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1.
Medicine (Baltimore) ; 100(35): e26777, 2021 Sep 03.
Artigo em Inglês | MEDLINE | ID: mdl-34477117

RESUMO

ABSTRACT: Aim of the study was to determine the characteristics and prognosis, and to identify the risk factors for mortality in patients with primary Sjögren syndrome (pSS) with interstitial lung disease (pSS-ILD).A total of 1422 patients with SS were screened and 178 patients with pSS-ILD were recruited. The medical records and outcomes were retrospectively reviewed. Overall survival and case control study were performed to explore the predictors of death.Among 178 pSS-ILD patients, 87.1% were women. Mean age was 61.59 ±â€Š11.69-year-old. Median disease duration was 72.0 (24.0, 156.0) months. Nonspecific interstitial pneumonia was the predominant high-resolution computed tomography pattern (44.9%). Impairment in diffusion capacity was the most common abnormality of pulmonary function test (75.8%) and the most severe consequence. Type 1 respiratory failure and hypoxia were observed in 15.0% and 30.0% patients, respectively. Mean survival time after confirmation of pSS-ILD diagnosis was 9.0 (6.8, 13.0) years. The 10-year survival rate for all patients with pSS-ILD was 81.7%. Forty-four (24.7%) of 178 patients died during the follow-up period. The most predominant cause of death was respiratory failure (n = 27). Twenty-seven patients died of ILD and formed study group. The 78 patients who survived formed control group. Age and smoking were risk factors for mortality in patients with pSS-ILD. In addition, severity of ILD, as reflected by high-resolution computed tomography, pulmonary function test, and arterial blood gas, was an independent risk factor. However, inflammation status (erythrocyte sedimentation rate, C-reactive protein) and anti-Sjögren syndrome-related antigen A and anti-Sjögren syndrome-related antigen B were not.ILD is a severe complication of pSS. Age, smoking, and severity of lung involvement are more critical for prognosis rather than inflammation status and autoantibodies.


Assuntos
Doenças Pulmonares Intersticiais/classificação , Síndrome de Sjogren/mortalidade , Idoso , China/epidemiologia , Feminino , Humanos , Modelos Logísticos , Doenças Pulmonares Intersticiais/fisiopatologia , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Síndrome de Sjogren/classificação , Síndrome de Sjogren/epidemiologia , Estatísticas não Paramétricas
3.
Intern Med ; 60(18): 2887-2897, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34526442

RESUMO

Objective To identify factors associated with pneumomediastinum during management of connective tissue disease (CTD)-related interstitial lung disease (ILD). Methods Patients diagnosed with pneumomediastinum after the initiation of corticosteroid therapy for their CTD-ILD were enrolled. The baseline characteristics of patients who developed pneumomediastinum after the initiation of corticosteroid therapy (n=13, all occurring within 120 days) were compared to those of patients who did not develop pneumomediastinum (n=49). A multivariate logistic regression analysis was performed to identify factors associated with pneumomediastinum. A receiver operating characteristic (ROC) curve analysis was also performed to assess the predictive performance. Results The body mass index (BMI) [odds ratio (OR) (95% confidence interval (CI)) 0.482 (0.272-0.853)] and serum lactate dehydrogenase (LDH) [OR (95% CI) 1.013 (1-1.025)] levels at baseline were identified as independent factors associated with pneumomediastinum after corticosteroid initiation. The optimal cut-off points of the BMI and LDH levels for predicting pneumomediastinum development, as estimated by the Youden index, were 20.2 kg/m2 and 378 U/L, respectively. LDH showed a sensitivity of 61.5% and the highest specificity of 87.8%. Importantly, combining these markers resulted in the highest sensitivity of 100% and a specificity of 71.4%. Conclusion A low BMI and high serum LDH levels at baseline are useful predictive factors for pneumomediastinum development in CTD-ILD patients.


