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1.
Ned Tijdschr Geneeskd ; 1642020 09 17.
Artigo em Holandês | MEDLINE | ID: mdl-33331709

RESUMO

BACKGROUND: In times of coronavirus, a patient with respiratory symptoms whose chest CT scan reveals ground-glass opacities, COVID-19 may seem an obvious diagnosis. CASE DESCRIPTION: At the (currently assumed) peak of the coronavirus crisis, a 12-year-old boy was admitted to the hospital twice for severe respiratory symptoms. A chest CT scan revealed ground-glass opacities.COVID-19 pneumonia was initially thought of. However, it turned out to be a rare interstitial pulmonary disease. CONCLUSION: Due to the increased awareness about COVID-19, tunnel vision is lurking. Even during a health crisis, doctors should remain alert to alternative diagnoses.


Assuntos
/diagnóstico , Doenças Pulmonares Intersticiais/diagnóstico , Pulmão , /epidemiologia , /psicologia , Criança , Tomada de Decisão Clínica , Diagnóstico Diferencial , Humanos , Julgamento , Pulmão/diagnóstico por imagem , Pulmão/fisiopatologia , Doenças Pulmonares Intersticiais/fisiopatologia , Masculino , Avaliação de Sintomas/métodos , Tomografia Computadorizada por Raios X/métodos
2.
Sarcoidosis Vasc Diffuse Lung Dis ; 37(2): 136-147, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33093777

RESUMO

Background: Interstitial lung disease (ILD) is a common complication of primary Sjögren's syndrome (pSS). Because there is a paucity of literature on the management of pSS-associated ILD (pSS-ILD), this retrospective cohort study assessed the efficacy of azathioprine and mycophenolate therapy in adult patients with pSS-ILD. Methods: A retrospective cohort study was performed using electronic health records to identify adults meeting the 2016 American College of Rheumatology/European League Against Rheumatism classification criteria for pSS. The presence of pSS-ILD was confirmed by characteristic high-resolution computed tomography and/or histopathology findings. Sociodemographic, clinical, and pulmonary function test (PFT) data were abstracted for patients meeting the criteria and followed longitudinally from the date of their ILD diagnosis. PFT values were anchored on time of treatment start, and linear mixed-effects modeling was used to analyze changes in diffusion capacity for carbon monoxide (DLCO) and forced vital capacity (FVC) before and after treatment initiation. Results: We identified 19 subjects who had pSS-ILD, of whom seven were treated with azathioprine and seven were treated with mycophenolate. Within the azathioprine treated group, FVC% slope change trended toward improvement from a rate of -9.8% per month pre-treatment to 2.1% per month post-treatment (p = 0.13). Within the mycophenolate treated group, FVC% slope change improved from a rate of 1.5% per month pre-treatment to 4.3% per month post-treatment (p = 0.02) and DLCO% slope changed from a rate of -3.8% to -1.3% per month (p = 0.01) after therapy start. Conclusions: Mycophenolate treatment was associated with significant improvement in PFTs of pSS-ILD patients over time, and azathioprine treatment followed a similar non-significanttrend. Additional prospective studies are needed to further evaluate these findings. (Sarcoidosis Vasc Diffuse Lung Dis 2020; 37 (2): 136-147).


Assuntos
Azatioprina/uso terapêutico , Imunossupressores/uso terapêutico , Doenças Pulmonares Intersticiais/tratamento farmacológico , Pulmão/efeitos dos fármacos , Ácido Micofenólico/uso terapêutico , Síndrome de Sjogren/tratamento farmacológico , Idoso , Idoso de 80 Anos ou mais , Azatioprina/efeitos adversos , Registros Eletrônicos de Saúde , Feminino , Humanos , Imunossupressores/efeitos adversos , Pulmão/diagnóstico por imagem , Pulmão/imunologia , Pulmão/fisiopatologia , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Doenças Pulmonares Intersticiais/imunologia , Doenças Pulmonares Intersticiais/fisiopatologia , Masculino , Pessoa de Meia-Idade , Ácido Micofenólico/efeitos adversos , Recuperação de Função Fisiológica , Estudos Retrospectivos , Síndrome de Sjogren/diagnóstico , Síndrome de Sjogren/imunologia , Fatores de Tempo , Resultado do Tratamento
3.
Sarcoidosis Vasc Diffuse Lung Dis ; 37(2): 231-233, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33093788

RESUMO

Background: A subgroup of patients with fibrotic ILD experience progression and several risk factors for ILD progression have been reported, such as male sex, older age, lower baseline pulmonary function, and a radiological or pathological pattern of usual interstitial pneumonia. Objective: To describe a possible new phenotype of rapidly non IPF progressive fibrosing with an IPF-like outcome. Methods: Three previously fit and well patients who developed a rapidly progressive ILD and died within 6 to 7 months from the initial development of respiratory symptoms. Results: Unlike what is currently known, our patients developed a severe fibrosing ILD with an IPF-like outcome despite a) being younger than the average patient with IPF, b) having received a non-IPF MDT diagnosis, c) having a non-UIP pattern on HRCT. Moreover and similarly to IPF, they failed to respond to immunosuppressive treatment which is the preferred treatment option in these cases. Conclusion: We believe that patients who present with similar characteristics should be considered as likely to develop a phenotype of rapidly progressive ILD and be treated with antifibrotic medications instead of immunosuppressive ones according to the favourable treatment response to antifibrotic therapy observed in clinical trials of patients with progressive fibrosing ILDs. (Sarcoidosis Vasc Diffuse Lung Dis 2020; 37 (2): 231-233).


