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2.
Respir Res ; 22(1): 224, 2021 Aug 09.
Artigo em Inglês | MEDLINE | ID: mdl-34372845

RESUMO

BACKGROUND: Sarcoidosis most commonly affects lungs and intrathoracic lymph nodes, but any other organ can be involved. In epidemiological studies, many occupational and environmental exposures have been linked to sarcoidosis but their relationship with the disease phenotype has barely been studied. OBJECTIVE: To investigate how occupational and environmental exposures prior to diagnosis relate to organ involvement in patients with sarcoidosis METHODS: We retrospectively studied patients seen at a sarcoidosis clinic between 2017 and 2020. Patients were included if they had a clinical presentation consistent with sarcoidosis and histologically confirmed epithelioid granulomas or had Löfgren syndrome. In a case-case analysis using multivariable logistic regression we calculated odds ratios (OR) of prespecified exposure categories (based on expert ascertainment) for cases with a given organ involvement versus cases without this organ involvement. RESULTS: We included 238 sarcoidosis patients. Sarcoidosis limited to pulmonary involvement was associated with exposure to inorganic dust prior to diagnosis (OR 2.11; 95% confidence interval [CI] 1.11-4.17). Patients with liver involvement had higher odds of contact with livestock (OR 3.68; 95% CI 0.91-12.7) or having jobs with close human contact (OR 4.33; 95% CI 1.57-11.3) than patients without liver involvement. Similar associations were found for splenic involvement (livestock: OR 4.94, 95% CI 1.46-16.1; close human contact: OR 3.78; 95% CI 1.47-9.46). Cardiac sarcoidosis was associated with exposure to reactive chemicals (OR 5.08; 95% CI 1.28-19.2) or livestock (OR 9.86; 95% CI 1.95-49.0). Active smokers had more ocular sarcoidosis (OR 3.26; 95% CI 1.33-7.79). CONCLUSIONS: Our study indicates that, in sarcoidosis patients, different exposures might be related to different organ involvements-hereby providing support for the hypothesis that sarcoidosis has more than one cause, each of which may promote a different disease phenotype.


Assuntos
Poeira , Exposição Ambiental/efeitos adversos , Pulmão/patologia , Linfonodos/patologia , Exposição Ocupacional/efeitos adversos , Sarcoidose Pulmonar/diagnóstico , Adulto , Animais , Feminino , Humanos , Gado , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Sarcoidose Pulmonar/epidemiologia , Sarcoidose Pulmonar/etiologia , Fumar/efeitos adversos , Fumar/epidemiologia
3.
Chest ; 160(4): 1360-1367, 2021 10.
Artigo em Inglês | MEDLINE | ID: mdl-34102140

RESUMO

Sarcoidosis is an important member of the family of granulomatous lung diseases. Since its recognition in the late 19th century, sarcoidosis has been thought of as a disease of unknown cause. Over the past 20 years, this paradigm has been shifting, more rapidly in the past 10 years. Epidemiologic studies, bolstered by case reports, have provided evidence of causal associations between occupational exposure to specific agents and sarcoidosis. Pathogenesis has been more clearly defined, including the role of gene-exposure interactions. The use of in vitro lymphocyte proliferation testing to detect sensitization to inorganic antigens is being examined in patients with sarcoidosis. These antigens include silica and certain metals. Results of studies to date show differences in immunoreactivity of occupationally exposed sarcoidosis cases compared with control cases, suggesting that lymphocyte proliferation testing may prove useful in diagnosing work-related disease. This review discusses recently published findings regarding associations between occupational exposure to silica and silicates, World Trade Center dust, and metals and risk for sarcoidosis, as well as advances in the development of diagnostic tools. Not all cases of sarcoidosis have an identified cause, but some do. Where the cause is occupational, its recognition is critical to enable effective treatment through removal of the affected worker from exposure and to inform intervention aimed at primary prevention.


Assuntos
Doenças Profissionais/epidemiologia , Exposição Ocupacional/estatística & dados numéricos , Sarcoidose Pulmonar/epidemiologia , Carbonato de Cálcio , Sulfato de Cálcio , Socorristas , Humanos , Testes Imunológicos , Ativação Linfocitária , Metais/efeitos adversos , Cidade de Nova Iorque/epidemiologia , Doenças Profissionais/etiologia , Exposição Ocupacional/efeitos adversos , Sarcoidose/epidemiologia , Sarcoidose/etiologia , Sarcoidose Pulmonar/etiologia , Ataques Terroristas de 11 de Setembro , Silicatos/efeitos adversos , Dióxido de Silício/efeitos adversos
4.
Artigo em Inglês | MEDLINE | ID: mdl-33093768

