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BACKGROUND: Different patterns of fibrosis on high-resolution CT scans (HRCT) have been associated with reduced survival in some interstitial lung diseases. Nothing is known about HRCT scan patterns and survival in sarcoidosis. RESEARCH QUESTION: Will a detailed description of the extent and pattern of HRCT scan fibrosis in patients with stage IV pulmonary sarcoidosis impact pulmonary function and survival? STUDY DESIGN AND METHODS: Two hundred forty patients with stage IV sarcoidosis at two large tertiary institutions were studied. The earliest HRCT scan with fibrosis was reviewed for extent of fibrosis (< 10%, 10%-20%, and > 20%) and presence of bronchiectasis, upper lobe fibrocystic changes, basal subpleural honeycombing, ground-glass opacities (GGOs), large bullae, and mycetomas. Presence of sarcoidosis-associated pulmonary hypertension (SAPH) and pulmonary function testing performed within 1 year of HRCT were recorded. Patients were followed up until last clinic visit, death, or lung transplantation. RESULTS: The mean age was 58.4 years. Seventy-four percent were Black, 63% were female, and mean follow-up was 7.4 years. Death or LT occurred in 53 patients (22%). Thirty-one percent had > 20% fibrosis, 25% had 10%-20% fibrosis, and 44% had < 10% fibrosis. The most common HRCT abnormalities were bronchiectasis (76%), upper lobe fibrocystic changes (36%), and GGOs (28%). Twelve percent had basal subpleural honeycombing, and 32% had SAPH. Patients with > 20% fibrosis had more severe pulmonary impairment, were more likely to have SAPH (53%), and had worse survival (44% mortality; P < .001). Upper lobe fibrocystic changes, basal subpleural honeycombing, and large bullae were associated with worse pulmonary function and worse survival. Patients with basal subpleural honeycombing had the worst pulmonary function and survival (55% mortality; P < .001). GGOs were associated with worse pulmonary function but not worse survival, and mycetomas were associated with worse survival but not worse pulmonary function. A Cox proportional hazards model indicated that basal subpleural honeycombing (hazard ratio, 7.95), diffusion capacity for carbon monoxide < 40% (HR, 5.67) and White race (hazard ratio, 2.61) were independent predictors of reduced survival. INTERPRETATION: HRCT scan features of fibrotic pulmonary sarcoidosis had an impact on pulmonary function and survival. Presence of >20% fibrosis and basal subpleural honeycombing are predictive of worse pulmonary function and worse survival in patients with stage IV pulmonary sarcoidosis.
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Bronquiectasia , Sarcoidosis Pulmonar , Sarcoidosis , Humanos , Femenino , Persona de Mediana Edad , Masculino , Sarcoidosis Pulmonar/complicaciones , Sarcoidosis Pulmonar/diagnóstico por imagen , Sarcoidosis Pulmonar/patología , Vesícula , Pulmón/diagnóstico por imagen , Pulmón/patología , Fibrosis , Tomografía Computarizada por Rayos X , Sarcoidosis/patología , Bronquiectasia/patología , Estudios RetrospectivosRESUMEN
INTRODUCTION: The pathogenesis of non-cystic fibrosis bronchiectasis has not been clearly clarified. This study aimed to investigate the expression of ciliary regulating protein forkhead box protein j1 (Foxj1) on airway epithelium in non-cystic fibrosis bronchiectasis and its association with airway cilia structure disorder and disease severity. METHODS: Lung tissue sections excised from 47 patients with non-cystic fibrosis bronchiectasis were included between January 2018 and June 2021. Specimens from 26 subjects who underwent a lobectomy due to lung nodule were chosen as controls. Clinical information was collected, and pathologic analysis was performed to assess the epithelial structure and expression of ciliary regulating Foxj1. RESULTS: Of the 47 patients with non-cystic fibrosis bronchiectasis, 25 were considered as mild, 12 were moderate whereas the remaining 10 cases were severe according to the bronchiectasis severity index score evaluation. Epithelial hyperplasia, hyperplasia of goblet cells and inflammatory cell infiltration were observed in non-cystic fibrosis bronchiectasis, compared with control subjects. Cilia length in non-cystic fibrosis bronchiectasis patients were shorter than that in the control group, (5.34 ± 0.89) µm versus (7.34 ± 0.71) µm, respectively (P = 0.002). The expression of Foxj1 was (2.69 ± 1.09) × 106 in non-cystic fibrosis bronchiectasis, compared with (6.67 ± 1.15) × 106 in the control group (P = 0.001). Moreover, patients with lower expression of Foxj1 showed shorter airway cilia and worse in disease severity. CONCLUSION: Foxj1 declined in the airway epithelium of patients with non-cystic fibrosis bronchiectasis, positively correlated to cilia length and might imply worse disease severity.