Assuntos
Doenças Pulmonares Intersticiais/diagnóstico , Pulmão/diagnóstico por imagem , Fibrose Pulmonar/diagnóstico , Tomografia Computadorizada por Raios X , Adulto , Antibacterianos/uso terapêutico , Progressão da Doença , Evolução Fatal , Volume Expiratório Forçado , Humanos , Imunossupressores/uso terapêutico , Pulmão/efeitos dos fármacos , Pulmão/imunologia , Pulmão/fisiopatologia , Doenças Pulmonares Intersticiais/tratamento farmacológico , Doenças Pulmonares Intersticiais/imunologia , Doenças Pulmonares Intersticiais/fisiopatologia , Masculino , Pessoa de Meia-Idade , Fenótipo , Fibrose Pulmonar/tratamento farmacológico , Fibrose Pulmonar/imunologia , Fibrose Pulmonar/fisiopatologia , Índice de Gravidade de Doença , Fatores de Tempo , Falha de Tratamento , Capacidade Vital
4.
Pediatr Pulmonol ; 55(12): 3579-3586, 2020 12.
Artigo em Inglês | MEDLINE | ID: mdl-32946202

RESUMO

BACKGROUND: We aim to assess the anxiety and depressive symptoms related to the COVID-19 pandemic in children with chronic lung disease and their parents and also to evaluate parents' coping strategies. METHODS: Parents of children aged 4-18 years, with chronic lung disease (study group n = 113) and healthy control (n = 108) were enrolled in the study. General Health Questionnaire-12, specific COVID-19 related anxiety questions, The Coping Orientation to Problems Experienced inventory, coronavirus-related psychiatric symptom scale in children-parental form were used to analyze the psychiatric effects of COVID-19. Parents were also asked about how online education affected their family life and children. All data were compared between children/parents in the study and control groups. Risk factors related with anxiety scores of children were also analyzed. RESULTS: Talking about the pandemic, concern about coronavirus transmission, taking precaution to prevent coronavirus transmission, making pressure to protect from COVID-19 were significantly higher in parents within the study group (p < .05). Parents in the study group used more problem-focused coping than parents in the control group (p = .003). Anxiety symptoms score was higher in children of the study group (p = .007). Parents in the study group found online education more useful than parents in the control group. CONCLUSION: Children with chronic lung diseases and their parents have more anxiety due to COVID-19 pandemic and these parents use more mature coping strategies to manage the stress of the pandemic. Longitudinal and larger studies should be done in all aspects of online education in children with chronic lung diseases.


Assuntos
Ansiedade/psicologia , Transtornos da Motilidade Ciliar/psicologia , Infecções por Coronavirus , Fibrose Cística/psicologia , Doenças Pulmonares Intersticiais/psicologia , Pandemias , Pais/psicologia , Pneumonia Viral , Estresse Psicológico/psicologia , Adaptação Psicológica , Adolescente , Adulto , Betacoronavirus , Estudos de Casos e Controles , Criança , Pré-Escolar , Transtornos da Motilidade Ciliar/fisiopatologia , Fibrose Cística/fisiopatologia , Feminino , Nível de Saúde , Humanos , Doenças Pulmonares Intersticiais/fisiopatologia , Masculino , Pessoa de Meia-Idade , Fatores de Risco
5.
Arch Cardiovasc Dis ; 113(10): 630-641, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32888873

RESUMO

BACKGROUND: Pulmonary hypertension (PH) is a heterogeneous, severe and progressive disease with an impact on quality of life and life-expectancy despite specific therapies. AIMS: (i) to compare prognosis significance of each PH subgroup in a cohort from a referral center, (ii) to identify phenotypically distinct high-risk PH patient using machine learning. METHODS: Patients with PH were included from 2002 to 2019 and routinely followed-up. We collected clinical, laboratory, imaging and hemodynamic variables. Four-year survival rate of each subgroups was then compared. Next, phenotypic domains were imputed with 5 eigenvectors for missing values and filtered if the Pearson correlation coefficient was>0.6. Thereafter, agglomerative hierarchical clustering was used for grouping phenotypic variables and patients: a heat map was generated and participants were separated using Penalized Model-Based Clustering. P<0.05 was considered significant. RESULTS: 328 patients were prospectively included (mean age 63±18 yo, 46% male). PH secondary to left heart disease (PH-LHD) and lung disease (PH-LD) had a significantly increased mortality compared to pulmonary arterial hypertension (PAH) patients: HR=2.43, 95%CI=(1.24-4.73) and 2.95, 95%CI=(1.43-6.07) respectively. 25 phenotypic domains were pinpointed and 3 phenogroups identified. Phenogroup 3 had a significantly increased mortality (log-rank P=0.046) compared to the others and was remarkable for predominant pulmonary disease in older male, accumulating cardiovascular risk factors, and simultaneous three major comorbidities: coronary artery disease, chronic kidney disease and interstitial lung disease. CONCLUSION: PH-LHD and PH-LD has 2-fold and 3-fold increase in mortality, respectively compared with PAH. PH patients with simultaneous kidney-cardiac-pulmonary comorbidities were identified as having high-risk of mortality. Specific targeted therapy in this phenogroup should be prospectively evaluated.


