfermedad , Femenino , Humanos , Fibrosis Pulmonar Idiopática/terapia , Masculino , Persona de Mediana Edad , Índice de Severidad de la Enfermedad , Factores de Tiempo , Tomografía Computarizada por Rayos XRESUMEN
BACKGROUND: Common variable immunodeficiency (CVID) is a group of heterogeneous primary immunodeficiencies characterised by a dysregulated and impaired immune response. In addition to an increased susceptibility to infection, it is also associated with noninfectious autoimmune and lymphoproliferative complications. CVID is rarely associated with neurological complications. Pulmonary involvement is more common, and patients can develop an interstitial lung disease known as granulomatous-lymphocytic interstitial lung disease (GLILD). CASE PRESENTATION: A 50-year-old Caucasian female with a history of Evans syndrome (idiopathic thrombocytopaenic purpura and autoimmune haemolytic anaemia) and hypogammaglobulinaemia initially presented to the neurology clinic with marked cerebellar ataxia and headaches. Following extensive investigation (which included brain biopsy), she was diagnosed with neuro-sarcoidosis and her symptoms resolved following treatment with immunosuppressive therapy. Over the following 10 years, she was extensively investigated for recurrent pulmonary infections and abnormal radiological findings, which included pulmonary nodules, infiltrates and splenomegaly. Subsequently, she was referred to an immunology clinic, where immunoglobulin replacement treatment was started for what was ultimately considered to be CVID. Shortly afterwards, evaluation of her clinical, radiological and histological findings at a specialist interstitial lung disease clinic led to a diagnosis of GLILD. CONCLUSION: CVID is a condition which should be suspected in patients with immunodeficiency and recurrent infections. Concomitant autoimmune disorders such as haemolytic anaemia and immune thrombocytopenia may further support the diagnosis. As illustrated in this case, there is a rare association between CVID and inflammatory involvement of the neurological system. Respiratory physicians should also suspect CVID with associated GLILD in patients with apparent pulmonary granulomatous disease and recurrent infections. In addition, this case also highlights the challenge of diagnosing CVID and its associated features, and how the definitive exclusion of other pathologies such as malignancy, mycobacterial infection and lymphoma is required as part of this diagnostic process.
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Enfermedades del Sistema Nervioso Central/etiología , Inmunodeficiencia Variable Común/complicaciones , Granuloma/etiología , Enfermedades Pulmonares Intersticiales/etiología , Sarcoidosis/etiología , Biopsia , Encéfalo/diagnóstico por imagen , Enfermedades del Sistema Nervioso Central/diagnóstico , Femenino , Granuloma/diagnóstico , Humanos , Pulmón/diagnóstico por imagen , Enfermedades Pulmonares Intersticiales/diagnóstico , Imagen por Resonancia Magnética , Persona de Mediana Edad , Sarcoidosis/diagnóstico , Tomografía Computarizada por Rayos XRESUMEN
RATIONALE: Quantitative computed tomographic (CT) measures of baseline disease severity might identify patients with idiopathic pulmonary fibrosis (IPF) with an increased mortality risk. We evaluated whether quantitative CT variables could act as a cohort enrichment tool in future IPF drug trials. OBJECTIVES: To determine whether computer-derived CT measures, specifically measures of pulmonary vessel-related structures (VRSs), can better predict functional decline and survival in IPF and reduce requisite sample sizes in drug trial populations. METHODS: Patients with IPF undergoing volumetric noncontrast CT imaging at the Royal Brompton Hospital, London, and St. Antonius Hospital, Utrecht, were examined to identify pulmonary function measures (including FVC) and visual and computer-derived (CALIPER [Computer-Aided Lung Informatics for Pathology Evaluation and Rating] software) CT features predictive of mortality and FVC decline. The discovery cohort comprised 247 consecutive patients, with validation of results conducted in a separate cohort of 284 patients, all fulfilling drug trial entry criteria. MEASUREMENTS AND MAIN RESULTS: In the discovery and validation cohorts, CALIPER-derived features, particularly VRS scores, were among the strongest predictors of survival and FVC decline. CALIPER results were accentuated in patients with less extensive disease, outperforming pulmonary function measures. When used as a cohort enrichment tool, a CALIPER VRS score greater than 4.4% of the lung was able to reduce the requisite sample size of an IPF drug trial by 26%. CONCLUSIONS: Our study has validated a new quantitative CT measure in patients with IPF fulfilling drug trial entry criteria-the VRS score-that outperformed current gold standard measures of outcome. When used for cohort enrichment in an IPF drug trial setting, VRS threshold scores can reduce a required IPF drug trial population size by 25%, thereby limiting prohibitive trial costs. Importantly, VRS scores identify patients in whom antifibrotic medication prolongs life and reduces FVC decline.