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Bronquiectasia , Cilios , Factores de Transcripción Forkhead , Humanos , Bronquiectasia/patología , Epitelio/metabolismo , Factores de Transcripción Forkhead/metabolismo , Hiperplasia/metabolismo , Hiperplasia/patología , Pulmón/patología , Gravedad del PacienteRESUMEN
OBJECTIVES: Disease-modifying agents (DMAs) for the treatment of spinal muscular atrophy (SMA) have evolved the SMA phenotype with improved survival. Ongoing oropharyngeal dysphagia and respiratory complications are reported. The extent of dysphagia and respiratory morbidity in this population, since DMAs' introduction, has not been well described. METHODS: A whole-population study involved all children with treated SMA types 1-3 in our facility. Videofluoroscopic swallow studies (type 1 alone), chest CT scans, and clinical data were collected. RESULTS: Thirty-six children were included (n = 9 type 1, n = 14 type 2, and n = 13 type 3; age range 0.3-15.4 years). Abnormal swallowing characteristics were demonstrated in all children with type 1 (n = 8; 100%). Bronchiectasis was found on chest CT: 3 of 9 (33.3%), 2 of 14 (14.3%), and 2 of 13 (15.4%) of type 1, 2, and 3, respectively. Atelectasis, mucus plugging, bronchial wall thickening, and parenchymal changes were common. DISCUSSION: Swallow impairments were universal in children with type 1. Bronchiectasis was common in all pediatric SMA types, with a prevalence of 1 in 5. Routine monitoring and management of dysphagia/recurrent respiratory infection should be implemented for improvement in lung health.
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Bronquiectasia , Trastornos de Deglución , Enfermedades Pulmonares , Atrofias Musculares Espinales de la Infancia , Niño , Preescolar , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Bronquiectasia/complicaciones , Bronquiectasia/diagnóstico por imagen , Bronquiectasia/patología , Estudios Transversales , Trastornos de Deglución/complicaciones , Trastornos de Deglución/diagnóstico , Trastornos de Deglución/fisiopatología , Enfermedades Pulmonares/complicaciones , Enfermedades Pulmonares/diagnóstico por imagen , Enfermedades Pulmonares/patología , Atelectasia Pulmonar/complicaciones , Atelectasia Pulmonar/diagnóstico por imagen , Atrofias Musculares Espinales de la Infancia/clasificación , Atrofias Musculares Espinales de la Infancia/complicaciones , Atrofias Musculares Espinales de la Infancia/tratamiento farmacológico , Atrofias Musculares Espinales de la Infancia/fisiopatologíaRESUMEN
BACKGROUND: Non cystic fibrosis, non primary ciliary dyskinesia bronchiectasis (nCFnPCD-BE) results in significant morbidity with few evidence-based treatments. OBJECTIVE: assessments are required to assess severity and evaluate treatment. Lung clearance index (LCI) measures ventilation inhomogeneity and is a sensitive test of disease in CF; its use in nCFnPCD-BE is unclear. METHODS: A systematic review of LCI in nCFnPCD-BE was performed using standard methodology (protocol registered on PROSPERO, University of York). RESULTS: Of 276 records identified, 12 articles, describing 519 adult and paediatric patients in cross-sectional studies were included, addressing several domains. 1: What is the utility of LCI in detecting disease and severity? LCI detected disease in adults, differentiating bronchiectasis from controls (AUC 0.90 to 0.96) and mild from moderate/severe bronchiectasis on CT (AUC 0.73). 2: Does LCI correlate with spirometry and imaging? LCI correlated with spirometry in adult (r = -0.37 to -0.61) and paediatric (r = -0.6) groups, signs of bronchiectasis on CT, and CT scoring systems (modified Reiff). 3: Does LCI relate to subjective scores of severity? In adults, LCI correlated with St. George's Respiratory Questionnaire (r = 0.18) and Bronchiectasis Severity Index (r = 0.45). 4: Does LCI identify response to intervention? LCI did not change in studies examining LCI pre-post intervention (adults treated for exacerbation and undergoing physiotherapy). Overall study quality was variable. CONCLUSION: Contrary to data in CF, the review did not identify good quality studies defining the role of LCI in children with bronchiectasis. In adults, LCI was a sensitive measure of disease severity and correlated with clinical assessment tools.
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Bronquiectasia , Trastornos de la Motilidad Ciliar , Adulto , Bronquiectasia/diagnóstico por imagen , Bronquiectasia/patología , Niño , Estudios Transversales , Fibrosis , Volumen Espiratorio Forzado/fisiología , Humanos , Pulmón/diagnóstico por imagen , Pulmón/patologíaRESUMEN
INTRODUCTION: There is sparse evidence in the literature in relation to the chest computed tomography (CT) findings among adult Indigenous Australians with chronic respiratory conditions. METHODS: In this retrospective study, patients who underwent chest CT between 2012 and 2020 among those referred to undergo lung function tests (spirometry) were assessed for the prevalence of abnormal chest CT radiological findings. RESULTS: Of the 402 patients (59% female) included in this study, 331 (82%) had an abnormality identified on chest CT. Most abnormalities occurred alongside one (25%) or multiple (46%) other CT abnormalities. Airway disease ((AD) (including, emphysema, airway wall thickening and small airway disease) (35%), atelectasis: segmental or lobar collapse (27%), inflammatory opacities (24%) and bronchiectasis (23%) were the most common findings. AD and bronchiectasis were also the most common concurrent abnormalities in 40-50%. Other CT abnormalities noted in isolation or in combination with other CT findings were lung nodules (19%), lymph node enlargement (17%), consolidation or mass (17%), followed by lung cysts, ground-glass opacity, lung parenchymal architectural distortion, cavitating lung lesions and chronic pleural effusion were observed in ≤10%. Predictive models for odds of abnormality and outcomes showed age, smoking and underweight were associated with AD, and male sex and very remote residence were associated with bronchiectasis. CONCLUSION: This study has illustrated that Indigenous Australian adults have a high prevalence of multiple chest CT abnormalities that may impose unprecedented diagnostic and therapeutic challenges in this population. Further studies are warranted to determine the long-term implications and prognostic significance of the CT findings as demonstrated in this study.