RESUMO

BACKGROUND: Sarcoidosis is a rare, chronic systemic disease. Earlier data (2006-2010) suggest that the incidence of pulmonary sarcoidosis in Silesian voivodeship increased, however there is no current data on other clinical forms of the disease. OBJECTIVES: The aim of presented study was an analysis of the actual epidemiological situation of sarcoidosis with simultaneous estimation of treatment cost financed from public funds. METHODS: Epidemiological descriptive study concerned registered cases of sarcoidosis diagnosed in adult inhabitants of the Silesian voivodeship in years 2011-2015. Secondary epidemiological data on the main diagnosis and co-morbidity were obtained from the National Health Fund (NFZ) database in Katowice. Territorial and temporal variability of standardized incidence rates were analysed with simultaneous estimation of treatment costs reimbursed from the state budget. RESULTS: Pulmonary sarcoidosis was the most frequently registered clinical form of such disease in the Silesian voivodeship (65% of total cases). The highest number of cases was diagnosed in the age 35-54 years, frequently in men than in women. Significantly decrease of the standardized incidence of sarcoidosis noticed between 2011 and 2015 is related with observed lower number of total cases of pulmonary disease. Observed territorial variability of the sarcoidosis incidence requires future, well-planned studies. The annual average direct cost of sarcoidosis treatment is high and exceed 538 EUR per patient. CONCLUSIONS: It was confirmed that sarcoidosis in the Silesian Voivodeship is a rare disease, however reimbursed direct costs of treatment remains very high. (Sarcoidosis Vasc Diffuse Lung Dis 2020; 37 (1): 43-52).


Assuntos
Custos de Cuidados de Saúde , Sarcoidose Pulmonar , Adulto , Idoso , Assistência Ambulatorial/economia , Comorbidade , Efeitos Psicossociais da Doença , Feminino , Custos Hospitalares , Humanos , Incidência , Reembolso de Seguro de Saúde/economia , Masculino , Pessoa de Meia-Idade , Polônia/epidemiologia , Fatores de Risco , Sarcoidose Pulmonar/diagnóstico , Sarcoidose Pulmonar/economia , Sarcoidose Pulmonar/epidemiologia , Sarcoidose Pulmonar/terapia , Fatores de Tempo , Resultado do Tratamento , Adulto Jovem
5.
Lung ; 198(5): 771-775, 2020 10.
Artigo em Inglês | MEDLINE | ID: mdl-32915271

RESUMO

PURPOSE: To investigate whether sarcoidosis patients infected with SARS-CoV-2 are at risk for adverse disease outcomes. STUDY DESIGN AND METHODS: This retrospective study was conducted in five hospitals within the Mount Sinai Health System during March 1, 2020 to July 29, 2020. All patients diagnosed with COVID-19 were included in the study. We identified sarcoidosis patients who met diagnostic criteria for sarcoidosis according to accepted guidelines. An adverse disease outcome was defined as the presence of intubation and mechanical ventilation or in-hospital mortality. In sarcoidosis patients, we reported (when available) the results of pulmonary function testing measured within 3 years prior to the time of SARS­CoV­2 infection. A multivariable logistic regression model was used to generate an adjusted odds ratio (aOR) to evaluate sarcoidosis as a risk factor for an adverse outcome. The same model was used to analyze sarcoidosis patients with moderate and/or severe impairment in pulmonary function. RESULTS: The study included 7337 patients, 37 of whom (0.5%) had sarcoidosis. The crude rate of developing an adverse outcome was significantly higher in patients with moderately and/or severely impaired pulmonary function (9/14 vs. 3/23, p = 0.003). While the diagnosis of sarcoidosis was not independently associated with risk of an adverse event, (aOR 1.8, 95% CI 0.9-3.6), the diagnosis of sarcoidosis in patients with moderately and/or severely impaired pulmonary function was associated with an adverse outcome (aOR 7.8, 95% CI 2.4-25.8). CONCLUSION: Moderate or severe impairment in pulmonary function is associated with mortality in sarcoidosis patients infected with SARS­CoV­2.


Assuntos
Betacoronavirus/isolamento & purificação , Infecções por Coronavirus , Pandemias , Pneumonia Viral , Testes de Função Respiratória/métodos , Sarcoidose Pulmonar , COVID-19 , Comorbidade , Infecções por Coronavirus/mortalidade , Infecções por Coronavirus/fisiopatologia , Infecções por Coronavirus/terapia , Feminino , Mortalidade Hospitalar , Humanos , Masculino , Pessoa de Meia-Idade , Avaliação de Processos e Resultados em Cuidados de Saúde , Pneumonia Viral/mortalidade , Pneumonia Viral/fisiopatologia , Pneumonia Viral/terapia , Respiração Artificial/estatística & dados numéricos , Estudos Retrospectivos , Fatores de Risco , SARS-CoV-2 , Sarcoidose Pulmonar/diagnóstico , Sarcoidose Pulmonar/epidemiologia , Sarcoidose Pulmonar/fisiopatologia , Estados Unidos/epidemiologia
6.
Lancet Respir Med ; 8(6): 585-596, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32526187