Assuntos
Mineração de Dados/métodos , Cardiopatias/epidemiologia , Nefropatias/epidemiologia , Doenças Pulmonares Intersticiais/epidemiologia , Aprendizado de Máquina , Hipertensão Arterial Pulmonar/epidemiologia , Idoso , Idoso de 80 Anos ou mais , Análise por Conglomerados , Comorbidade , Feminino , França/epidemiologia , Nível de Saúde , Cardiopatias/diagnóstico , Cardiopatias/mortalidade , Cardiopatias/fisiopatologia , Humanos , Nefropatias/diagnóstico , Nefropatias/mortalidade , Nefropatias/fisiopatologia , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/mortalidade , Doenças Pulmonares Intersticiais/fisiopatologia , Masculino , Pessoa de Meia-Idade , Fenótipo , Prognóstico , Estudos Prospectivos , Hipertensão Arterial Pulmonar/diagnóstico , Hipertensão Arterial Pulmonar/mortalidade , Hipertensão Arterial Pulmonar/fisiopatologia , Sistema de Registros , Medição de Risco , Fatores de Risco
6.
BMJ Open Respir Res ; 7(1)2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-32963028

RESUMO

Reviews of COVID-19 CT imaging along with postmortem lung biopsies and autopsies indicate that the majority of patients with COVID-19 pulmonary involvement have secondary organising pneumonia (OP) or its histological variant, acute fibrinous and organising pneumonia, both well-known complications of viral infections. Further, many publications on COVID-19 have debated the puzzling clinical characteristics of 'silent hypoxemia', 'happy hypoxemics' and 'atypical ARDS', all features consistent with OP. The recent announcement that RECOVERY, a randomised controlled trial comparing dexamethasone to placebo in COVID-19, was terminated early due to excess deaths in the control group further suggests patients present with OP given that corticosteroid therapy is the first-line treatment. Although RECOVERY along with other cohort studies report positive effects with corticosteroids on morbidity and mortality of COVID-19, treatment approaches could be made more effective given that secondary OP often requires prolonged duration and/or careful and monitored tapering of corticosteroid dose, with 'pulse' doses needed for the well-described fulminant subtype. Increasing recognition of this diagnosis will thus lead to more appropriate and effective treatment strategies in COVID-19, which may lead to a further reduction of need for ventilatory support and improved survival.


Assuntos
Infecções por Coronavirus/fisiopatologia , Pneumonia em Organização Criptogênica/diagnóstico , Erros de Diagnóstico , Hipóxia/fisiopatologia , Pulmão/diagnóstico por imagem , Pneumonia Viral/fisiopatologia , Betacoronavirus , Infecções por Coronavirus/complicações , Infecções por Coronavirus/tratamento farmacológico , Pneumonia em Organização Criptogênica/tratamento farmacológico , Pneumonia em Organização Criptogênica/etiologia , Pneumonia em Organização Criptogênica/fisiopatologia , Dexametasona/uso terapêutico , Glucocorticoides/uso terapêutico , Humanos , Hipóxia/etiologia , Pulmão/patologia , Pulmão/fisiopatologia , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/tratamento farmacológico , Doenças Pulmonares Intersticiais/etiologia , Doenças Pulmonares Intersticiais/fisiopatologia , Pandemias , Pneumonia Viral/complicações , Pneumonia Viral/tratamento farmacológico , Tomografia Computadorizada por Raios X
7.
Am J Respir Crit Care Med ; 202(10): 1430-1444, 2020 11 15.
Artigo em Inglês | MEDLINE | ID: mdl-32602730

RESUMO

Rationale: Chronic hypersensitivity pneumonitis (CHP) is caused by an immune response to antigen inhalation and is characterized by variable histopathological and clinical features. A subset of subjects with CHP have usual interstitial pneumonia and appear to be clinically similar to subjects with idiopathic pulmonary fibrosis (IPF).Objectives: To determine the common and unique molecular features of CHP and IPF.Methods: Transcriptome analysis of lung samples from CHP (n = 82), IPF (n = 103), and unaffected controls (n = 103) was conducted. Differential gene expression was determined adjusting for sex, race, age, and smoking history and using false discovery rate to control for multiple comparisons.Measurements and Main Results: When compared with controls, we identified 413 upregulated and 317 downregulated genes in CHP and 861 upregulated and 322 downregulated genes in IPF. Concordantly upregulated or downregulated genes in CHP and IPF were related to collagen catabolic processes and epithelial development, whereas genes specific to CHP (differentially expressed in CHP when compared with control and not differentially expressed in IPF) were related to chemokine-mediated signaling and immune responsiveness. Using weighted gene coexpression network analysis, we found that among subjects with CHP, genes involved in adaptive immunity or epithelial cell development were associated with improved or reduced lung function, respectively, and that MUC5B expression was associated with epithelial cell development. MUC5B expression was also associated with lung fibrosis and honeycombing.Conclusions: Gene expression analysis of CHP and IPF identified signatures common to CHP and IPF, as well as genes uniquely expressed in CHP. Select modules of gene expression are characterized by distinct clinical and pathological features of CHP.


Assuntos
Alveolite Alérgica Extrínseca/genética , Alveolite Alérgica Extrínseca/imunologia , Perfilação da Expressão Gênica , Fibrose Pulmonar Idiopática/genética , Fibrose Pulmonar Idiopática/imunologia , Doenças Pulmonares Intersticiais/genética , Doenças Pulmonares Intersticiais/imunologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Alveolite Alérgica Extrínseca/fisiopatologia , Feminino , Expressão Gênica , Humanos , Fibrose Pulmonar Idiopática/fisiopatologia , Doenças Pulmonares Intersticiais/fisiopatologia , Masculino , Pessoa de Meia-Idade
8.
G Ital Cardiol (Rome) ; 21(8): 575-583, 2020 Aug.
Artigo em Italiano | MEDLINE | ID: mdl-32686781