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Bronquiectasia , Enfermedad Pulmonar Obstructiva Crónica , Adulto , Bronquiectasia/diagnóstico por imagen , Bronquiectasia/epidemiología , Bronquiectasia/patología , Femenino , Humanos , Pulmón/diagnóstico por imagen , Pulmón/patología , Masculino , Northern Territory/epidemiología , Enfermedad Pulmonar Obstructiva Crónica/patología , Estudios Retrospectivos , Tomografía Computarizada por Rayos X/métodosAsunto(s)
Inmunodeficiencia Variable Común/etiología , Hipertensión Pulmonar/complicaciones , Hipertensión Pulmonar/patología , Bronquiectasia/patología , Inmunodeficiencia Variable Común/diagnóstico , Humanos , Enfermedades Pulmonares Intersticiales/patología , Enfermedad Pulmonar Obstructiva Crónica/patologíaRESUMEN
Airway inflammation plays a central role in bronchiectasis. Protease-antiprotease balance is crucial in bronchiectasis pathophysiology and increased presence of unopposed proteases activity may contribute to bronchiectasis onset and progression. Proteases' over-reactivity and antiprotease deficiency may have a role in increasing inflammation in bronchiectasis airways and may lead to extracellular matrix degradation and tissue damage. Imbalances in serine proteases and matrix-metallo proteinases (MMPs) have been associated to bronchiectasis. Active neutrophil elastase has been associated with disease severity and poor long-term outcomes in this disease. Moreover, high levels of MMPs have been associated with radiological and disease severity. Finally, severe deficiency of α1-antitrypsin (AAT), as PiSZ and PiZZ (proteinase inhibitor SZ and ZZ) phenotype, have been associated with bronchiectasis development. Several treatments are under study to reduce protease activity in lungs. Molecules to inhibit neutrophil elastase activity have been developed in both oral or inhaled form, along with compounds inhibiting dipeptydil-peptidase 1, enzyme responsible for the activation of serine proteases. Finally, supplementation with AAT is in use for patients with severe deficiency. The identification of different targets of therapy within the protease-antiprotease balance contributes to a precision medicine approach in bronchiectasis and eventually interrupts and disrupts the vicious vortex which characterizes the disease.
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Bronquiectasia/metabolismo , Péptido Hidrolasas/metabolismo , Inhibidores de Proteasas/metabolismo , Deficiencia de alfa 1-Antitripsina/metabolismo , Bronquiectasia/enzimología , Bronquiectasia/genética , Bronquiectasia/patología , Humanos , Elastasa de Leucocito , Pulmón/metabolismo , Pulmón/patología , Metaloproteinasas de la Matriz/genética , Metaloproteinasas de la Matriz/metabolismo , Neutrófilos/metabolismo , Neutrófilos/patología , Serina Proteasas/genética , Serina Proteasas/metabolismo , Deficiencia de alfa 1-Antitripsina/genética , Deficiencia de alfa 1-Antitripsina/patologíaRESUMEN
BACKGROUND: Serum biomarkers associated with the severity of non-cystic fibrosis (CF) bronchiectasis are insufficient. This study determined the association of serum hepatocyte growth factor (HGF), osteopontin, and pentraxin-3 levels with disease severity and exacerbation in patients with non-CF bronchiectasis. METHODS: Serum levels of HGF, osteopontin, and pentraxin-3 were measured in patients with clinically stable non-CF bronchiectasis (n = 61). The correlation between the biomarkers and bronchiectasis severity index (BSI) and FACED score was assessed using univariate and multivariate linear regression analyses. Predictive variables associated with exacerbation were analyzed using a Cox proportional hazards model and the time to first exacerbation in high and low HGF groups during the observation period was compared using Kaplan-Meier survival curves. RESULTS: The BSI showed significant correlation with HGF (r = 0.423; p = 0.001) and pentraxin-3 (r = 0.316; p = 0.013). The FACED score was significantly correlated with HGF (r = 0.406; p = 0.001). Univariate and multivariate linear regression analysis revealed that serum level of HGF was independently associated with both scoring systems. The high HGF group showed a significantly shorter time to first exacerbation (Log-rank test, p = 0.014). Multivariate Cox proportional hazards regression analysis revealed that high serum HGF level and colonization with non-pseudomonas organisms were independent predictors of future exacerbations (HR 2.364; p = 0.024 and HR 2.438; p = 0.020, respectively). CONCLUSION: Serum level of HGF is a potential biomarker that is closely associated with disease severity and future risk of exacerbations in patients with non-CF bronchiectasis.