RESUMO

BACKGROUND: Previous attempts to characterise the burden of chronic respiratory diseases have focused only on specific disease conditions, such as chronic obstructive pulmonary disease (COPD) or asthma. In this study, we aimed to characterise the burden of chronic respiratory diseases globally, providing a comprehensive and up-to-date analysis on geographical and time trends from 1990 to 2017. METHODS: Using data from the Global Burden of Diseases, Injuries, and Risk Factors Study (GBD) 2017, we estimated the prevalence, morbidity, and mortality attributable to chronic respiratory diseases through an analysis of deaths, disability-adjusted life-years (DALYs), and years of life lost (YLL) by GBD super-region, from 1990 to 2017, stratified by age and sex. Specific diseases analysed included asthma, COPD, interstitial lung disease and pulmonary sarcoidosis, pneumoconiosis, and other chronic respiratory diseases. We also assessed the contribution of risk factors (smoking, second-hand smoke, ambient particulate matter and ozone pollution, household air pollution from solid fuels, and occupational risks) to chronic respiratory disease-attributable DALYs. FINDINGS: In 2017, 544·9 million people (95% uncertainty interval [UI] 506·9-584·8) worldwide had a chronic respiratory disease, representing an increase of 39·8% compared with 1990. Chronic respiratory disease prevalence showed wide variability across GBD super-regions, with the highest prevalence among both males and females in high-income regions, and the lowest prevalence in sub-Saharan Africa and south Asia. The age-sex-specific prevalence of each chronic respiratory disease in 2017 was also highly variable geographically. Chronic respiratory diseases were the third leading cause of death in 2017 (7·0% [95% UI 6·8-7·2] of all deaths), behind cardiovascular diseases and neoplasms. Deaths due to chronic respiratory diseases numbered 3 914 196 (95% UI 3 790 578-4 044 819) in 2017, an increase of 18·0% since 1990, while total DALYs increased by 13·3%. However, when accounting for ageing and population growth, declines were observed in age-standardised prevalence (14·3% decrease), age-standardised death rates (42·6%), and age-standardised DALY rates (38·2%). In males and females, most chronic respiratory disease-attributable deaths and DALYs were due to COPD. In regional analyses, mortality rates from chronic respiratory diseases were greatest in south Asia and lowest in sub-Saharan Africa, also across both sexes. Notably, although absolute prevalence was lower in south Asia than in most other super-regions, YLLs due to chronic respiratory diseases across the subcontinent were the highest in the world. Death rates due to interstitial lung disease and pulmonary sarcoidosis were greater than those due to pneumoconiosis in all super-regions. Smoking was the leading risk factor for chronic respiratory disease-related disability across all regions for men. Among women, household air pollution from solid fuels was the predominant risk factor for chronic respiratory diseases in south Asia and sub-Saharan Africa, while ambient particulate matter represented the leading risk factor in southeast Asia, east Asia, and Oceania, and in the Middle East and north Africa super-region. INTERPRETATION: Our study shows that chronic respiratory diseases remain a leading cause of death and disability worldwide, with growth in absolute numbers but sharp declines in several age-standardised estimators since 1990. Premature mortality from chronic respiratory diseases seems to be highest in regions with less-resourced health systems on a per-capita basis. FUNDING: Bill & Melinda Gates Foundation.


Assuntos
Efeitos Psicossociais da Doença , Carga Global da Doença/estatística & dados numéricos , Doenças Respiratórias/epidemiologia , Adolescente , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Asma/epidemiologia , Asma/mortalidade , Criança , Pré-Escolar , Doença Crônica , Feminino , Humanos , Lactente , Recém-Nascido , Expectativa de Vida , Doenças Pulmonares Intersticiais/epidemiologia , Doenças Pulmonares Intersticiais/mortalidade , Masculino , Pessoa de Meia-Idade , Pneumoconiose/epidemiologia , Pneumoconiose/mortalidade , Prevalência , Doença Pulmonar Obstrutiva Crônica/epidemiologia , Doença Pulmonar Obstrutiva Crônica/mortalidade , Anos de Vida Ajustados por Qualidade de Vida , Doenças Respiratórias/mortalidade , Fatores de Risco , Sarcoidose Pulmonar/epidemiologia , Sarcoidose Pulmonar/mortalidade , Fatores Sexuais , Adulto Jovem
7.
Chest ; 158(5): 2007-2014, 2020 11.
Artigo em Inglês | MEDLINE | ID: mdl-32534908

RESUMO

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.


Assuntos
Volume Expiratório Forçado/fisiologia , Pulmão/fisiopatologia , Capacidade de Difusão Pulmonar/fisiologia , Sarcoidose Pulmonar/fisiopatologia , Inglaterra/epidemiologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prevalência , Estudos Retrospectivos , Sarcoidose Pulmonar/epidemiologia , Espirometria
8.
BMJ ; 367: l5553, 2019 Oct 22.
Artigo em Inglês | MEDLINE | ID: mdl-31641045

RESUMO

Sarcoidosis is a highly variable granulomatous multisystem syndrome. It affects individuals in the prime years of life; both the frequency and severity of sarcoidosis are greater in economically disadvantaged populations. The diagnosis, assessment, and management of pulmonary sarcoidosis have evolved as new technologies and therapies have been adopted. Transbronchial needle aspiration guided by endobronchial ultrasound has replaced mediastinoscopy in many centers. Advanced imaging modalities, such as fluorodeoxyglucose positron emission tomography scanning, and the widespread availability of magnetic resonance imaging have led to more sensitive assessment of organ involvement and disease activity. Although several new insights about the pathogenesis of sarcoidosis exist, no new therapies have been specifically developed for use in the disease. The current or proposed use of immunosuppressive medications for sarcoidosis has been extrapolated from other disease states; various novel pathways are currently under investigation as therapeutic targets. Coupled with the growing recognition of corticosteroid toxicities for managing sarcoidosis, the use of corticosteroid sparing anti-sarcoidosis medications is likely to increase. Besides treatment of granulomatous inflammation, recognition and management of the non-granulomatous complications of pulmonary sarcoidosis are needed for optimal outcomes in patients with advanced disease.