Assuntos
Doenças do Tecido Conjuntivo , Doenças Pulmonares Intersticiais , Enfisema Mediastínico , Biomarcadores , Doenças do Tecido Conjuntivo/complicações , Humanos , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/epidemiologia , Enfisema Mediastínico/diagnóstico por imagem , Enfisema Mediastínico/etiologia , Prognóstico , Estudos Retrospectivos
5.
Arthritis Res Ther ; 23(1): 212, 2021 08 14.
Artigo em Inglês | MEDLINE | ID: mdl-34391465

RESUMO

Screening and follow-up of interstitial lung disease associated with rheumatoid arthritis (RA-ILD) is a challenge in clinical practice. In fact, the majority of RA-ILD patients are asymptomatic and optimal tools for early screening and regular follow-up are lacking. Furthermore, some patients may remain oligosymptomatic despite significant radiological abnormalities. In RA-ILD, usual interstitial pneumonia (UIP) is the most frequent radiological and pathological pattern, associated with a poor prognosis and a high risk to develop acute exacerbations and infections. If RA-ILD can be identified early, there may be an opportunity for an early treatment and close follow-up that might delay ILD progression and improve the long-term outcome.In connective tissue disease-associated interstitial lung disease (CTD-ILD), lung ultrasound (LUS) with the assessment of B-lines and serum Krebs von den Lungen-6 antigen (KL-6) has been recognized as sensitive biomarkers for the early detection of ILD. B-line number and serum KL-6 level were found to correlate with high-resolution computed tomography (HRCT), pulmonary function tests (PFTs), and other clinical parameters in systemic sclerosis-associated ILD (SSc-ILD). Recently, the significant correlation between B-lines and KL-6, two non-ionizing and non-invasive biomarkers, was demonstrated. Hence, the combined use of LUS and KL-6 to screen and follow up ILD in RA patients might be useful in clinical practice in addition to existing tools. Herein, we review relevant literature to support this concept, propose a preliminary screening algorithm, and present 2 cases where the algorithm was used.


Assuntos
Artrite Reumatoide , Doenças Pulmonares Intersticiais , Algoritmos , Artrite Reumatoide/complicações , Artrite Reumatoide/diagnóstico por imagem , Seguimentos , Humanos , Pulmão/diagnóstico por imagem , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Mucina-1
6.
Medicina (Kaunas) ; 57(7)2021 Jul 06.
Artigo em Inglês | MEDLINE | ID: mdl-34356972

RESUMO

Myositis-related interstitial lung disease presents with a wide variety of lesions, ranging from chronic to acute. It can be divided into two main forms by the types of onsets, namely, chronic to subacute type showing nonspecific interstitial pneumonia (NSIP) or NSIP with an organizing pneumonia (OP)/fibrosing OP (FOP) pattern and acute type showing acute lung injury (ALI) to diffuse alveolar damage (DAD) pattern. Anti-aminoacyl tRNA Synthetase antibody-positive cases mainly show an NSIP or FOP pattern, whereas anti-melanoma differentiation-associated gene 5 antibody-positive cases show ALI to DAD pattern. Bilateral consolidation with or without ground-glass opacification with lower lobe predominance is common as a major pattern in all types, but the distribution or extent is sometimes different. The early detection of findings that indicate a rapid progressive course is vital. Diffuse cranio-caudal distribution and multiple ground-glass opacifications with random distribution might indicate a poorer prognosis.


Assuntos
Aminoacil-tRNA Sintetases , Doenças Pulmonares Intersticiais , Miosite , Humanos , Pulmão/diagnóstico por imagem , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Doenças Pulmonares Intersticiais/etiologia , Miosite/complicações , Miosite/diagnóstico por imagem , Estudos Retrospectivos , Tomografia Computadorizada por Raios X
7.
R I Med J (2013) ; 104(7): 26-29, 2021 Sep 01.
Artigo em Inglês | MEDLINE | ID: mdl-34437662