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Bronquiectasia/diagnóstico , Factor de Crecimiento de Hepatocito/sangre , Anciano , Biomarcadores/sangre , Bronquiectasia/mortalidad , Bronquiectasia/patología , Proteína C-Reactiva , Progresión de la Enfermedad , Femenino , Humanos , Modelos Lineales , Masculino , Persona de Mediana Edad , Osteopontina/sangre , Valor Predictivo de las Pruebas , Modelos de Riesgos Proporcionales , Riesgo , Componente Amiloide P Sérico , Índice de Severidad de la Enfermedad , Tasa de Supervivencia , Factores de TiempoRESUMEN
BACKGROUND: Bronchiectasis severity Index (BSI) score which predicts the severity of the disease along with future exacerbations and mortality rate has been well validated in European patients; however there is paucity of data evaluating its validity in Indian patients. The authors therefore decided to evaluate the utility of BSI to predict exacerbations and mortality rate in patients with post tubercular bronchiectasis presenting to our facility. METHODS: The study was a retrospective observational study done in patients with bronchiectasis secondary to tuberculosis. These patients were followed up for 4 years. BSI was calculated from different variables and descriptive statistics along with regression analysis were used to evaluate utility of BSI. RESULTS: A total of 48 patients of post tubercular bronchiectasis were included in the study. Majority of our patients belonged to severe bronchiectasis group seen in 23 patients (48%) while those with mild and moderate bronchiectasis were seen in 13 (27%) and 12 (25%) patients respectively. The exacerbation rate in mild group was comparable to the predicted BSI exacerbation at 1 year while the predicted and observed rates were statistically significant for moderate and severe bronchiectasis group (p value < 0.05). Mortality rates at 1 year were comparable in all the groups of bronchiectasis while it was comparable only in mild and moderate group bronchiectasis at 4 years. CONCLUSION: Bronchiectasis severity index seems to predict mortality at 1 year in post tuberculosis bronchiectasis. However, it under predicts 1 year and 4 year exacerbation rates. Hence BSI may not be useful as a prognostic tool in Indian patients with bronchiectasis. Larger multi-centred studies may be required to further evaluate the clinical utility of BSI among Indian population.
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Bronquiectasia/diagnóstico , Índice de Severidad de la Enfermedad , Tuberculosis Pulmonar/diagnóstico , Bronquiectasia/mortalidad , Bronquiectasia/patología , Femenino , Humanos , India , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Centros de Atención Terciaria , Tuberculosis Pulmonar/mortalidad , Tuberculosis Pulmonar/patologíaRESUMEN
ABSTRACT: The incidence of pulmonary nontuberculous mycobacterial (NTM) infection is high in patients with underlying lung disease such as bronchiectasis. Although previous studies have reported many risk factors contributing to the development of NTM-lung disease (LD), only a few reports on the relationship of the characteristics of patients, such as body mass index (BMI), skeletal mass, and fat mass, with NTM-LD have been published. We aimed to investigate the association between these parameters and NTM-LD in patients with bronchiectasis.A monocentric retrospective study in a university hospital was conducted over 4âyears (2013-2016). Parameters including BMI, skeletal mass, and fat mass were measured using bioelectrical impedance analysis in noncystic fibrosis bronchiectasis patients. Patients were grouped by the presence or absence of NTM-LD, and the differences in BMI, skeletal mass, and fat mass between the 2 groups were compared. In the NTM-LD group, the progression of disease was also followed.Two hundred forty-five patients with bronchiectasis were enrolled in the study. One hundred six subjects (48%) had NTM-LD. These patients with NTM-LD were predominantly female, and had a significantly lower body weight (58.20â±â8.84 vs 54.16â±â8.99, Pâ<â.001), BMI (22.67â±â3.04 vs 21.20â±â2.59âkg/m2, Pâ<â.001), fat mass (16.19â±â6.60 vs 14.23â±â5.79, Pâ=â.013), and fat mass index (FMI; 6.79â±â2.70 vs 5.57â±â2.27âkg/m2, Pâ<â.001). Multivariate regression analysis showed that both female sex and lower FMI but not skeletal muscle index were independent risk factors for NTM-LD after adjusting for age, bronchiectasis severity index, and BMI (odds ratio 3.86 (1.99-7.78); 0.72 (0.63-0.82), Pâ<â.001, respectively).Our results suggest that lower FMI may contribute to susceptibility to NTM infection in patients with bronchiectasis, independent of age or its severity.