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Fibrosis Pulmonar Idiopática/diagnóstico por imagen , Tomografía Computarizada por Rayos X/métodos , Anciano , Femenino , Humanos , Fibrosis Pulmonar Idiopática/diagnóstico , Fibrosis Pulmonar Idiopática/mortalidad , Fibrosis Pulmonar Idiopática/fisiopatología , Masculino , Persona de Mediana Edad , Pronóstico , Pruebas de Función Respiratoria , Capacidad VitalRESUMEN
BACKGROUND AND OBJECTIVE: This study evaluated whether patients with combined pulmonary fibrosis and emphysema (CPFE) have an increased likelihood of pulmonary hypertension (PHT) when compared with idiopathic pulmonary fibrosis (IPF) patients without emphysema. METHODS: Two consecutive IPF populations having undergone transthoracic echocardiography were examined (n = 223 and n = 162). Emphysema and interstitial lung disease (ILD) extent were quantified visually; ILD extent was also quantified by a software tool, CALIPER. Echocardiographic criteria categorized PHT risk. RESULTS: The prevalence of an increased PHT likelihood was 29% and 31% in each CPFE cohort. Survival at 12 months was 60% across both CPFE cohorts with no significantly worsened outcome identified when compared with IPF patients without emphysema. Using logistic regression models in both cohorts, total computed tomography (CT) disease extent (ILD and emphysema) predicted the likelihood of PHT. After adjustment for total disease extent, CPFE had no stronger association with PHT likelihood than IPF patients without emphysema. CONCLUSION: Our findings indicate that the reported association between CPFE and PHT is explained by the summed baseline CT extents of ILD and emphysema. Once baseline severity is taken into account, CPFE is not selectively associated with a malignant microvascular phenotype, when compared with IPF patients without emphysema.
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Hipertensión Pulmonar/epidemiología , Fibrosis Pulmonar Idiopática/epidemiología , Enfisema Pulmonar/epidemiología , Anciano , Ecocardiografía , Femenino , Humanos , Hipertensión Pulmonar/diagnóstico por imagen , Fibrosis Pulmonar Idiopática/complicaciones , Fibrosis Pulmonar Idiopática/diagnóstico por imagen , Funciones de Verosimilitud , Modelos Logísticos , Enfermedades Pulmonares Intersticiales/complicaciones , Enfermedades Pulmonares Intersticiales/diagnóstico por imagen , Enfermedades Pulmonares Intersticiales/epidemiología , Masculino , Prevalencia , Enfisema Pulmonar/complicaciones , Enfisema Pulmonar/diagnóstico por imagen , Estudios Retrospectivos , Factores de Riesgo , Índice de Severidad de la Enfermedad , Tomografía Computarizada por Rayos XRESUMEN
We conducted an international study of idiopathic pulmonary fibrosis (IPF) diagnosis among a large group of physicians and compared their diagnostic performance to a panel of IPF experts.A total of 1141 respiratory physicians and 34 IPF experts participated. Participants evaluated 60 cases of interstitial lung disease (ILD) without interdisciplinary consultation. Diagnostic agreement was measured using the weighted kappa coefficient (κw). Prognostic discrimination between IPF and other ILDs was used to validate diagnostic accuracy for first-choice diagnoses of IPF and were compared using the C-index.A total of 404 physicians completed the study. Agreement for IPF diagnosis was higher among expert physicians (κw=0.65, IQR 0.53-0.72, p<0.0001) than academic physicians (κw=0.56, IQR 0.45-0.65, p<0.0001) or physicians with access to multidisciplinary team (MDT) meetings (κw=0.54, IQR 0.45-0.64, p<0.0001). The prognostic accuracy of academic physicians with >20â years of experience (C-index=0.72, IQR 0.0-0.73, p=0.229) and non-university hospital physicians with more than 20â years of experience, attending weekly MDT meetings (C-index=0.72, IQR 0.70-0.72, p=0.052), did not differ significantly (p=0.229 and p=0.052 respectively) from the expert panel (C-index=0.74 IQR 0.72-0.75).Experienced respiratory physicians at university-based institutions diagnose IPF with similar prognostic accuracy to IPF experts. Regular MDT meeting attendance improves the prognostic accuracy of experienced non-university practitioners to levels achieved by IPF experts.