RESUMO

The new coronavirus disease 2019 (COVID-19), which is causing hundreds of thousands of deaths worldwide, is complex and can present with a multi-organ localization. One of its worst complications is an interstitial pneumonia with acute respiratory failure also known as severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), which requires non-invasive or invasive ventilation. A severe coagulopathy with poor prognosis is found in 5-10% of cases. SARS-CoV-2 is manifesting as a multi-dimensional disease and, recently, unique co-existing pathophysiological and clinical aspects are being defined: (i) an increased immune and inflammatory response with the activation of a cytokine storm and consequent coagulopathy, which promote both venous thromboembolic events and in situ thrombosis localized in small arterioles and pulmonary alveolar capillaries; (ii) a high intrapulmonary shunt, which often accounts for the severity of respiratory failure, due to reduced hypoxic pulmonary vasoconstriction with pulmonary neo-angiogenetic phenomena. Furthermore, the high incidence of venous thromboembolism in COVID-19 patients admitted to the intensive care unit and the autoptic findings of in situ micro-thrombosis at the pulmonary vascular level, suggest that in this disease coagulopathy, unlike septic disseminated intravascular coagulation, is driven towards a hyper-thrombogenic state, giving rise to a debate (with ongoing studies) about the preventive use of anticoagulant doses of heparin to reduce mortality. The aim of this position paper from the Italian Association of Hospital Cardiologists (ANMCO) is to highlight the main implications that COVID-19 infection has on the pulmonary circulation from a pathophysiological, clinical and management point of view.


Assuntos
Causas de Morte , Infecções por Coronavirus/epidemiologia , Doenças Pulmonares Intersticiais/mortalidade , Pandemias/estatística & dados numéricos , Pneumonia Viral/epidemiologia , Síndrome Respiratória Aguda Grave/epidemiologia , Tromboembolia Venosa/etiologia , Cardiologia , Doenças Transmissíveis Emergentes/epidemiologia , Infecções por Coronavirus/diagnóstico , Progressão da Doença , Feminino , Humanos , Incidência , Itália/epidemiologia , Doenças Pulmonares Intersticiais/etiologia , Doenças Pulmonares Intersticiais/fisiopatologia , Masculino , Pneumonia Viral/diagnóstico , Circulação Pulmonar/fisiologia , Medição de Risco , Síndrome Respiratória Aguda Grave/diagnóstico , Sociedades Médicas , Análise de Sobrevida , Tromboembolia Venosa/mortalidade , Tromboembolia Venosa/fisiopatologia
9.
Telemed J E Health ; 26(10): 1304-1307, 2020 10.
Artigo em Inglês | MEDLINE | ID: mdl-32654656

RESUMO

Purpose: Coronavirus disease 2019 (COVID-19) caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) and is an acute respiratory illness. Although most infected persons are asymptomatic or have only mild symptoms, some patients progress to devastating disease; such progression is difficult to predict or identify in a timely manner. COVID-19 patients who do not require hospitalization can self-isolate at home. Calls from one disease epicenter identify the need for homebased isolation with telemedicine surveillance to monitor for impending deterioration. Methodology: Although the dominant approach for these asymptomatic/paucisymptomatic patients is to monitor oxygen saturation, we suggest additionally considering the potential merits and utility of home-based imaging. Chest computed tomography is clearly impractical, but ultrasound has shown comparable sensitivity for lung involvement, with major advantages of short and simple procedures, low cost, and excellent repeatability. Thoracic ultrasound may thus allow remotely identifying the development of pneumonitis at an early stage of illness and potentially averting the risk of insidious deterioration to severe pneumonia and critical illness while in home isolation. Conclusions: Lung sonography can be easily performed by motivated nonmedical caregivers when directed and supervised in real time by experts. Remote mentors could thus efficiently monitor, counsel, and triage multiple home-based patients from their "control center." Authors believe that this approach deserves further attention and study to reduce delays and failures in timely hospitalization of home-isolated patients.


Assuntos
Infecções por Coronavirus/diagnóstico por imagem , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Monitorização Fisiológica/métodos , Saúde do Trabalhador , Pneumonia Viral/diagnóstico por imagem , Consulta Remota/métodos , Ultrassonografia Doppler/métodos , Infecções por Coronavirus/epidemiologia , Transmissão de Doença Infecciosa/prevenção & controle , Feminino , Hospitalização/estatística & dados numéricos , Humanos , Controle de Infecções/métodos , Doenças Pulmonares Intersticiais/fisiopatologia , Doenças Pulmonares Intersticiais/virologia , Masculino , Tutoria/métodos , Pandemias , Segurança do Paciente , Pneumonia Viral/epidemiologia , Melhoria de Qualidade , Síndrome Respiratória Aguda Grave/diagnóstico por imagem
10.
Rev Mal Respir ; 37(5): 389-398, 2020 May.
Artigo em Francês | MEDLINE | ID: mdl-32278507

RESUMO

Few studies have examined the effects of air pollution in diffuse interstitial lung disease and they have focused on small numbers of patients. Most data are available in idiopathic pulmonary fibrosis and studies suggest that the level of exposure to pollutants may influence the development of acute exacerbations (ozone and NO2), their incidence (NO2), decline in respiratory function (PM10) and death (PM10 and PM2.5). Several studies show an increase in the incidence of rheumatoid arthritis in people living near busy roads. In systemic scleroderma, hypersensitivity pneumonitis and sarcoidosis although negative effects of pollution have been reported the data are insufficient to be conclusive. Nevertheless, the observed effects of air pollution are consistent with those described for other chronic respiratory diseases. Exposure to pollution induces oxidative stress, chronic inflammation and shortening of telomeres, which are all mechanisms described in fibrogenesis. New epidemiological studies are needed with individual measurements of exposure to outdoor and indoor pollution, as well as fundamental studies to clarify the effect of pollution on fibrogenesis.