RESUMO

Idiopathic pulmonary fibrosis (IPF) is the most common of the idiopathic interstitial pneumonias. Its signs and symptoms are relatively non-specific, and patients often present with chronic cough, progressive dyspnea, resting or exertional hypoxemia, and inspiratory crackles on lung auscultation. Definitive diagnosis requires the exclusion of known causes of pulmonary fibrosis and identification of the usual interstitial pneumonia (UIP) pattern of disease either on high-resolution computed tomography (HRCT) scan of the chest or on surgical lung biopsy. Multidisciplinary discussion involving pulmonologists, radiologists, and pathologists with expertise in the diagnosis of IPF and other forms of interstitial lung disease is recommended and often required. Management focuses on anti-fibrotic therapy and early referral to lung transplant centers for those who are candidates. This review will discuss the current recommendations for the diagnosis, prognostication, and management of patients with IPF.


Assuntos
Fibrose Pulmonar Idiopática , Doenças Pulmonares Intersticiais , Biópsia , Diagnóstico Diferencial , Humanos , Fibrose Pulmonar Idiopática/diagnóstico por imagem , Fibrose Pulmonar Idiopática/terapia , Pulmão/diagnóstico por imagem , Doenças Pulmonares Intersticiais/diagnóstico , Tomografia Computadorizada por Raios X
8.
Am J Case Rep ; 22: e932924, 2021 Aug 06.
Artigo em Inglês | MEDLINE | ID: mdl-34358221

RESUMO

BACKGROUND The efficacy and safety of re-challenge with immune checkpoint inhibitors after immune-related adverse events have not been established. We report a case of successful re-administration of nivolumab in metastatic renal cell carcinoma after discontinuation due to immune-related adverse events. CASE REPORT Laparoscopic nephrectomy was performed on a 52-year-old man diagnosed with renal cell carcinoma pT1bN0M0. After surgery, left adrenal and lung metastases appeared. Nivolumab was administered as a sixth-line therapy, and he achieved a partial response, but interstitial pneumonia occurred. He was diagnosed with grade 2 immune-related adverse events, and nivolumab treatment was discontinued. Interstitial pneumonia was well controlled by steroids. He maintained a partial response for a long time, and the lung metastases disappeared 7 months after discontinuation. However, bilateral lung metastases reappeared 10 months after the discontinuation. We decided to re-administer nivolumab, while carefully monitoring the patient and fully explaining the risk of recurrence of immune-related adverse events. After 5 cycles of re-administration, computed tomography revealed a reduction in metastases without re-activation of interstitial pneumonia. He experienced a grade 1 fever the day after re-administration, but continued nivolumab therapy without other adverse events. After 7 cycles of re-administration, the lung metastases increased, and nivolumab treatment was terminated. Two months later, a grade 2 interstitial pneumonia recurred, but improved rapidly with oral steroids. CONCLUSIONS For patients who have discontinued immune checkpoint inhibitors due to immune-related adverse events, re-challenge of immune checkpoint inhibitors may be an option after explaining the risk of relapse of immune-related adverse events.


Assuntos
Carcinoma de Células Renais , Neoplasias Renais , Doenças Pulmonares Intersticiais , Carcinoma de Células Renais/tratamento farmacológico , Humanos , Neoplasias Renais/tratamento farmacológico , Doenças Pulmonares Intersticiais/induzido quimicamente , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia , Nivolumabe/efeitos adversos
9.
J Assoc Physicians India ; 69(7): 11-12, 2021 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-34431265