Assuntos
Glucocorticoides/uso terapêutico , Hipertensão Pulmonar/prevenção & controle , Imunossupressores/uso terapêutico , Fibrose Pulmonar/prevenção & controle , Sarcoidose Pulmonar/diagnóstico , Biomarcadores/sangue , Broncoscopia/métodos , Aspiração por Agulha Fina Guiada por Ultrassom Endoscópico/métodos , Fluordesoxiglucose F18/administração & dosagem , Humanos , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/mortalidade , Incidência , Pulmão/diagnóstico por imagem , Pulmão/patologia , Tomografia por Emissão de Pósitrons/métodos , Prognóstico , Fibrose Pulmonar/etiologia , Fibrose Pulmonar/mortalidade , Sarcoidose Pulmonar/complicações , Sarcoidose Pulmonar/tratamento farmacológico , Sarcoidose Pulmonar/epidemiologia , Resultado do Tratamento
9.
Circ J ; 83(11): 2329-2388, 2019 10 25.
Artigo em Inglês | MEDLINE | ID: mdl-31597819
10.
Ther Adv Respir Dis ; 13: 1753466619868935, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31409257

RESUMO

Pulmonary sarcoidosis is the most common form of sarcoidosis, accounting for the initial presentation in over 70% patients and with eventual presence in 90% of patients with sarcoidosis. However, the course of the disease is often unpredictable; its manifestations can be highly variable and its treatment may not be effective in all patients. As such, the optimized treatment of pulmonary sarcoidosis often requires a thoughtful personalized approach with the need to get the patient involved in decisions of management. In many patients with pulmonary sarcoidosis, the disease is self-limited and nonprogressive, thus treatment is not necessary. In other patients, the presence of significant symptoms or functional limitation often associated with worsening radiological changes and pulmonary function tests warrants treatment. Corticosteroids are the first-line treatment for pulmonary sarcoidosis; antimetabolites are second-line agents, with methotrexate being most commonly employed. Antitumor necrosis alpha antibodies, especially infliximab, are emerging as potential third-line agents. A high index of suspicion should be held for pulmonary hypertension and other comorbidities that may complicate the course of patients with advanced sarcoidosis. Lung transplantation may be the only option for patients who have refractory disease despite maximal medical therapy.


Assuntos
Corticosteroides/uso terapêutico , Imunossupressores/uso terapêutico , Transplante de Pulmão , Pulmão/efeitos dos fármacos , Pulmão/cirurgia , Metotrexato/uso terapêutico , Sarcoidose Pulmonar/terapia , Inibidores do Fator de Necrose Tumoral/uso terapêutico , Corticosteroides/efeitos adversos , Algoritmos , Animais , Comorbidade , Técnicas de Apoio para a Decisão , Humanos , Pulmão/patologia , Pulmão/fisiopatologia , Transplante de Pulmão/efeitos adversos , Metotrexato/efeitos adversos , Fatores de Risco , Sarcoidose Pulmonar/diagnóstico , Sarcoidose Pulmonar/epidemiologia , Sarcoidose Pulmonar/fisiopatologia , Resultado do Tratamento , Inibidores do Fator de Necrose Tumoral/efeitos adversos
11.
Can Respir J ; 2019: 3808206, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31281552

RESUMO

Introduction: Sarcoidosis is a multisystem granulomatous disease of unknown aetiology, commonly involving the lungs.  Cough is a frequent and troublesome symptom of sarcoidosis that reduces patients' quality of life. Aim: Retrospective analysis of different factors-smoking history, Scadding stage, results of lung function testing, calcium metabolism, endobronchial finding, CD4+/CD8+ T-cell ratio in bronchoalveolar lavage fluid (BALF), and other sarcoidosis symptoms in relationship to presence/absence of cough in sarcoidosis patients. Methods: We retrospectively studied sarcoidosis patients diagnosed at the Clinic of Pneumology and Phthisiology of Martin University Hospital between 1998 and 2018. Patients with a history of cough-relevant comorbidities were excluded from the study. GraphPad Prism 7.0 software was used to perform statistical analysis. Results: 101 sarcoidosis patients were included to the study: 65 patients reporting from cough and 36 without cough. The cough was slightly more frequent in nonsmokers (p=0.166) and in women (p=0.688). Cough was associated with dyspnoea (p=0.0007), fever (p=0.0324), and chest pain (p=0.0206) and did not associate with arthralgia (p=0.317) and erythema nodosum (p=0.505). Patients with cough had significantly a lower average value of calciuria (p=0.0014) and lower MEF25 (p=0.0304), MEF50 (p=0.0061), FEV1 (p=0.0025), and FVC (p=0.0025) in % of predicted values, and more often positive endobronchial finding (p=0.0206), compared to patients without cough. Calcemia, FEV1/FVC, DLCO, and CD4+/CD8+ T-cell ratio in BALF and occurrence of cough did not differ between different stages of the disease. Conclusions: We found significant differences between sarcoidosis patients with/without cough regarding symptoms, results of lung function tests, endobronchial finding, and calcium metabolism. Further research is needed to understand the etiopathogenesis of cough in sarcoidosis patients.