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Técnicas de Diagnóstico del Sistema Respiratorio/normas , Precisión de la Medición Dimensional , Fibrosis Pulmonar Idiopática/diagnóstico , Neumólogos/normas , Derivación y Consulta/normas , Competencia Clínica , Diagnóstico Diferencial , Femenino , Hospitales Universitarios/normas , Humanos , Cooperación Internacional , Masculino , Persona de Mediana Edad , Pronóstico , Calidad de la Atención de Salud/normas , Reproducibilidad de los ResultadosRESUMEN
The porcine model has contributed significantly to biomedical research over many decades. The similar size and anatomy of pig and human organs make this model particularly beneficial for translational research in areas such as medical device development, therapeutics and xenotransplantation. In recent years, a major limitation with the porcine model was overcome with the successful generation of gene-targeted pigs and the publication of the pig genome. As a result, the role of this model is likely to become even more important. For the respiratory medicine field, the similarities between pig and human lungs give the porcine model particular potential for advancing translational medicine. An increasing number of lung conditions are being studied and modeled in the pig. Genetically modified porcine models of cystic fibrosis have been generated that, unlike mouse models, develop lung disease similar to human cystic fibrosis. However, the scientific literature relating specifically to porcine lung anatomy and airway histology is limited and is largely restricted to veterinary literature and textbooks. Furthermore, methods for in vivo lung procedures in the pig are rarely described. The aims of this review are to collate the disparate literature on porcine lung anatomy, histology, and microbiology; to provide a comparison with the human lung; and to describe appropriate bronchoscopy procedures for the pig lungs to aid clinical researchers working in the area of translational respiratory medicine using the porcine model.
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Pulmón/anatomía & histología , Pulmón/fisiología , Animales , Investigación Biomédica , Biopsia , Bronquios/fisiología , Broncoscopía , Cartílago/fisiología , Modelos Animales de Enfermedad , Genoma , Humanos , Inflamación , Respiración , Porcinos , Investigación Biomédica Traslacional , Trasplante HeterólogoAsunto(s)
Fibrosis Pulmonar Idiopática/diagnóstico por imagen , Procesamiento de Imagen Asistido por Computador/métodos , Tomografía Computarizada por Rayos X , Humanos , Fibrosis Pulmonar Idiopática/mortalidad , Fibrosis Pulmonar Idiopática/terapia , Modelos Lineales , Reconocimiento de Normas Patrones Automatizadas , Modelos de Riesgos Proporcionales , Resultado del Tratamiento , Capacidad VitalRESUMEN
A novel actinomycete, strain N1286(T), isolated from a lung transplant patient with a pulmonary infection, was provisionally assigned to the genus Nocardia. The strain had chemotaxonomic and morphological properties typical of members of the genus Nocardia and formed a distinct phyletic line in the Nocardia 16S rRNA gene tree. Isolate N1286(T) was most closely related to Nocardia farcinica DSM 43665(T) (99.8% gene sequence similarity) but could be distinguished from the latter by the low level of DNA-DNA relatedness. These strains were also distinguishable on the basis of a broad range of phenotypic properties. It is concluded that strain N1286(T) represents a novel species of the genus Nocardia for which the name Nocardia kroppenstedtii sp. nov. is proposed. The type strain is N1286(T) (â=âDSM 45810(T)â=âNCTC 13617(T)).