Assuntos
Poluição do Ar/efeitos adversos , Doenças Pulmonares Intersticiais/etiologia , Poluentes Atmosféricos/efeitos adversos , Poluentes Atmosféricos/toxicidade , Poluição do Ar/estatística & dados numéricos , Alveolite Alérgica Extrínseca/epidemiologia , Alveolite Alérgica Extrínseca/etiologia , Doenças Cardiovasculares/epidemiologia , Doenças Cardiovasculares/etiologia , Exposição Ambiental/efeitos adversos , Humanos , Fibrose Pulmonar Idiopática/epidemiologia , Fibrose Pulmonar Idiopática/etiologia , Incidência , Doenças Pulmonares Intersticiais/epidemiologia , Doenças Pulmonares Intersticiais/fisiopatologia , Ozônio/efeitos adversos , Material Particulado/efeitos adversos , Fenômenos Fisiológicos Respiratórios/efeitos dos fármacos , Fatores de Risco
11.
Rev Assoc Med Bras (1992) ; 66(1): 48-54, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32130381

RESUMO

INTRODUCTION: Systemic sclerosis (SSC) is an autoimmune disorder that affects several organs of unknown etiology, characterized by vascular damage and fibrosis of the skin and organs. Among the organs involved are the esophagus and the lung. OBJECTIVES: To relate the profile of changes in esophageal electromanometry (EM), the profile of skin involvement, interstitial pneumopathy (ILD), and esophageal symptoms in SSC patients. METHODS: This is an observational, cross-sectional study carried out at the SSC outpatient clinic of the Hospital de Clínicas of the Federal University of Uberlândia. After approval by the Ethics Committee and signed the terms of consent, 50 patients were initially enrolled, from 04/12/2014 to 06/25/2015. They were submitted to the usual investigations according to the clinical picture. The statistical analysis was descriptive in percentage, means, and standard deviation. The Chi-square test was used to evaluate the relationship between EM, high-resolution tomography, and esophageal symptoms. RESULTS: 91.9% of the patients had some manometric alterations. 37.8% had involvement of the esophageal body and lower esophageal sphincter. 37.8% had ILD. 24.3% presented the diffuse form of SSC. No association was found between manometric changes and clinical manifestations (cutaneous, pulmonary, and gastrointestinal symptoms). CONCLUSION: The present study confirms that esophageal motility alterations detected by EM are frequent in SSC patients, but may not be related to cutaneous extension involvement, the presence of ILD, or the gastrointestinal complaints of patients.


Assuntos
Transtornos da Motilidade Esofágica/fisiopatologia , Esôfago/fisiopatologia , Doenças Pulmonares Intersticiais/fisiopatologia , Manometria/métodos , Escleroderma Sistêmico/fisiopatologia , Adulto , Idoso , Estudos Transversais , Ensaio de Imunoadsorção Enzimática , Transtornos da Motilidade Esofágica/complicações , Transtornos da Motilidade Esofágica/diagnóstico por imagem , Esfíncter Esofágico Inferior/patologia , Esfíncter Esofágico Inferior/fisiopatologia , Esôfago/diagnóstico por imagem , Esôfago/patologia , Feminino , Hemaglutinação , Humanos , Doenças Pulmonares Intersticiais/complicações , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Masculino , Pessoa de Meia-Idade , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/diagnóstico por imagem , Tomografia Computadorizada por Raios X/métodos
12.
PLoS Genet ; 16(3): e1008651, 2020 03.
Artigo em Inglês | MEDLINE | ID: mdl-32150563

RESUMO

Neonatal interstitial lung diseases due to abnormal surfactant biogenesis are rare in humans and have never been reported as a spontaneous disorder in animals. We describe here a novel lung disorder in Airedale Terrier (AT) dogs with clinical symptoms and pathology similar to the most severe neonatal forms of human surfactant deficiency. Lethal hypoxic respiratory distress and failure occurred within the first days or weeks of life in the affected puppies. Transmission electron microscopy of the affected lungs revealed maturation arrest in the formation of lamellar bodies (LBs) in the alveolar epithelial type II (AECII) cells. The secretory organelles were small and contained fewer lamellae, often in combination with small vesicles surrounded by an occasionally disrupted common limiting membrane. A combined approach of genome-wide association study and whole exome sequencing identified a recessive variant, c.1159G>A, p.(E387K), in LAMP3, a limiting membrane protein of the cytoplasmic surfactant organelles in AECII cells. The substitution resides in the LAMP domain adjacent to a conserved disulfide bond. In summary, this study describes a novel interstitial lung disease in dogs, identifies a new candidate gene for human surfactant dysfunction and brings important insights into the essential role of LAMP3 in the process of the LB formation.