Assuntos
Tosse/etiologia , Sarcoidose Pulmonar/complicações , Adulto , Idoso , Cálcio/sangue , Cálcio/urina , Tosse/epidemiologia , Endoscopia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Testes de Função Respiratória , Estudos Retrospectivos , Sarcoidose Pulmonar/epidemiologia , Eslováquia/epidemiologia , Fumar/efeitos adversos
12.
Lung ; 197(4): 427-436, 2019 08.
Artigo em Inglês | MEDLINE | ID: mdl-31190130

RESUMO

BACKGROUND: Sarcoidosis is a systemic disease in which the personal environment seems to drive a differentiated disease frequency and clinical expression. The main epidemiological studies suggest a key influence of potential environmentally linked exposures related to the type of occupation, the household, life style, socioeconomic status, and region of residence. OBJECTIVE: To provide an update on how sarcoidosis may be modulated by environmental factors. DATA SOURCES: We searched PubMed for epidemiological studies. SYNTHESIS: The risk of sarcoidosis is enhanced in people working in jobs related to agriculture, water, construction, metal machining, education, and health, and reduced in those working in jobs mainly centered on personal care. Studies have confirmed seasonal-related peaks of sarcoidosis incidence that follow geographical North-South and West-East gradients. Other personal factors include smoking, personal household exposures, and leisure activities. The evidence pointing to the crucial role of the environment in the etiopathogenesis of sarcoidosis is mounting rapidly. Few diseases so strongly combine geography, environment, gender, and ethnicity as key etiopathogenic factors, with susceptibility to any putative agent being modulated by the individual exposome and genome. CONCLUSION: Geoepidemiological research should focus on evaluating the combined effects of environmental and genetic factors, the identification of clusters of geographically driven exposures, and more precise measurement of all personal exposures (degree of combination, length, and level of exposure).


Assuntos
Meio Ambiente , Exposição Ambiental/efeitos adversos , Estilo de Vida , Sarcoidose Pulmonar/epidemiologia , Determinantes Sociais da Saúde , Características da Família , Humanos , Exposição Ocupacional/efeitos adversos , Prognóstico , Características de Residência , Medição de Risco , Fatores de Risco , Sarcoidose Pulmonar/diagnóstico , Classe Social , Tempo (Meteorologia)
13.
Respir Med ; 152: 1-6, 2019 06.
Artigo em Inglês | MEDLINE | ID: mdl-31128602

RESUMO

OBJECTIVES: To describe the clinical features and outcomes in elderly patients with sarcoidosis and to compare them with younger patients. MATERIAL AND METHODS: Retrospective study of a large cohort of 668 consecutive patients with sarcoidosis prospectively collected during 42 years at the Bellvitge University Hospital, a tertiary care single-centre in Barcelona, Spain. Elderly sarcoidosis was defined as sarcoidosis diagnosed in patients ≥65 years-old. RESULTS: Elderly sarcoidosis was diagnosed in 47 (7%) patients. In younger patients, Löfgren's syndrome was the predominant mode of onset (8.5% vs. 42.2%, p < 0.001). At diagnosis, elderly patients more frequently demonstrated radiographic stage III and IV sarcoidosis (21.3% vs. 7.6%, p = 0.001), isolated extrapulmonary involvement (21.3% vs. 8.2%, p = 0.003), subcutaneous nodules (17% vs. 3.4%, p < 0.001) and intraabdominal/retroperitoneal lymph nodes (23.4% vs. 9.5%, p = 0.003). Furthermore, patients with elderly sarcoidosis achieved remission (spontaneous and under treatment) less frequently during the follow-up period (14 patients, 35% vs. 305 patients, 53%, p = 0.027) and had a higher incidence of pulmonary fibrosis (15% vs. 6.1%, p = 0.029). Death related to sarcoidosis was more prevalent in elderly patients (6.4% vs. 1.3%, p = 0.036). CONCLUSIONS: Sarcoidosis in elderly patients requires a high index of suspicion. Aged pulmonary sarcoidosis patients presented with more severe disease at presentation and worse outcomes compared to younger patients. Isolated extrapulmonary involvement at diagnosis and certain particular extrapulmonary organ involvement were more frequent in elderly sarcoidosis. Remission was less frequent in elderly sarcoidosis.