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Enfermedades Pulmonares/microbiología , Nocardia/clasificación , Filogenia , Técnicas de Tipificación Bacteriana , ADN Bacteriano/genética , Ácidos Grasos/química , Humanos , Trasplante de Pulmón , Datos de Secuencia Molecular , Nocardia/genética , Nocardia/aislamiento & purificación , Hibridación de Ácido Nucleico , ARN Ribosómico 16S/genética , Análisis de Secuencia de ADN , Vitamina K 2/análogos & derivados , Vitamina K 2/químicaRESUMEN
INTRODUCTION: Sarcoidosis is a multi-system granulomatous disease most commonly involving the lungs. It may be incidentally diagnosed during imaging studies for other conditions or non-specific symptoms. The appropriate follow-up of incidentally diagnosed asymptomatic stage 1 disease has not been well defined. OBJECTIVE: To define the clinical course of incidentally diagnosed asymptomatic stage 1 sarcoidosis and propose an algorithm for the follow-up of these patients. METHODOLOGY: A retrospective case note analysis was performed of all EBUS-TBNA (endobronchial ultrasound-guided transbronchial needle aspiration)-confirmed cases of stage 1 sarcoidosis presenting incidentally to Bristol and Liverpool Interstitial Lung Disease services. Clinical history, serology results, imaging scans, and lung function parameters were examined at baseline, 12, and 24 months. A cost analysis was performed comparing the cost of the current 2-year follow-up guidance to a 1 year follow-up period. RESULTS: Sixty-seven patients were identified as the final cohort. There was no significant change in the pulmonary function tests over the two-year follow-up period. Radiological disease stability was observed in the majority of patients (58%, n = 29), and disease regression was evidenced in 40% (n = 20) at 1 year. Where imaging was performed at 2 years, the majority (69.8%, n = 37) had radiological evidence of disease regression, and 30.2% (n = 16) showed radiological evidence of stability. All patients remained asymptomatic and did not require therapeutic intervention over the study period. CONCLUSIONS: Our results show that asymptomatic patients with incidental findings of thoracic lymph nodal non-caseating granulomas do not progress over a 2-year period. Our results suggest that the prolonged secondary-care follow-up of such patients may not be necessary. We propose that these patients are followed up for 1 year with a further year of patient-initiated follow-up (PIFU) prior to discharge.
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PURPOSE OF REVIEW: Pulmonary arterial hypertension (PAH) is a complex disease with a high mortality. Management of this disease is underpinned by supportive and general therapies delivered by multidisciplinary teams in specialist centres. In recent years, a number of PAH-specific therapies have improved patient outcomes. This article will discuss the management of PAH in the context of relevant recently published studies in this area. RECENT FINDINGS: PAH-specific therapies are targeted towards dysfunctional signalling identified within the pulmonary circulation, and include endothelin receptor antagonists, phosphodiesterase type-5 inhibitors and prostanoids. Combination of these therapies is considered in patients with more severe disease. In addition, timely referral for surgical intervention (e.g. atrial septostomy, lung transplantation) should be made in selected patients with advanced disease. New treatment modalities currently in development may further improve patient outcomes in future years. However, further development and expansion of patient registries is vital for enhanced understanding of this disease, and may guide the optimal use of existing therapies and the development of new treatment approaches. SUMMARY: Outcomes in PAH have improved in recent years through a management approach characterized by general and supportive measures, and PAH-specific and surgical therapies in selected patients. Continued development of patient registries is vital to improve understanding and outcomes of this disease.
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Anticoagulantes/uso terapéutico , Antihipertensivos/uso terapéutico , Diuréticos/uso terapéutico , Antagonistas de los Receptores de Endotelina , Hipertensión Pulmonar/tratamiento farmacológico , Inhibidores de Fosfodiesterasa 5/uso terapéutico , Prostaglandinas/uso terapéutico , Terapia Combinada , Hipertensión Pulmonar Primaria Familiar , Femenino , Guías como Asunto , Humanos , Oxigenoterapia Hiperbárica , Hipertensión Pulmonar/mortalidad , Hipertensión Pulmonar/cirugía , Masculino , Evaluación de Resultado en la Atención de Salud , Selección de Paciente , Sistema de RegistrosRESUMEN
SARS-CoV-2 vaccination promises to improve outcomes for patients with COVID-19 pneumonia (most notably those with advanced age and at high risk for severe disease). Here, we examine serum 25-Hydroxyvitamin D (25(OH)D) status and outcomes in both old (>70 years) and young vaccinated (n = 80) and unvaccinated (n = 91) subjects, who were hospitalized due to COVID-19 pneumonia in a single center (Connolly Hospital Dublin). Outcomes included ICU admission and mortality. Serum 25(OH)D levels were categorized as D30 (<30 nmol/L), D40 (30-49.99 nmol/L) and D50 (≥50 nmol/L). In multivariate analyses, D30 was independently associated with ICU admission (OR: 6.87 (95% CI: 1.13-41.85) (p = 0.036)) and mortality (OR: 24.81 (95% CI: 1.57-392.1) (p = 0.023)) in unvaccinated patients, even after adjustment for major confounders including age, sex, obesity and pre-existing diabetes mellitus. While mortality was consistently higher in all categories of patients over 70 years of age, the highest observed mortality rate of 50%, seen in patients over 70 years with a low vitamin D state (D30), appeared to be almost completely corrected by either vaccination, or having a higher vitamin D state, i.e., mortality was 14% for vaccinated patients over 70 years with D30 and 16% for unvaccinated patients over 70 years with a 25(OH)D level greater than 30 nmol/L. We observe that high mortality from COVID-19 pneumonia occurs in older patients, especially those who are unvaccinated or have a low vitamin D state. Recent vaccination or having a high vitamin D status are both associated with reduced mortality, although these effects do not fully mitigate the mortality risk associated with advanced age.