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Composición Corporal , Bronquiectasia/complicaciones , Infecciones por Mycobacterium no Tuberculosas/complicaciones , Adulto , Anciano , Índice de Masa Corporal , Bronquiectasia/diagnóstico por imagen , Bronquiectasia/patología , Estudios de Casos y Controles , Femenino , Humanos , Masculino , Persona de Mediana Edad , Infecciones por Mycobacterium no Tuberculosas/microbiología , Estudios Retrospectivos , Factores de RiesgoRESUMEN
Background: Distal airway metaplasia may precede honeycombing in progressive fibrosing interstitial lung disease (ILD). The SCGB1A1+ bronchiolar-specific club cell may play a role in this aberrant regenerative process. Objective: To assess the presence of club cells in the small airways of patients suffering from ILD. Methods: Small airways (internal diameter <2 mm) in lung samples [surgical lung biopsy (SLB) and/or transbronchial lung cryobiopsy (TBLC)] from 14 patients suffering from ILD and 10 controls were morphologically assessed and stained for SCGB1A1. SCGB1A1 was weighted by epithelial height as a marker of airway generation (SCGB1A1/EH). Correlations between clinical, functional, and high-resolution CT (HRCT) prognostic factors and histomorphometry were assessed. Results: Small airways from samples with ILD patterns were significantly less dense in terms of SCGB1A1+ cells [0.064 (0.020-0.172)] as compared to controls' sample's small airways [0.393 (0.082-0.698), p < 0.0001]. Usual interstitial pneumonia (UIP) patterns most frequently contained small airways with limited or absent SCGB1A1 expression (SCGB1A1/EH <0.025): UIP (18/33; 55%) as compared with non-UIP patterns (4/31; 13%) or controls (0/29; 0%): p < 0.0001. In addition, correlations with HRCT indicated a significant negative relationship between SCGB1A1 and bronchiectasis as a feature of bronchiolization (Rho -0.63, p < 0.001) and a positive relationship with both forced vital capacity (FVC) and Hounsfield unit (HU)-distribution pattern in kurtosis (Rho 0.38 and 0.50, respectively, both p < 0.001) as markers of fibrotic changes. Conclusion: Compared with controls, the small airways of patients with ILD more often lack SCGB1A1, especially so in UIP. Low densities of SCGB1A1-marked cells correlate with bronchiectasis and fibrotic changes. Further research investigating SCGB1A1 staining as a pathological feature of the bronchiolization process is merited.
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Enfermedades Pulmonares Intersticiales/metabolismo , Enfermedades Pulmonares Intersticiales/patología , Metaplasia/patología , Adulto , Anciano , Bronquiectasia/patología , Bronquiolos/patología , Células Epiteliales/patología , Femenino , Humanos , Pulmón/patología , Masculino , Metaplasia/fisiopatología , Persona de Mediana Edad , Estudios Prospectivos , Fumar , Uteroglobina/metabolismoRESUMEN
A total of 115 convalescent inpatients with COVID-19 were enrolled. According to the results of scans of lung lesions via computed tomography (CT), the patients were divided into mild, moderate, and severe groups. The clinical data of the patients were collected, including age, gender, finger pulse oxygen pressure, ventricular rate, body temperature, etc. The correlation between the clinical indicators and the lesions of high-resolution CT (HRCT) and bronchiectasis was analyzed. Among the 115 patients, 82 had no bronchiectasis and 33 had bronchiectasis. The bronchodilation-prone layers mainly included the left and right lower lobe of the lung. The probability of branching in the inflamed area was greater than that in the noninflamed area in patients with COVID-19. There were significant differences in gender, CT lesion range, and number of incidents of bronchiectasis between noninflamed and inflamed areas (P < 0.05). Moreover, there were significant differences in age, total proportion of CT lesions, volume of CT lesions, and total number of patients with bronchiectasis among the three groups (P < 0.05). CT lesion range was positively correlated with the total number of patients with bronchiectasis and patient age (respectively, r = 0.186, P < 0.05; r = 0.029, P < 0.05). The lesion range in HRCT images of lungs in patients with COVID-19 is correlated with bronchodilation. The larger the lesion, the higher the probability of bronchiectasis and the more incidents of bronchiectasis.
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Bronquiectasia/patología , Bronquiectasia/virología , COVID-19/patología , COVID-19/virología , Pulmón/patología , Pulmón/virología , Neumonía/patología , Neumonía/virología , Adulto , Femenino , Humanos , Masculino , Estudios Retrospectivos , SARS-CoV-2/patogenicidad , Índice de Severidad de la Enfermedad , Tomografía Computarizada por Rayos X/métodosRESUMEN
STAT1 gain-of-function (GOF) mutations are associated with a rare autosomal dominant immunodeficiency disorder with main clinical manifestations including chronic mucocutaneous candidiasis (CMC) and bronchiectasis. In addition, these patients show higher incidences of cerebral and extracerebral aneurysm, malignancies and various autoimmune conditions compared to the general population. Although previous publications have reported clinical findings in patients with STAT1 GOF mutation, they did not include histopathologic features. Herein, we describe the first case with detailed histologic findings in the lung of a 5-year-old patient with a de novo STAT1 GOF mutation, who presented with CMC and bronchiectasis. The biopsy showed severe bronchiolectasis with extensive airway dilatation and occasional disruptions. Peribronchiolar inflammation was not always present and evident mainly in areas of airway disruption; inflammation may have not been a main driver of the airway damage in this case. The airway dilatation often showed an interesting herniating pattern, possibly implying a connective tissue etiology. This case also demonstrates the diagnostic utility of whole exome sequencing as STAT1 GOF mutations are not detected by routine workup. The definitive diagnosis will lead to more specific treatments and increased surveillance for serious conditions, such as cerebral aneurysms and malignancies.