Assuntos
Linfonodos/patologia , Sarcoidose Pulmonar/patologia , Sarcoidose/epidemiologia , Sarcoidose/patologia , Tela Subcutânea/patologia , Adulto , Idoso , Humanos , Incidência , Pessoa de Meia-Idade , Prevalência , Estudos Prospectivos , Fibrose Pulmonar/epidemiologia , Fibrose Pulmonar/etiologia , Radiografia Torácica/métodos , Remissão Espontânea , Estudos Retrospectivos , Sarcoidose/diagnóstico por imagem , Sarcoidose/tratamento farmacológico , Sarcoidose Pulmonar/epidemiologia , Espanha/epidemiologia , Centros de Atenção Terciária
14.
Paediatr Respir Rev ; 29: 53-59, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30917882

RESUMO

Paediatric sarcoidosis is an extremely rare disease characterized by a granulomatous inflammation. The estimated incidence is 0.6-1.02/100,000 children, but in the absence of international registers, the disease is probably under-reported. Its pathophysiologic basis is not clearly understood but the current hypothesis is a combination of a genetic predisposition and an environmental exposure that could be either organic or mineral. Contrary to adult forms of the disease, general symptoms are often at the forefront at diagnosis. In its most frequent form, paediatric sarcoidosis is a multi-organ disorder affecting preferentially the lungs, the lymphatic system and the liver, but all organs can be affected. This review aims to provide an overview of current knowledge on sarcoidosis in children, providing a summary of the data available from cohort studies on the presentation, the management and the evolution of the disease in this specific population.


Assuntos
Sarcoidose Pulmonar/epidemiologia , Adolescente , Criança , Pré-Escolar , Progressão da Doença , Predisposição Genética para Doença , Humanos , Lactente , Pulmão/diagnóstico por imagem , Pulmão/patologia , Sarcoidose/diagnóstico , Sarcoidose/epidemiologia , Sarcoidose/fisiopatologia , Sarcoidose/terapia , Sarcoidose Pulmonar/diagnóstico , Sarcoidose Pulmonar/fisiopatologia , Sarcoidose Pulmonar/terapia
15.
Sleep Med ; 56: 123-127, 2019 04.
Artigo em Inglês | MEDLINE | ID: mdl-30879993

RESUMO

BACKGROUND AND AIM: Several studies reported a high prevalence of Obstructive Sleep Apnoea (OSA) in patients with Idiopathic Pulmonary Fibrosis (IPF) or restrictive end-stage lung disease (ESLD). Besides the known risk factors for OSA like high Body Mass Index (BMI), reduced static and dynamic volumes for IPF patients and reduced DLCO and low minimal O2 saturation during sleep for ESLD patients were associated with higher Apnoea-Hypopnoea Index (AHI). The aim of our study was to determine potential predictive factors of OSA in patients with Fibrotic Lung Diseases (FLD). MATERIALS AND METHODS: In this study, 49 patients with FLD and BMI ≤30 kg/m2 were included. All patients underwent portable cardiorespiratory polysomnography (PSG) and were asked to fill in Epworth Sleepiness Scale (ESS). Their epidemiological, medication and subsidiary exams data were retrieved from their hospital records. Univariate and multivariate correlation models were obtained. RESULTS: Approximately 70% of patients had an AHI ≥5 events/h. In an univariate correlation model, AHI showed a statistically significant correlation with age, BMI, the duration of immunosuppressant treatment, and Forced Expiratory Volume in the first second (FEV1). Only BMI remained an independent predictor of OSA in a multivariate correlation model adjusted for the other statistically meaningful variables. CONCLUSIONS: FLD patients, in general, show a prevalence of OSA superior to that of the general population. Excess of weight might predict a higher risk for OSA in FLD patients. Larger and more homogenous studies are warranted to clarify the associations between OSA severity and lung function impairment and the duration of immunosuppressant treatment.


Assuntos
Alveolite Alérgica Extrínseca/epidemiologia , Índice de Massa Corporal , Fibrose Pulmonar Idiopática/epidemiologia , Sarcoidose Pulmonar/epidemiologia , Apneia Obstrutiva do Sono/diagnóstico , Apneia Obstrutiva do Sono/epidemiologia , Idoso , Idoso de 80 Anos ou mais , Comorbidade , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Polissonografia , Testes de Função Respiratória , Índice de Gravidade de Doença
16.
Ann Glob Health ; 85(1)2019 01 22.
Artigo em Inglês | MEDLINE | ID: mdl-30741505

RESUMO

More than 100 different conditions are grouped under the term interstitial lung disease (ILD). A diagnosis of an ILD primarily relies on a combination of clinical, radiological, and pathological criteria, which should be evaluated by a multidisciplinary team of specialists. Multiple factors, such as environmental and occupational exposures, infections, drugs, radiation, and genetic predisposition have been implicated in the pathogenesis of these conditions. Asbestosis and other pneumoconiosis, hypersensitivity pneumonitis (HP), chronic beryllium disease, and smoking-related ILD are specifically linked to inhalational exposure of environmental agents. The recent Global Burden of Disease Study reported that ILD rank 40th in relation to global years of life lost in 2013, which represents an increase of 86% compared to 1990. Idiopathic pulmonary fibrosis (IPF) is the prototype of fibrotic ILD. A recent study from the United States reported that the incidence and prevalence of IPF are 14.6 per 100,000 person-years and 58.7 per 100,000 persons, respectively. These data suggests that, in large populated areas such as Brazil, Russia, India, and China (the BRIC region), there may be approximately 2 million people living with IPF. However, studies from South America found much lower rates (0.4-1.2 cases per 100,000 per year). Limited access to high-resolution computed tomography and spirometry or to multidisciplinary teams for accurate diagnosis and optimal treatment are common challenges to the management of ILD in developing countries.