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Vacunas contra la COVID-19 , COVID-19 , Humanos , Anciano , Anciano de 80 o más Años , SARS-CoV-2 , COVID-19/prevención & control , Vitamina D , Vitaminas , Hospitales , VacunaciónRESUMEN
The aim of this study was to evaluate the risk factors for and outcomes of acute exacerbations in patients with advanced idiopathic pulmonary fibrosis (IPF), and to examine the relationship between disease severity and neovascularisation in explanted IPF lung tissue. 55 IPF patients assessed for lung transplantation were divided into acute (n=27) and non-acute exacerbation (n=28) groups. Haemodynamic data was collected at baseline, at the time of acute exacerbation and at lung transplantation. Histological analysis and CD31 immunostaining to quantify microvessel density (MVD) was performed on the explanted lung tissue of 13 transplanted patients. Acute exacerbations were associated with increased mortality (p=0.0015). Pulmonary hypertension (PH) at baseline and acute exacerbations were associated with poor survival (p<0.01). PH at baseline was associated with a significant risk of acute exacerbations (HR 2.217, p=0.041). Neovascularisation (MVD) was significantly increased in areas of cellular fibrosis and significantly decreased in areas of honeycombing. There was a significant inverse correlation between mean pulmonary artery pressure and MVD in areas of honeycombing. Acute exacerbations were associated with significantly increased mortality in patients with advanced IPF. PH was associated with the subsequent development of an acute exacerbation and with poor survival. Neovascularisation was significantly decreased in areas of honeycombing, and was significantly inversely correlated with mean pulmonary arterial pressure in areas of honeycombing.
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Hipertensión Pulmonar/complicaciones , Fibrosis Pulmonar Idiopática/complicaciones , Pulmón/fisiopatología , Enfermedad Aguda , Femenino , Hemodinámica , Humanos , Hipertensión Pulmonar/mortalidad , Fibrosis Pulmonar Idiopática/mortalidad , Fibrosis Pulmonar Idiopática/cirugía , Trasplante de Pulmón , Masculino , Persona de Mediana Edad , Neovascularización Patológica , Pronóstico , Factores de Riesgo , Análisis de SupervivenciaRESUMEN
COVID-19 and a low vitamin D state share common risk factors, which might explain why vitamin D deficiency has been linked with higher COVID-19 mortality. Moreover, measures of serum vitamin D may become lower during systemic inflammatory responses, further confounding the association via reverse causality. In this prospective study (recruited over 12 months), we examined whether the association between a low vitamin D state and in-hospital mortality due to SARS-CoV-2 pneumonia in unvaccinated subjects is explained by (i) the presence of shared risk factors (e.g., obesity, advanced age) or (ii) a reduction in serum 25(OH)D due to COVID-19 (i.e., reverse causality). In this cohort of 232 (mean age = 56 years) patients (all had SARS-CoV-2 diagnosed via PCR AND required supplemental oxygen therapy), we failed to find an association between serum vitamin D and levels of CRP, or other inflammatory markers. However, the hazard ratio for mortality for subjects over 70 years of age (13.2) and for subjects with a serum 25(OH)D level less than 30 nmol·L−1 (4.6) remained significantly elevated even after adjustment for gender, obesity and the presence of diabetes mellitus. Subjects <70 years and >70 years had significantly higher mortality with a serum 25(OH)D less than 30 nmol·L−1 (11.8% and 55%), than with a serum 25(OH)D greater than 30 nmol·L−1 (2.2% and 25%). Unvaccinated Caucasian adults with a low vitamin D state have higher mortality due to SARS CoV-2 pneumonia, which is not explained by confounders and is not closely linked with elevated serum CRP.