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Bronquiectasia/diagnóstico , Mutación con Ganancia de Función , Factor de Transcripción STAT1/genética , Bronquiectasia/complicaciones , Bronquiectasia/genética , Bronquiectasia/patología , Candidiasis Mucocutánea Crónica/complicaciones , Candidiasis Mucocutánea Crónica/diagnóstico , Candidiasis Mucocutánea Crónica/genética , Candidiasis Mucocutánea Crónica/patología , Preescolar , Femenino , Marcadores Genéticos , Humanos , Secuenciación del ExomaRESUMEN
BACKGROUND: Sarcoidosis is a systemic granulomatous disease that in most patients affects the lung. Pulmonary fibrotic sarcoidosis is clinically, radiologically, and pathologically a heterogeneous condition. Although substantial indirect evidence suggests small airways involvement, direct evidence currently is lacking. RESEARCH QUESTION: What is the role of the (small) airways in fibrotic sarcoidosis? STUDY DESIGN AND METHODS: Airway morphologic features were investigated in seven explant lungs with end-stage fibrotic sarcoidosis using a combination of CT scanning (large airways), micro-CT scanning (small airways), and histologic examination and compared with seven unused donor lungs as controls with specific attention focused on different radiologically defined sarcoidosis subtypes. RESULTS: Patients with central bronchial distortion (n = 3), diffuse bronchiectasis (n = 3), and usual interstitial pneumonia pattern (n = 1) were identified based on CT scan, showing a decrease and narrowing of large airways, a similar airway number and increased airway diameter of more distal airways, or an increase in airway number and airway diameter, respectively, compared with control participants. The number of terminal bronchioles per milliliter and the total number of terminal bronchioles were decreased in all forms of fibrotic sarcoidosis. Interestingly, the number of terminal bronchioles was inversely correlated with the degree of fibrosis. Furthermore, we identified granulomatous remodeling as a cause of small airways loss using serial micro-CT scanning and histologic examination. INTERPRETATION: The large airways are involved differentially in subtypes of sarcoidosis, but the terminal bronchioles universally are lost. This suggests that small airways loss forms an important aspect in the pathophysiologic features of fibrotic pulmonary sarcoidosis.
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Bronquiectasia/patología , Enfermedades Pulmonares Intersticiales/patología , Fibrosis Pulmonar/patología , Sarcoidosis Pulmonar/patología , Anciano , Bélgica , Bronquiectasia/diagnóstico por imagen , Progresión de la Enfermedad , Femenino , Humanos , Enfermedades Pulmonares Intersticiales/diagnóstico por imagen , Trasplante de Pulmón , Masculino , Persona de Mediana Edad , Fibrosis Pulmonar/diagnóstico por imagen , Fibrosis Pulmonar/cirugía , Sarcoidosis Pulmonar/diagnóstico por imagen , Sarcoidosis Pulmonar/cirugía , Tomografía Computarizada por Rayos X , Microtomografía por Rayos XRESUMEN
BACKGROUND: Bronchiectasis is an independent risk factor for cardiovascular diseaseï¼CVDï¼and cardiac dysfunction. Endothelial progenitor cells (EPCs) play a crucial role in maintaining endothelial function, and is inversely correlated with cardiovascular risk factors or cardiac dysfunction. However, the relationship between EPCs and bronchiectasis is unknown. METHODS: Twenty-nine patients with stable bronchiectasis and 15 healthy controls were recruited. Fasting venous blood were collected for determining circulating EPC number and activity as well as systemic inflammatory cytokines. RESULTS: The number and migratory or proliferative activity of circulating EPCs in bronchiectasis patients were significantly reduced (p < 0.001). In high E-FACED group, the number of circulating EPCs evaluated by cell culture assay and EPC proliferation were decreased (p < 0.05). Similarly, the number and function of circulating EPCs were both reduced in low forced expiratory volume in 1 s (FEV1) or high mMRC group (p < 0.05). There was a significant correlation between circulating EPCs and bronchiectasis disease severity, according to the E-FACED score (p < 0.05), particularly to FEV1 (p < 0.05) and mMRC dyspnea score (p < 0.05). The count and activity of EPCs inversely correlated with hsCRP levels and IL-6 levels (p < 0.01). CONCLUSIONS: Deficiencies in the number and function of circulating EPCs are present in patients with bronchiectasis. The changes are related to disease severity and may be partly attributed to systemic inflammation. The current findings may provide novel surrogate evaluation biomarkers and potential therapeutic target for bronchiectasis.