RESUMO

INTRODUCTION: As India recovers from the two waves of the Covid-19 pandemic, its sequelae are posing a new challenge to the physician. These may vary from fatigue and myalgia to persistent, and even worsening breathlessness, due to pulmonary fibrosis. Management of post-COVID-19 pulmonary fibrosis is currently limited to symptomatic management and largely an unexplored aspect. OBJECTIVES: To draw attention to the imminent threat of post-COVID-19 interstitial lung disease (PC-ILD) in COVID survivors through a case series. METHODS: A retrospective analysis of data was done in patients admitted with severe COVID in December 2020 at our tertiary care hospital, and who had a prolonged stay with symptoms and signs suggestive of pulmonary fibrosis. HRCT was done to make a diagnosis of pulmonary fibrosis or ILD. Three such patients were identified. RESULTS: All the three cases were laboratory proven SARS CoV-2 positive cases and had developed pulmonary fibrosis, with traction bronchiectasis, termed here as PC-ILD (Post Covid-Interstitial Lung Disease). Two of them survived and had improved oxygen saturation on room air at three-month follow-up, while one patient had developed arrhythmia and died. CONCLUSION: PC- ILD is one of the emerging complications of COVID-19 pneumonia. A proactive follow-up programme should be undertaken to identify and manage this looming epidemic.


Assuntos
COVID-19 , Doenças Pulmonares Intersticiais , Humanos , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/epidemiologia , Doenças Pulmonares Intersticiais/etiologia , Pandemias , Estudos Retrospectivos , SARS-CoV-2 , Tomografia Computadorizada por Raios X
10.
Eur J Radiol ; 142: 109866, 2021 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-34365304

RESUMO

PURPOSE: The pulmonary function test (PFT) has played an essential role in diagnosing and managing interstitial lung disease (ILD) but has its contraindications and difficult conditions to perform. Therefore, the present study aimed to evaluate dynamic chest radiography (DCR) ability to predict forced vital capacity (FVC) and other PFT parameters of ILD patients. METHOD: The prospective observational study included 97 patients who underwent DCR at Tenri Hospital (Tenri, Japan) between June 2019 and April 2020. Twenty-five patients with stable disease status underwent DCR twice to evaluate test-retest reliability using the intraclass correlation coefficient. From the lung field areas measured by DCR, lung volumes at maximum inspiration (V.ins) and expiration (V.exp) were estimated. Correlation coefficients between the measured values of DCR and PFT parameters were calculated. Multilinear models for predicting FVC and other PFT parameters were developed. RESULTS: Intraclass correlation coefficients between first and second measurements of V.ins and V.exp were 0.94 (95% CI: 0.89-0.97, p < 0.001) and 0.88 (95% CI: 0.78-0.94, p < 0.001), respectively. The correlation coefficient between V.ins and FVC was 0.86 (95% CI: 0.79-0.90, p < 0.001). A multilinear model for predicting FVC was developed using V.ins, V.exp, age, sex, and body mass index as predictor variables, wherein the adjusted coefficient of determination was 0.814. CONCLUSIONS: Lung volumes measured by DCR correlated with the lung function of ILD patients. Prediction models with high predictive power and internal validity were developed, suggesting that DCR can predict FVC and other PFT parameters of ILD patients.


Assuntos
Doenças Pulmonares Intersticiais , Humanos , Pulmão/diagnóstico por imagem , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Radiografia , Reprodutibilidade dos Testes , Testes de Função Respiratória , Capacidade Vital
11.
Pan Afr Med J ; 39: 30, 2021.
Artigo em Francês | MEDLINE | ID: mdl-34394821

RESUMO

Diffuse infiltrate lung diseases (DILDs) are frequent in patients with connective tissue diseases. They can be suggestive of connective tissue diseases or occur during follow-up. Antisynthetase syndrome (ASS) is a complex and heterogeneous connective tissue disease. Antisynthetase antibodies, in particular the anti-Jo1 antibody, are found in patients with this syndrome. The prognosis of ASS is conditioned by the occurrence of DILD and its severity, thus guiding therapeutic management. We here report the case of a 57-year-old female patient presenting with acute febrile DILD revealing ASS. Outcome was favorable under bolus corticosteroids in combination with cyclophosphamide treatment.