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COVID-19 , Deficiencia de Vitamina D , Adulto , Anciano , Anciano de 80 o más Años , Humanos , Persona de Mediana Edad , Obesidad , Estudios Prospectivos , SARS-CoV-2 , Vitamina D , VitaminasRESUMEN
BACKGROUND: In cohort studies of pulmonary sarcoidosis, abnormal ventilatory patterns have generally been subdivided into restrictive and obstructive defects. Mixed ventilatory defects have largely been overlooked in pulmonary sarcoidosis, as total lung capacity has seldom been taken into account in historical series. RESEARCH QUESTION: This study evaluated the prevalence of mixed disease in pulmonary sarcoidosis and its clinical associations. STUDY DESIGN AND METHODS: In patients with pulmonary sarcoidosis (N = 1,110), mixed defects were defined according to American Thoracic Society/European Respiratory Society criteria. Clinical data, pulmonary function variables, and vital status were abstracted from clinical records. Chest radiographs were evaluated independently by two experienced radiologists. RESULTS: The prevalence of a mixed ventilatory defect was 10.4% in the whole cohort, rising to 25.9% in patients with airflow obstruction. Compared with isolated airflow obstruction, mixed defects were associated with lower diffusing lung capacity for carbon monoxide levels (50.7 ± 16.3 vs 70.8 ± 18.1; P < .0001), a higher prevalence of chest radiographic stage IV disease (63.5% vs 38.3%; P < .0001), and higher mortality (hazard ratio, 2.36; 95% CI, 1.34-4.15; P = .003). These findings were reproduced in all patient subgroup analyses, including patients with a histologic diagnosis, a clinical diagnosis, incident disease, and prevalent disease. INTERPRETATION: Mixed disease is present in approximately 25% of patients with pulmonary sarcoidosis and airflow obstruction and is associated with lower diffusing lung capacity for carbon monoxide levels, a higher prevalence of stage IV disease, and higher mortality than seen in a pure obstructive defect. These observations identify a distinct phenotype associated with a mixed ventilatory defect, justifying future studies of its clinical and pathogenetic significance.
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Volumen Espiratorio Forzado/fisiología , Pulmón/fisiopatología , Capacidad de Difusión Pulmonar/fisiología , Sarcoidosis Pulmonar/fisiopatología , Inglaterra/epidemiología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Prevalencia , Estudios Retrospectivos , Sarcoidosis Pulmonar/epidemiología , EspirometríaRESUMEN
PURPOSE: To determine the prevalence and severity of tracheomalacia in adults with cystic fibrosis (CF) by using dynamic cine multidetector computed tomography (CT) and to correlate these findings with pulmonary function test (PFT) results and the severity of parenchymal lung disease. MATERIALS AND METHODS: In this institutional review board-approved HIPAA-compliant study, 40 patients with CF (22 men, 18 women; mean age, 28 years +/- 8 [standard deviation]; age range, 18-54 years) prospectively underwent PFTs, standard thin-section CT, and two dynamic cine multidetector CT acquisitions. Ten control subjects underwent dynamic cine multidetector CT. After standard thin-section CT was completed, dynamic cine multidetector CT was performed during a forced expiratory maneuver and during coughing. Dynamic cine multidetector CT images in nine patients were excluded. Maximal inspiratory, dynamic expiratory, and end-expiratory tracheal luminal areas were compared (Student t test) and correlated (Spearman rank) with PFT results and severity of parenchymal lung disease. RESULTS: Mean predicted forced expiratory volume in 1 second (FEV(1)) was 70.6% +/- 20.7, and mean Bhalla CT score was 41.8% +/- 13.6. In patients with CF, dynamic cine mean tracheal cross-sectional area reduction was 51.7% +/- 18.4 (range, 9%-89%) for forced expiratory maneuvers and 68.8% +/- 11.7 (range, 18%-88%) for coughing (P = .001). Tracheomalacia was demonstrated in 24 (69%) patients and no control subjects during forced expiratory maneuvers (P = .001) and in 10 (29%) patients and one (10%) control subject during coughing. For end-expiration images, mean tracheal luminal reduction was 16.1% +/- 14.0% (range, 0.0%-53.0%), with one patient demonstrating tracheal luminal reduction of more than 50%. There was no correlation between tracheal cross-sectional luminal reduction and either predicted FEV(1) or CT Bhalla score. CONCLUSION: Tracheomalacia depicted at dynamic cine multidetector CT is a highly prevalent finding in adults with CF. SUPPLEMENTAL MATERIAL: http://radiology.rsnajnls.org/cgi/content/full/2522081956/DC1.