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Bronquiectasia/patología , Células Progenitoras Endoteliales/patología , Anciano , Biomarcadores/sangre , Bronquiectasia/sangre , Bronquiectasia/diagnóstico , Bronquiectasia/fisiopatología , Movimiento Celular , Proliferación Celular , Células Cultivadas , Femenino , Volumen Espiratorio Forzado , Humanos , Inflamación , Interleucina-6/sangre , Masculino , Persona de Mediana Edad , Índice de Severidad de la EnfermedadRESUMEN
Bronchiectasis is a chronic airway disease with abnormal and persistent bronchial dilatation caused by a variety of reasons. In recent years, numerous reports have shown that bronchiectasis is heterogeneous, the clinical characteristics of patients with different phenotypes are different, and the efficacy of a treatment regimen may vary greatly in patients with different bronchiectasis phenotypes. This paper summarizes the current clinical phenotypic classification of bronchiectasis from the perspective of etiology, microbiology, and the frequency of acute exacerbation, and cluster analysis was used to determine new clinical phenotypes and their statistical and clinical significance. Different tools for assessing disease severity yield different outcomes. This article summarizes the research progress in the above areas, hoping to provide a more comprehensive understanding of the disease.
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Bronquios/patología , Bronquiectasia , Bronquiectasia/etiología , Bronquiectasia/patología , Bronquiectasia/terapia , China , Humanos , Enfermedades Pulmonares/microbiología , Lesión Pulmonar/patología , Fenotipo , Enfermedad Pulmonar Obstructiva Crónica/patología , Índice de Severidad de la EnfermedadRESUMEN
Background/aim: Two different scoring systems were developed to determine the severity of bronchiectasis: FACED scoring and the bronchiectasis severity index (BSI). In this study, we aim to compare these 2 scoring systems according to the 6-min walking distance test and a disease-specific health status questionnaire in patients with noncystic fibrosis bronchiectasis (NCFB). Materials and methods: Smoking history, emergency and hospital admissions, and body mass index were obtained from NCFB patients admitted to our hospitals' pulmonary rehabilitation unit between 2013 and 2018. Detailed pulmonary function tests were performed for all participants. Dyspnea perceptions were determined according to the mMRC dyspnea scale. The 6-min walking test was used to determine exercise capacity. The Saint George respiratory questionnaire (SGRQ) was applied to determine health status. Both FACED and BSI scores were calculated for all participants. Results: There were a total of 183 participants, 153 of whom were men. A significant and strong correlation was found between FACED and BSI scores. As the severity of bronchiectasis increased, walking distance was significantly decreased and health status was significantly worse in both FACED and BSI scoring. A statistically significant but weak negative correlation was found between FACED score and walking distance. There was a significant negative correlation between BSI and walking distance, a stronger negative correlation than with FACED. Similarly, there was a significant negative correlation between health status and both FACED and BSI, but this correlation was stronger in the BSI score. Conclusions: Although both FACED and BSI scores were negatively correlated with walking distance and health status in patients with NCFB, BSI was more strongly associated.
Asunto(s)
Bronquiectasia/fisiopatología , Prueba de Esfuerzo/métodos , Tolerancia al Ejercicio/fisiología , Caminata/fisiología , Adulto , Anciano , Bronquiectasia/patología , Femenino , Fibrosis/fisiopatología , Humanos , Masculino , Persona de Mediana Edad , Consumo de OxígenoRESUMEN
An increasing number of patients infected with nontuberculous mycobacteria (NTM) are observed worldwide. However, it is challenging to identify NTM lung diseases from pulmonary tuberculosis (PTB) due to considerable overlap in classic manifestations and clinical and radiographic characteristics. This study quantifies both cavitary and bronchiectasis regions in CT images and explores a machine learning approach for the differentiation of NTM lung diseases and PTB. It involves 116 patients and 103 quantitative features. After the selection of informative features, a linear support vector machine performs disease classification, and simultaneously, discriminative features are recognized. Experimental results indicate that bronchiectasis is relatively more informative, and two features are figured out due to promising prediction performance (area under the curve, 0.84 ± 0.06; accuracy, 0.85 ± 0.06; sensitivity, 0.88 ± 0.07; and specificity, 0.80 ± 0.12). This study provides insight into machine learning-based identification of NTM lung diseases from PTB, and more importantly, it makes early and quick diagnosis of NTM lung diseases possible that can facilitate lung disease management and treatment planning.