Assuntos
Doenças Pulmonares Intersticiais/genética , Proteína 3 de Membrana Associada ao Lisossomo/genética , Transportadores de Cassetes de Ligação de ATP/genética , Animais , Cães , Feminino , Estudo de Associação Genômica Ampla , Pulmão/metabolismo , Doenças Pulmonares Intersticiais/fisiopatologia , Proteína 3 de Membrana Associada ao Lisossomo/metabolismo , Glicoproteínas de Membrana Associadas ao Lisossomo/genética , Glicoproteínas de Membrana Associadas ao Lisossomo/metabolismo , Masculino , Microscopia Eletrônica de Transmissão , Mutação de Sentido Incorreto , Organelas/metabolismo , Alvéolos Pulmonares/metabolismo , Surfactantes Pulmonares , Vesículas Secretórias/metabolismo
13.
Arthritis Rheumatol ; 72(7): 1049-1058, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-32134199

RESUMO

Systemic sclerosis (SSc) is an autoimmune rheumatic disease with heterogeneous clinical manifestations and a variable course in which the severity of the pathology dictates the disease prognosis and course. Among autoimmune rheumatic diseases, SSc has the highest mortality rate among all rheumatic diseases, though there are exciting new therapeutic targets that appear to halt the progression of SSc manifestations such as skin or lung fibrosis. In selected patients, high-intensity regimens with autologous stem cell transplantation can favorably modify the course. In what was once thought to be an untreatable disease, targeted therapies have now changed the outlook of SSc to a treatable disorder. Herein, we discuss the targeted therapies modifying the outlook on selected organ involvement and creating opportunities for future treatment. We also present a framework for defining low disease activity in SSc.


Assuntos
Cardiopatias/terapia , Nefropatias/terapia , Doenças Pulmonares Intersticiais/terapia , Hipertensão Arterial Pulmonar/terapia , Doença de Raynaud/terapia , Escleroderma Sistêmico/terapia , Úlcera Cutânea/terapia , Doença Aguda , Anti-Hipertensivos/uso terapêutico , Progressão da Doença , Antagonistas dos Receptores de Endotelina/uso terapêutico , Fibrose , Dedos , Cardiopatias/etiologia , Cardiopatias/fisiopatologia , Transplante de Células-Tronco Hematopoéticas , Humanos , Imunossupressores/uso terapêutico , Nefropatias/etiologia , Nefropatias/fisiopatologia , Doenças Pulmonares Intersticiais/etiologia , Doenças Pulmonares Intersticiais/fisiopatologia , Miocárdio/patologia , Avaliação de Resultados em Cuidados de Saúde , Prostaglandinas I/uso terapêutico , Hipertensão Arterial Pulmonar/etiologia , Hipertensão Arterial Pulmonar/fisiopatologia , Doença de Raynaud/etiologia , Doença de Raynaud/fisiopatologia , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/fisiopatologia , Índice de Gravidade de Doença , Úlcera Cutânea/etiologia , Úlcera Cutânea/fisiopatologia
14.
Intern Med ; 59(3): 401-408, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32009089

RESUMO

We herein report a case of anti-MDA5 antibody-positive, clinically amyopathic dermatomyositis complicated by unilateral interstitial lung disease (ILD) in a 78-year-old man with a history of left lung tumor resection. He was admitted due to a persistent fever and abnormal right pulmonary opacity. A transbronchial lung cryobiopsy revealed pulmonary fibrosis, and combined immunosuppressive therapy was initiated. Findings from multiple evaluations, including dynamic breathing magnetic resonance imaging, supported decreased perfusion, ventilation, and mobility of the left lung as etiological factors of unilateral lung ILD. When patients present with laterality of such findings, clinicians should be aware that atypical imaging findings may be observed.


Assuntos
Corticosteroides/uso terapêutico , Dermatomiosite/complicações , Dermatomiosite/fisiopatologia , Doenças Pulmonares Intersticiais/tratamento farmacológico , Doenças Pulmonares Intersticiais/etiologia , Fibrose Pulmonar/tratamento farmacológico , Fibrose Pulmonar/etiologia , Idoso , Autoanticorpos/sangue , Humanos , Helicase IFIH1 Induzida por Interferon/sangue , Doenças Pulmonares Intersticiais/fisiopatologia , Masculino , Fibrose Pulmonar/fisiopatologia , Resultado do Tratamento
16.
Int J Cardiovasc Imaging ; 36(5): 883-888, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32060775

RESUMO

This study examined the relationship between global longitudinal strain (GLS) and pulmonary function tests (PFT) in patients with systemic sclerosis (SS) and normal ejection fraction (EF) and pulmonary artery pressure (PAP) and healthy controls. Sixty patients in two groups underwent extensive screening, including echocardiography, physical examination, the modified Rodnan Skin Score, and pulmonary function tests. Pulmonary interstitial disease was diagnosed by the pulmonary function test and by CT scan in case of indication. GLS score was computed as the mean peak systolic strain for 17 segments. The mean GLS score was - 18.36 ± 2.1 in the case group and - 20.66 ± 1.6 in the control group (P value < 0.001). GLS scores had a significant inverse relationship with the forced expiratory volume in one second (FEV1) to forced vital capacity (FVC) ratio (P value = 0.049) and both FEV and FVC in patients younger than 35 years old (P = 0.046 and 0.049, respectively). GLS scores had no significant relationship with time elapsed since the onset of skin manifestations, and Raynaud phenomenon, Rodnan score, EF, systolic PAP, or the six-minute walk test results. The patients' six-minute walk test had a significant positive relationship with FVC and right ventricular end diastolic diameter (P value = 0.018 and 0.047, respectively). According to our findings, GLS is significantly lower in patients with SS (with normal EF & PAP) than in healthy individuals. It is also related with certain pulmonary function indices including FEV1/FVC. The reduction in GLS is associated with reduced pulmonary function strength.