Assuntos
Anticorpos Antinucleares/imunologia , Doenças Pulmonares Intersticiais/diagnóstico , Miosite/diagnóstico , Corticosteroides/administração & dosagem , Autoanticorpos/imunologia , Ciclofosfamida/administração & dosagem , Feminino , Humanos , Imunossupressores/administração & dosagem , Doenças Pulmonares Intersticiais/tratamento farmacológico , Doenças Pulmonares Intersticiais/etiologia , Pessoa de Meia-Idade , Miosite/complicações , Miosite/imunologia
12.
Int J Mol Sci ; 22(16)2021 Aug 19.
Artigo em Inglês | MEDLINE | ID: mdl-34445658

RESUMO

Interstitial lung diseases (ILDs) that are known as diffuse parenchymal lung diseases (DPLDs) lead to the damage of alveolar epithelium and lung parenchyma, culminating in inflammation and widespread fibrosis. ILDs that account for more than 200 different pathologies can be divided into two groups: ILDs that have a known cause and those where the cause is unknown, classified as idiopathic interstitial pneumonia (IIP). IIPs include idiopathic pulmonary fibrosis (IPF), non-specific interstitial pneumonia (NSIP), cryptogenic organizing pneumonia (COP) known also as bronchiolitis obliterans organizing pneumonia (BOOP), acute interstitial pneumonia (AIP), desquamative interstitial pneumonia (DIP), respiratory bronchiolitis-associated interstitial lung disease (RB-ILD), and lymphocytic interstitial pneumonia (LIP). In this review, our aim is to describe the pathogenic mechanisms that lead to the onset and progression of the different IIPs, starting from IPF as the most studied, in order to find both the common and standalone molecular and cellular key players among them. Finally, a deeper molecular and cellular characterization of different interstitial lung diseases without a known cause would contribute to giving a more accurate diagnosis to the patients, which would translate to a more effective treatment decision.


Assuntos
Biomarcadores/metabolismo , Regulação da Expressão Gênica , Fibrose Pulmonar Idiopática/patologia , Doenças Pulmonares Intersticiais/patologia , Animais , Humanos , Fibrose Pulmonar Idiopática/genética , Fibrose Pulmonar Idiopática/metabolismo , Doenças Pulmonares Intersticiais/genética , Doenças Pulmonares Intersticiais/metabolismo
13.
Clin Immunol ; 230: 108827, 2021 09.
Artigo em Inglês | MEDLINE | ID: mdl-34428741

RESUMO

BAL cellularity and lymphocyte immunophenotyping offer insights into lung inflammatory status. Natural killer (NK) cells are efficient effector cells, producing pro-inflammatory cytokines. A better understanding of the biology of NK cells in BAL in the lungs is necessary to improve the pathogenesis of fibrotic ILD and develop prospective targeted treatments. Our aim was to analyse NK and NKT-like cell percentages in BAL from 159 patients with different ILD: f-HP, f-NSIP, IPF and CTD-ILD, to evaluate their potential diagnostic/prognostic role. BAL NK cell percentages showed significantly higher values in IPF than in f-HP and f-NSIP, while BAL NKT-like cells showed significantly lower values in the f-NSIP than the f-HP and IPF. A cut-off of 4%NK cells in BAL of IPF showed a significant difference in survival rate. It suggests a possible new marker of survival and raises the possibility of new targeted approach in treatment and management of IPF.


Assuntos
Células Matadoras Naturais/imunologia , Doenças Pulmonares Intersticiais/imunologia , Idoso , Idoso de 80 Anos ou mais , Alveolite Alérgica Extrínseca/imunologia , Alveolite Alérgica Extrínseca/patologia , Alveolite Alérgica Extrínseca/fisiopatologia , Líquido da Lavagem Broncoalveolar/citologia , Líquido da Lavagem Broncoalveolar/imunologia , Doenças do Tecido Conjuntivo/imunologia , Doenças do Tecido Conjuntivo/patologia , Doenças do Tecido Conjuntivo/fisiopatologia , Feminino , Fibrose , Humanos , Fibrose Pulmonar Idiopática/imunologia , Fibrose Pulmonar Idiopática/patologia , Fibrose Pulmonar Idiopática/fisiopatologia , Imunofenotipagem , Estimativa de Kaplan-Meier , Células Matadoras Naturais/classificação , Células Matadoras Naturais/patologia , Pulmão/imunologia , Pulmão/patologia , Pulmão/fisiopatologia , Doenças Pulmonares Intersticiais/patologia , Doenças Pulmonares Intersticiais/fisiopatologia , Masculino , Pessoa de Meia-Idade , Células T Matadoras Naturais/classificação , Células T Matadoras Naturais/imunologia , Células T Matadoras Naturais/patologia , Prognóstico , Testes de Função Respiratória
14.
Clin Immunol ; 230: 108813, 2021 09.
Artigo em Inglês | MEDLINE | ID: mdl-34333094