Assuntos
Poluição do Ar/estatística & dados numéricos , Países em Desenvolvimento , Exposição Ambiental/estatística & dados numéricos , Doenças Pulmonares Intersticiais/epidemiologia , Exposição Ocupacional/estatística & dados numéricos , Alveolite Alérgica Extrínseca/diagnóstico , Alveolite Alérgica Extrínseca/epidemiologia , Alveolite Alérgica Extrínseca/fisiopatologia , Alveolite Alérgica Extrínseca/terapia , Asbestos , Doenças do Tecido Conjuntivo/diagnóstico , Doenças do Tecido Conjuntivo/epidemiologia , Doenças do Tecido Conjuntivo/fisiopatologia , Doenças do Tecido Conjuntivo/terapia , Acesso aos Serviços de Saúde , Humanos , Pneumonias Intersticiais Idiopáticas/diagnóstico , Pneumonias Intersticiais Idiopáticas/epidemiologia , Pneumonias Intersticiais Idiopáticas/fisiopatologia , Pneumonias Intersticiais Idiopáticas/terapia , Fibrose Pulmonar Idiopática/diagnóstico , Fibrose Pulmonar Idiopática/epidemiologia , Fibrose Pulmonar Idiopática/fisiopatologia , Fibrose Pulmonar Idiopática/terapia , Incidência , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/fisiopatologia , Doenças Pulmonares Intersticiais/terapia , Pneumoconiose/diagnóstico , Pneumoconiose/epidemiologia , Pneumoconiose/fisiopatologia , Pneumoconiose/terapia , Prevalência , Sarcoidose Pulmonar/diagnóstico , Sarcoidose Pulmonar/epidemiologia , Sarcoidose Pulmonar/fisiopatologia , Sarcoidose Pulmonar/terapia , Espirometria , Tomografia Computadorizada por Raios X
17.
Med Sci (Paris) ; 34 Focus issue F1: 121-133, 2018 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-30403187

RESUMO

Sarcoidosis is a disease involving the growth of abnormal inflammatory granulomas and affecting multisystems. It has an unknown etiology. The lung and the skin are the most commonly involved organs. Although large amounts of research have focused on the pathogenesis of sarcoidosis, little is known about the link between cutaneous sarcoidosis and pulmonary sarcoidosis. Moreover, the gene expression profiles provide a novel way to find diagnostic or prognostic biomarkers. Therefore, the aim of this study was to analyze the differentially expressed genes (DEGs) in pulmonary sarcoidosis and cutaneous sarcoidosis patients and to compare them to healthy individuals. DEGs and their biological functions are dynamically dysregulated, and several common disease-related genes and mutual disease progression-related genes were identified which linked pulmonary sarcoidosis and cutaneous sarcoidosis together. The biological functional pathways regulated by these DEGs may allow to define the common mechanism shared by different type of sarcoidosis, providing novel insight into the common pathogenesis of sarcoidosis and opening the way to the development of new therapeutic strategies.


Assuntos
Sarcoidose Pulmonar/genética , Sarcoidose/genética , Dermatopatias/genética , Transcriptoma , Biomarcadores/análise , Biomarcadores/metabolismo , Estudos de Casos e Controles , Conjuntos de Dados como Assunto , Perfilação da Expressão Gênica , Humanos , Prognóstico , Sarcoidose/diagnóstico , Sarcoidose/epidemiologia , Sarcoidose Pulmonar/diagnóstico , Sarcoidose Pulmonar/epidemiologia , Transdução de Sinais/genética , Dermatopatias/diagnóstico , Dermatopatias/epidemiologia
18.
Wiad Lek ; 71(3 pt 1): 501-507, 2018.
Artigo em Ucraniano | MEDLINE | ID: mdl-29783213

RESUMO

OBJECTIVE: Introduction: Lung sarcoidosis is a systemic granulomatous disease that can affect various organs and systems of a person. Due to the lack of a uniform standardized approach to the diagnosis of sarcoidosis, the epidemiological pattern is heterogeneous and depends on many factors. The aim: To investigate the correlation between the number of patients with sarcoidosis among the population of the Poltava region (Ukraine) and the ecological characteristics of the industrial activity of the region in comparison with the data on the availability of subspecialists in respiratory diseases. PATIENTS AND METHODS: Materials and methods: The study is based on a retrospective analysis of patients with sarcoidosis living in the Poltava region (Ukraine) for the period from 2008 to 2018. RESULTS: Results: The analysis of the correlation between the intensity of environmental impacts on the region and the number of patients with sarcoidosis did not reveal statistically significant changes. An odds ratio (OR) of the occurrence of sarcoidosis among the urban population has not experienced significant dynamics (OR 1,337, 95% CI: 0.96-1.86) compared with those living in rural areas. The number of specialists performing the duties of a pulmonologist in the region is associated with a significantly higher number of registered patients with various forms of sarcoidosis (r=0.27, p=0.04). CONCLUSION: Conclusions: There was no reliable relationship between the risk of sarcoidosis and habitat in areas with increased ecological and industrial load in the Poltava region. The uneven distribution of specialized medical care reduces the patient's odds of establishing a diagnosis of sarcoidosis in the countryside.