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Fibrosis Quística/complicaciones , Fibrosis Quística/diagnóstico por imagen , Tomografía Computarizada por Rayos X/métodos , Traqueomalacia/complicaciones , Traqueomalacia/diagnóstico por imagen , Adolescente , Adulto , Humanos , Masculino , Prevalencia , Reproducibilidad de los Resultados , Sensibilidad y Especificidad , Adulto JovenRESUMEN
Bilateral diaphragmatic paralysis is a known cause of respiratory failure. Diagnosis can be difficult, particularly in the acute setting. We present the case of a gentleman diagnosed with bilateral diaphragmatic paralysis secondary to phrenic neuropathy in the setting of cervical spondylosis.
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Disnea , Enfermedades del Sistema Nervioso Periférico , Nervio Frénico/fisiopatología , Parálisis Respiratoria , Médula Cervical/diagnóstico por imagen , Diagnóstico Diferencial , Humanos , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Radiografía TorácicaRESUMEN
BACKGROUND: FEV1 may remain stable while high-resolution CT (HRCT) appearances deteriorate in children with cystic fibrosis (CF). However, spirometry results commonly decline in older age groups. OBJECTIVES: To compare the rate of decline in HRCT abnormalities and spirometry results over time in an adult cohort with CF. METHODS: The HRCT scans of 39 consecutive patients (19 males and 20 females; mean age, 22 years; range, 16 to 48 years) with two HRCT scans > 18 months apart were randomly and blindly scored using a modified Bhalla scoring system by two independent chest radiologists. Age, body mass index, spirometry, and sputum cultures were recorded at the time of both HRCTs. Rates of change in clinical parameters and HRCT abnormalities were calculated and compared using repeated-measures analysis of variance. RESULTS: Mean FEV1 declined at a rate of - 2.3% per year, while mean HRCT total score declined at a rate of -2.7% per year. Several individual HRCT abnormalities as well as HRCT total scores declined significantly faster than FEV1 (p < 0.001). Six patients showed stable spirometry results but worsening HRCT scores. Mucus plugging and extent of bronchiectasis deteriorated at a more rapid rate in the group with mildly impaired lung function. Air trapping, collapse/consolidation, peribronchial thickening, severity of bronchiectasis, and generations of bronchial divisions involved deteriorated at a more rapid rate in the group with moderate-to-severely impaired lung function. CONCLUSIONS: Adult CF patients have more rapid rates of decline in HRCT abnormalities than in spirometry results. Individual HRCT abnormalities decline at different rates depending on the degree of lung function impairment.
Asunto(s)
Fibrosis Quística/diagnóstico por imagen , Fibrosis Quística/fisiopatología , Volumen Espiratorio Forzado/fisiología , Pulmón/anomalías , Pulmón/diagnóstico por imagen , Tomografía Computarizada por Rayos X , Adolescente , Adulto , Análisis de Varianza , Bronquiectasia/patología , Fibrosis Quística/patología , Femenino , Humanos , Estudios Longitudinales , Pulmón/patología , Masculino , Persona de Mediana Edad , Moco , Pseudomonas aeruginosa/patogenicidad , Espirometría , Esputo/microbiologíaRESUMEN
Pulmonary Nodular Lymphoid Hyperplasia (PNLH) is a rare benign pulmonary disorder characterized by a localized, reactive polyclonal lymphoproliferation. Although the radiological features of this disease have not been clearly defined, they usually consist of a solitary non-cavitatory pulmonary nodule. In this report, we describe two cases of histologically confirmed PNLH presenting as multiple bilateral cavitatory lesions on CT Thorax.