Assuntos
Pressão Arterial , Doenças Pulmonares Intersticiais/etiologia , Pulmão/irrigação sanguínea , Contração Miocárdica , Hipertensão Arterial Pulmonar/etiologia , Artéria Pulmonar/fisiopatologia , Escleroderma Sistêmico/complicações , Disfunção Ventricular Esquerda/etiologia , Função Ventricular Esquerda , Adulto , Estudos de Casos e Controles , Tolerância ao Exercício , Feminino , Volume Expiratório Forçado , Humanos , Pulmão/fisiopatologia , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/fisiopatologia , Masculino , Pessoa de Meia-Idade , Prognóstico , Hipertensão Arterial Pulmonar/diagnóstico , Hipertensão Arterial Pulmonar/fisiopatologia , Fatores de Risco , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/fisiopatologia , Disfunção Ventricular Esquerda/diagnóstico por imagem , Disfunção Ventricular Esquerda/fisiopatologia , Função Ventricular Direita , Capacidade Vital
17.
Acta Radiol ; 61(10): 1350-1358, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32028775

RESUMO

BACKGROUND: Phase-contrast magnetic resonance imaging (PC-MRI) can determine pulmonary hemodynamics non-invasively. Pulmonary hypertension causes changes in pulmonary hemodynamics and is a factor for acute exacerbation and death in interstitial lung diseases (ILD). PURPOSE: To determine associations between pulmonary hemodynamics measured by PC-MRI and short-term mortality in patients with ILD. MATERIAL AND METHODS: Pulmonary hemodynamics, measured by PC-MRI in 43 patients with ILD, were reviewed retrospectively. Evaluation parameters included heart rate, right cardiac output, average flow, average velocity, acceleration time, acceleration volume (AV), maximal change in flow rate during ejection (M), M/AV, maximum area, minimum area, and relative area change in the pulmonary artery (PA). All causes of death within one year from the day of the MRI examination were assessed by reviewing medical records. Associations between evaluation parameters and outcome were determined by univariate and multivariate Cox regression analysis. RESULTS: Six patients (13.9%) died by the one-year follow-up. Age (hazard ratio [HR] 1.116, 95% confidence interval [CI] 1.015-1.269), average flow (HR 0.932, 95% CI 0.870-0.984), average velocity (HR 0.778, 95% CI 0.573-0.976), right cardiac output (HR 0.870, 95% CI 0.758-0.967), AV (HR 0.840, 95% CI 0.669-0.985), M/AV (HR 1.008, 95% CI 1.001-1.014), and PA relative area change (HR 0.715, 95% CI 0.459-0.928) predicted death in univariate Cox analysis. Multivariate Cox analysis showed decreased right cardiac output (HR 0.547, 95% CI 0.160-0.912) and decreased PA relative area change (HR 0.538, 95% CI 0.177-0.922) were independently associated with death. CONCLUSION: Reduction in right cardiac output and decreased PA relative area change, detected by PC-MRI, were associated with increased mortality in ILD.


Assuntos
Doenças Pulmonares Intersticiais/diagnóstico por imagem , Doenças Pulmonares Intersticiais/mortalidade , Imagem por Ressonância Magnética/métodos , Idoso , Idoso de 80 Anos ou mais , Feminino , Fibrose , Hemodinâmica , Humanos , Doenças Pulmonares Intersticiais/fisiopatologia , Masculino , Pessoa de Meia-Idade , Circulação Pulmonar , Testes de Função Respiratória , Estudos Retrospectivos , Fatores de Risco
18.
Eur Radiol ; 30(5): 2722-2730, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32040727

RESUMO

OBJECTIVES: To investigate CT imaging features associated with poor clinical outcome after corticosteroid treatment in patients diagnosed with cryptogenic organizing pneumonia (COP) and connective tissue disease-related organizing pneumonia (CTD-OP) and to assess the difference in CT findings and treatment responses between COP and CTD-OP. METHODS: Chest CT images from 166 patients (COP, 131; CTD-OP, 35) with pathologically proven organizing pneumonia were reviewed by two thoracic radiologists. The type, distribution pattern, and extent of parenchymal abnormalities, along with other associated imaging features, were assessed for each patient. Logistic regression analyses were used to identify features associated with poor clinical outcomes such as residual disease (RD) and disease relapse. The differences between COP and CTD-OP were also analyzed. RESULTS: Consolidation involving more than 10% of parenchyma (hazard ratio [HR], 2.27), detectable bronchiectasis (HR, 3.59), and diagnosis of CTD-OP (HR, 4.31) were associated with a higher risk of RD after adjustments for patient age and sex. More than 10% consolidation involvement (HR, 2.54) and diagnosis of CTD-OP (HR, 6.42) were also associated with a higher risk of disease relapse. Compared with COP, CTD-OP demonstrated a greater extent of parenchymal abnormalities, especially consolidation, and was less likely to show a peribronchovascular distribution pattern. CONCLUSION: Bronchiectasis and a greater extent of consolidation were associated with RD, with the latter also being associated with disease relapse. Compared with COP, CTD-OP was associated with worse treatment outcomes and demonstrated a greater extent of parenchymal abnormalities, which were also less likely to show a peribronchovascular pattern. KEY POINTS: • The presence of bronchiectasis and a high parenchymal involvement of consolidation on initial chest CT were associated with a worse response to corticosteroids in patients with organizing pneumonia. • Connective tissue disease-related organizing pneumonia (CTD-OP) was associated with worse treatment outcomes than its idiopathic counterpart cryptogenic organizing pneumonia (COP). • Compared with COP, CTD-OP generally demonstrated a greater extent of parenchymal abnormalities, especially consolidation, and was less likely to show a peribronchovascular distribution pattern.