Assuntos
Exposição Ambiental , Sarcoidose Pulmonar/epidemiologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Humanos , Pessoa de Meia-Idade , Estudos Retrospectivos , Sarcoidose Pulmonar/induzido quimicamente , Sarcoidose Pulmonar/etiologia , Ucrânia/epidemiologia , Adulto Jovem
19.
Chest ; 154(5): 1052-1060, 2018 11.
Artigo em Inglês | MEDLINE | ID: mdl-29778660

RESUMO

BACKGROUND: The diagnosis of sarcoidosis is made by the combination of clinical features and biopsy results. The clinical features of sarcoidosis can be quite variable. We developed a Sarcoidosis Diagnostic Score (SDS) to summarize the clinical features of patients with possible sarcoidosis. METHODS: Biopsy-confirmed patients with sarcoidosis seen during a 7-month period at the University of Cincinnati sarcoidosis clinic were prospectively identified. Patients with nonsarcoidosis seen at the same clinic were used as control patients. Using a modified World Association of Sarcoidosis and Other Granulomatous Disorders organ assessment instrument, we scored all patients for presence of biopsy, ≥1 highly probable symptom, and ≥1 at least probable symptom for each area. Two sarcoidosis scores were generated: SDS biopsy (with biopsy) and SDS clinical (without biopsy). RESULTS: The 980 evaluable patients were divided into two cohorts: an initial 600 patients (450 with biopsy-confirmed sarcoidosis, 150 control patients) to establish cutoff values for SDS biopsy and an SDS clinical and a validation cohort of 380 patients (103 biopsy-confirmed patients with sarcoidosis and 277 control patients). The best cutoff value for SDS biopsy was ≥ 6 (sensitivity, 99.3%; specificity, 100%). For the total the 980 patients, an SDS clinical ≥ 3 had a sensitivity of 90.6%, specificity of 88.5%, and a likelihood ratio of 7.9. An SDS clinical score ≥ 4 had a lower sensitivity of (76.9%) but higher specificity (98.6%). CONCLUSIONS: For sarcoidosis, the presence of specific clinical features, especially multiorgan involvement, can enhance the diagnostic certainty. The SDS scoring system quantitated the clinical features consistent with sarcoidosis.


Assuntos
Biópsia , Granuloma/patologia , Doenças Pulmonares Intersticiais/diagnóstico , Pneumopatias Obstrutivas/diagnóstico , Pulmão , Sarcoidose Pulmonar/diagnóstico , Adulto , Biópsia/métodos , Biópsia/estatística & dados numéricos , Diagnóstico Diferencial , Feminino , Granuloma/etiologia , Humanos , Pulmão/diagnóstico por imagem , Pulmão/patologia , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Estudos Prospectivos , Reprodutibilidade dos Testes , Projetos de Pesquisa , Sarcoidose Pulmonar/epidemiologia , Avaliação de Sintomas/métodos , Avaliação de Sintomas/estatística & dados numéricos , Estados Unidos/epidemiologia
20.
Sci Rep ; 8(1): 6968, 2018 05 03.
Artigo em Inglês | MEDLINE | ID: mdl-29725035

RESUMO

Sarcoidosis commonly affects the lung. Lung transplantation (LT) is required when there is a severe and refractory involvement. We compared post-transplant survival rates of sarcoidosis patients with chronic obstructive pulmonary disease (COPD) and idiopathic pulmonary fibrosis (IPF). We also explored whether the race and age of the donor, and double lung transplant have any effect on the survival in the post transplant setting. We analyzed 9,727 adult patients with sarcoidosis, COPD, and IPF who underwent LT worldwide between 2005-2015 based on United Network for Organ Sharing (UNOS) database. Survival rates were compared with Kaplan-Meier, and risk factors were investigated by Cox-regression analysis. 469 (5%) were transplanted because of sarcoidosis, 3,688 (38%) for COPD and 5,570 (57%) for IPF. Unadjusted survival analysis showed a better post-transplant survival rate for patients with sarcoidosis (p < 0.001, Log-rank test). In Cox-regression analysis, double lung transplant and white race of the lung donor showed to have a significant survival advantage. Since double lung transplant, those who are younger and have lower Lung Allocation Score (LAS) at the time of transplant have a survival advantage, we suggest double lung transplant as the procedure of choice, especially in younger sarcoidosis subjects and with lower LAS scores.


Assuntos
Seleção do Doador , Transplante de Pulmão , Sarcoidose Pulmonar/terapia , Adulto , Fatores Etários , Idoso , Seleção do Doador/métodos , Feminino , Sobrevivência de Enxerto , Humanos , Fibrose Pulmonar Idiopática/epidemiologia , Fibrose Pulmonar Idiopática/terapia , Transplante de Pulmão/métodos , Masculino , Pessoa de Meia-Idade , Modelos de Riscos Proporcionais , Doença Pulmonar Obstrutiva Crônica/epidemiologia , Doença Pulmonar Obstrutiva Crônica/terapia , Sarcoidose Pulmonar/epidemiologia , Análise de Sobrevida , Taxa de Sobrevida , Resultado do Tratamento
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