RESUMO

Cigarette smoking has been implicated in the pathogenesis of seropositive rheumatoid arthritis (RA), as well as RA-associated lung disease. Fibrotic interstitial lung disease as well as emphysema occur in RA and cause substantial morbidity. We used arthritis-susceptible HLA-DQ8 transgenic mice to generate RA-associated lung disease. Mice were exposed to cigarette smoke (CS) prior to induction of arthritis, and subsequently injected with a low dose of bleomycin intra-tracheally to induce lung injury. Exposure of arthritic mice to both CS and bleomycin led to a significant reduction in lung compliance consistent with development of diffuse lung disease. Morphologic evaluation of the lung demonstrated areas of emphysematous change and co-existent fibrosis, consistent with a combined pattern of fibrosis and emphysema. These changes were accompanied by inflammatory cell infiltration and upregulation of fibrosis-associated genes. This humanized mouse model can serve as a valuable research tool to understand the pathogenesis of RA associated lung disease.


Assuntos
Artrite Reumatoide/complicações , Doenças Pulmonares Intersticiais/etiologia , Animais , Artrite Reumatoide/etiologia , Bleomicina/toxicidade , Fumar Cigarros/efeitos adversos , Colágeno/metabolismo , Modelos Animais de Doenças , Feminino , Expressão Gênica , Humanos , Pulmão/metabolismo , Pulmão/patologia , Complacência Pulmonar/efeitos dos fármacos , Doenças Pulmonares Intersticiais/patologia , Masculino , Camundongos , Camundongos Transgênicos , Enfisema Pulmonar/etiologia , Fibrose Pulmonar/etiologia
16.
Chron Respir Dis ; 18: 14799731211035822, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34382888

RESUMO

In people with advanced respiratory disease, we examined (i) the impact of COVID-19-related physical and social isolation on physical activity and (ii) relationships between time spent in isolation and disability in activities of daily living. Cross-sectional analysis was conducted in adults with advanced non-small cell lung cancer, chronic obstructive lung disease or interstitial lung disease. Measures included change in physical activity since physically and socially isolating (Likert scale) and disability (Barthel Index and Lawton-Brody IADL scale) or difficulty (World Health Organisation Disability Assessment Schedule-2.0) in daily activities. Multiple logistic regression was used to examine factors associated with disability in daily activities. 194/201 participants were isolating for a median [IQR] 5 [3-8]-month period, often leading to lower levels of physical activity at home (n = 94, 47%), and outside home (n = 129, 65%). 104 (52%) and 142 (71%) were not fully independent in basic and instrumental activities of daily living, respectively. 96% reported some degree of difficulty in undertaking daily activities. Prolonged physical and social isolation related to increased disability in basic (r = -0.28, p < 0.001) and instrumental (r = -0.24, p < 0.001) activities of daily living, and greater difficulty in daily activities (r = 0.22, p = 0.002). Each month spent in physical or social isolation was independently related to disability in basic activities of daily living (odds ratio [OR], 1.17 [95% CI: 1.03-1.33], p = 0.013). These findings suggest disability in daily activities is associated with prolonged physical or social isolation, which may present as difficulty in people who are fully independent. Post-isolation recovery and rehabilitation needs should be considered for all people deemed extremely clinically vulnerable.