Assuntos
Bronquiectasia/diagnóstico por imagem , Doenças do Tecido Conjuntivo/complicações , Pneumonia em Organização Criptogênica/diagnóstico por imagem , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Corticosteroides/uso terapêutico , Idoso , Artrite Reumatoide/complicações , Pneumonia em Organização Criptogênica/tratamento farmacológico , Pneumonia em Organização Criptogênica/fisiopatologia , Dermatomiosite/complicações , Feminino , Humanos , Pulmão/diagnóstico por imagem , Pulmão/fisiopatologia , Doenças Pulmonares Intersticiais/tratamento farmacológico , Doenças Pulmonares Intersticiais/etiologia , Doenças Pulmonares Intersticiais/fisiopatologia , Masculino , Pessoa de Meia-Idade , Prognóstico , Modelos de Riscos Proporcionais , Capacidade de Difusão Pulmonar , Recidiva , Escleroderma Sistêmico/complicações , Tomografia Computadorizada por Raios X , Capacidade Pulmonar Total , Resultado do Tratamento , Capacidade Vital
19.
Am J Respir Crit Care Med ; 201(10): 1230-1239, 2020 05 15.
Artigo em Inglês | MEDLINE | ID: mdl-32011901

RESUMO

Rationale: The preclinical natural history of progressive lung fibrosis is poorly understood.Objectives: Our goals were to identify risk factors for interstitial lung abnormalities (ILA) on high-resolution computed tomography (HRCT) scans and to determine progression toward clinical interstitial lung disease (ILD) among subjects in a longitudinal cohort of self-reported unaffected first-degree relatives of patients with familial interstitial pneumonia.Methods: Enrollment evaluation included a health history and exposure questionnaire and HRCT scans, which were categorized by visual assessment as no ILA, early/mild ILA, or extensive ILA. The study endpoint was met when ILA were extensive or when ILD was diagnosed clinically. Among subjects with adequate study time to complete 5-year follow-up HRCT, the proportion with ILD events (endpoint met or radiographic ILA progression) was calculated.Measurements and Main Results: Among 336 subjects, the mean age was 53.1 (SD, 9.9) years. Those with ILA (early/mild [n = 74] or extensive [n = 3]) were older, were more likely to be ever smokers, had shorter peripheral blood mononuclear cell telomeres, and were more likely to carry the MUC5B risk allele. Self-reported occupational or environmental exposures, including aluminum smelting, lead, birds, and mold, were independently associated with ILA. Among 129 subjects with sufficient study time, 25 (19.4%) had an ILD event by 5 years after enrollment; of these, 12 met the study endpoint and another 13 had radiologic progression of ILA. ILD events were more common among those with early/mild ILA at enrollment (63.3% vs. 6.1%; P < 0.0001).Conclusions: Rare and common environmental exposures are independent risk factors for radiologic abnormalities. In 5 years, progression of ILA occurred in most individuals with early ILA detected at enrollment.


Assuntos
Doenças Pulmonares Intersticiais/diagnóstico por imagem , Pulmão/diagnóstico por imagem , Adulto , Idoso , Fumar Cigarros/epidemiologia , Estudos de Coortes , Progressão da Doença , Exposição Ambiental/estatística & dados numéricos , Feminino , Volume Expiratório Forçado , Predisposição Genética para Doença , Genótipo , Humanos , Estudos Longitudinais , Pulmão/fisiopatologia , Doenças Pulmonares Intersticiais/epidemiologia , Doenças Pulmonares Intersticiais/genética , Doenças Pulmonares Intersticiais/fisiopatologia , Masculino , Pessoa de Meia-Idade , Mucina-5B/genética , Capacidade de Difusão Pulmonar , Tomografia Computadorizada por Raios X , Capacidade Pulmonar Total , Capacidade Vital
20.
Am J Respir Crit Care Med ; 201(10): 1240-1248, 2020 05 15.
Artigo em Inglês | MEDLINE | ID: mdl-32011908

RESUMO

Rationale: Although relatives of patients with familial pulmonary fibrosis (FPF) are at an increased risk for interstitial lung disease (ILD), the risk among relatives of sporadic idiopathic pulmonary fibrosis (IPF) is not known.Objectives: To identify the prevalence of interstitial lung abnormalities (ILA) and ILD among relatives of patients with FPF and sporadic IPF.Methods: Undiagnosed first-degree relatives of patients with pulmonary fibrosis (PF) consented to participate in a screening study that included the completion of questionnaires, pulmonary function testing, chest computed tomography, a blood sample collection for immunophenotyping, telomere length assessments, and genetic testing.Measurements and Main Results: Of the 105 relatives in the study, 33 (31%) had ILA, whereas 72 (69%) were either indeterminate or had no ILA. Of the 33 relatives with ILA, 19 (58%) had further evidence for ILD (defined by the combination of imaging findings and pulmonary function testing decrements). There was no evidence in multivariable analyses that the prevalence of either ILA or ILD differed between the 46 relatives with FPF and the 59 relatives with sporadic IPF. Relatives with decrements in either total lung or diffusion capacity had a greater than 9-fold increase in their odds of having ILA (odds ratio, 9.6; 95% confidence interval, 3.1-29.8; P < 0.001).Conclusions: An undiagnosed form of ILD may be present in greater than 1 in 6 older first-degree relatives of patients with PF. First-degree relatives of patients with both familial and sporadic IPF appear to be at similar risk. Our findings suggest that screening for PF in relatives might be warranted.


Assuntos
Família , Fibrose Pulmonar Idiopática , Doenças Pulmonares Intersticiais/epidemiologia , Pulmão/diagnóstico por imagem , Idoso , Feminino , Humanos , Pulmão/fisiopatologia , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Doenças Pulmonares Intersticiais/fisiopatologia , Masculino , Pessoa de Meia-Idade , Prevalência , Testes de Função Respiratória , Tomografia Computadorizada por Raios X
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