Asunto(s)
Interpretación de Imagen Asistida por Computador/métodos , Aprendizaje Automático , Infecciones por Mycobacterium no Tuberculosas/diagnóstico por imagen , Tomografía Computarizada por Rayos X/métodos , Tuberculosis Pulmonar/diagnóstico por imagen , Adulto , Anciano , Bronquiectasia/diagnóstico por imagen , Bronquiectasia/patología , Femenino , Humanos , Pulmón/diagnóstico por imagen , Masculino , Persona de Mediana Edad , Infecciones por Mycobacterium no Tuberculosas/clasificación , Infecciones por Mycobacterium no Tuberculosas/patología , Micobacterias no Tuberculosas , Sensibilidad y Especificidad , Tuberculosis Pulmonar/clasificación , Tuberculosis Pulmonar/patologíaRESUMEN
PURPOSE: We aimed to demonstrate the computed tomography (CT) findings observed at the initial presentation of coronavirus disease 2019 (COVID-19) pneumonia and reveal the most frequent infiltration and distribution patterns of the disease. METHODS: One hundred and eighty-five patients (87 men, 98 women; mean age, 48.7 years), who underwent RT-PCR sampling and high-resolution CT examination in our hospital between March 15, 2020, and April 15, 2020, and got a definitive diagnosis of COVID-19 disease via initial or follow-up RT-PCR test, were included in the study. We comprehensively analyzed the most common and relatively rare CT imaging features (e.g., distribution pattern, density of the lesions, additional CT signs) in patients diagnosed with COVID-19 pneumonia. RESULTS: Thirty-eight patients (20.6%) had no evidence of pneumonia on their initial high-resolution CT images. Among 147 patients (79.4%) who had parenchymal infiltration consistent with pneumonia, 10 (6.8%) had a negative baseline RT-PCR test, and positivity was detected as a result of repeated tests. Most of the patients had multifocal (89.1%) and bilateral (86.4%) lesions. The most common location, right lower lobe, was affected in 87.8% of the patients. Lesions were distributed predominantly at peripheral (87.1%) and posterior (46.3%) areas of lung parenchyma. Most of the patients had pure ground glass opacity (GGO) (82.3%) followed by GGO with consolidation (32.7%) and crazy paving pattern (21.8%). Pure consolidation, solid nodules, halo sign, reverse halo sign, vascular enlargement, subpleural line, air-bronchogram, and bronchiectasis were the other findings observed in at least 15% of the cases. Halo sign, acinar nodules, air-bubble sign, pleural thickening and effusion, mediastinal and/or hilar lymphadenopathy were seen rarely (2%-12.9%). Pericardial effusion, pneumothorax, cavitation, and tree-in-bud pattern were not detected in our study group. CONCLUSION: Multifocal and bilateral GGO infiltration predominantly distributed in peripheral, posterior, and lower lung areas was the most common infiltration pattern.
Asunto(s)
Betacoronavirus/genética , Infecciones por Coronavirus/diagnóstico por imagen , Neumonía Viral/diagnóstico por imagen , Neumonía/diagnóstico por imagen , Tomografía Computarizada por Rayos X/métodos , Adulto , Anciano , Bronquiectasia/diagnóstico por imagen , Bronquiectasia/patología , Bronquiectasia/virología , COVID-19 , Infecciones por Coronavirus/epidemiología , Infecciones por Coronavirus/virología , Progresión de la Enfermedad , Femenino , Humanos , Pulmón/diagnóstico por imagen , Pulmón/patología , Pulmón/virología , Linfadenopatía/diagnóstico por imagen , Linfadenopatía/patología , Linfadenopatía/virología , Masculino , Mediastino/diagnóstico por imagen , Mediastino/patología , Mediastino/virología , Persona de Mediana Edad , Pandemias , Derrame Pericárdico/diagnóstico por imagen , Derrame Pericárdico/patología , Derrame Pericárdico/virología , Neumonía/patología , Neumonía/virología , Neumonía Viral/epidemiología , Neumonía Viral/virología , Neumotórax/diagnóstico por imagen , Neumotórax/patología , Neumotórax/virología , Estudios Retrospectivos , Reacción en Cadena de la Polimerasa de Transcriptasa Inversa/métodos , SARS-CoV-2 , Turquía/epidemiologíaRESUMEN
OBJECTIVE: Bronchiectasis is a common chronic airway disease. We investigated the economic burden and associated factors of bronchiectasis in China. METHODS: In this multicenter retrospective cohort study, we reviewed medical records of patients admitted to 18 tertiary hospitals during 2010 to 2014 with a bronchiectasis-related diagnosis. RESULTS: A total 5469 patients with bronchiectasis were admitted, accounting for 3.13% ± 1.80% of all discharged patients with any diagnosis during the same period; 13 patients died upon discharge. The median hospitalization cost was RMB 8421.52 (RMB 5849.88-12,294.47). Risk factors associated with hospitalization costs included age at admission (>70 vs. <40 years, odds ratio (OR) = 1.221, 95% confidence interval (CI) = 1.082-1.379; >80 vs. <40 years, OR = 1.251, 95% CI = 1.089-1.438), smoking (≤15 packs/year vs. non-smokers, OR = 1.125, 95% CI = 1.006-1.271; >15 packs/year vs. non-smokers, OR = 1.127, 95% CI = 1.062-1.228), length of hospitalization (OR = 1.05, 95% CI = 1.046-1.054), combination antibiotic treatment (OR = 1.089, 95% CI = 1.033-1.148), cough (OR = 0.851, 95% CI = 0.751-0.965), dyspnea (OR = 0.93, 95% CI = 0.878-0.984), chronic obstructive pulmonary disease (OR = 0.935, 95% CI = 0.878-0.996), respiratory failure (OR = 0.923, 95% CI = 0.862-0.989), cor pulmonale (OR = 0.919, 95% CI = 0.859-0.982), and death (OR = 1.816, 95% CI = 1.113-2.838). CONCLUSIONS: Age, smoking status, symptoms, and respiratory comorbidities were associated with hospitalization costs of bronchiectasis.