Assuntos
Atividades Cotidianas , COVID-19/prevenção & controle , Carcinoma Pulmonar de Células não Pequenas/fisiopatologia , Exercício Físico , Doenças Pulmonares Intersticiais/fisiopatologia , Neoplasias Pulmonares/fisiopatologia , Doença Pulmonar Obstrutiva Crônica/fisiopatologia , Idoso , Controle de Doenças Transmissíveis , Feminino , Humanos , Modelos Logísticos , Masculino , Pessoa de Meia-Idade , Razão de Chances , Distanciamento Físico , SARS-CoV-2 , Isolamento Social
17.
Arthritis Res Ther ; 23(1): 205, 2021 08 03.
Artigo em Inglês | MEDLINE | ID: mdl-34344444

RESUMO

BACKGROUND: Circulating fibrocytes are an important source of fibroblasts and myofibroblasts, which are involved in fibrotic processes, including systemic sclerosis (SSc). The study aimed to investigate the effect of nintedanib (a tyrosine kinase inhibitor) in inhibiting the in vitro transition of circulating SSc fibrocytes into myofibroblasts and their pro-fibrotic activity. METHODS: Circulating fibrocytes were obtained from 18 SSc patients and 5 healthy subjects (HSs). Cultured SSc fibrocytes were maintained in growth medium (untreated cells) or treated with nintedanib 0.1 and 1 µM for 3 and 24 h. Fibroblast-specific protein-1 (S100A4) and α-smooth muscle actin (αSMA), as markers of fibroblast/myofibroblast phenotype, together with type I collagen (COL1) and fibronectin (FN), were investigated by qRT-PCR and Western blotting. Non-parametric tests were used for statistical analysis. RESULTS: Significantly elevated gene and protein expressions of αSMA, S100A4, COL1, and FN were observed in SSc fibrocytes compared to HS fibrocytes (gene: αSMA p < 0.001; others p < 0.0001; protein: all p < 0.05). Interestingly, an increased gene and protein expression of αSMA and S100A4 was found in fibrocytes from SSc patients positive for anti-Scl70 and with interstitial lung disease (ILD) (Scl70+ILD+) compared to Scl70-ILD- patients (S100A4: gene: p < 0.01; protein: p < 0.05), whereas no differences were observed for COL1 and FN. Nintedanib reduced gene and protein expression of αSMA, S100A4, COL1, and FN in SSc fibrocytes compared to untreated ones with different statistical significance. Noteworthy, nintedanib significantly downregulated gene and protein expression of αSMA, S100A4, COL1, and FN in Scl70+ILD+ fibrocytes (all p < 0.05), whereas only that of S100A4 and FN was significantly downregulated (p < 0.05) in Scl70-ILD- fibrocytes compared to the related untreated cells. CONCLUSIONS: Nintedanib seems to downregulate in vitro the transition of fibrocytes into myofibroblasts and their pro-fibrotic activity, particularly in cells isolated from Scl70+ILD+ SSc patients.


Assuntos
Doenças Pulmonares Intersticiais , Escleroderma Sistêmico , Células Cultivadas , Fibroblastos , Humanos , Indóis/farmacologia , Miofibroblastos , Escleroderma Sistêmico/tratamento farmacológico
18.
Lancet Respir Med ; 9(9): 1065-1076, 2021 09.
Artigo em Inglês | MEDLINE | ID: mdl-34331867

RESUMO

Many patients with interstitial lung disease (ILD) develop pulmonary fibrosis, which can lead to reduced quality of life and early mortality. Patients with fibrotic ILD often have considerable diagnostic delay, and are exposed to unnecessary and costly diagnostic procedures, and ineffective and potentially harmful treatments. Non-specific and insidious presenting symptoms, along with scarce knowledge of fibrotic ILD among primary care physicians and non-ILD experts, are some of the main causes of diagnostic delay. Here, we outline and discuss the challenges facing both patients and physicians in making an early diagnosis of fibrotic ILD, and explore strategies to facilitate early identification of patients with fibrotic ILD, both in the general population and among individuals at highest risk of developing the disease. Finally, we discuss controversies and key uncertainties in screening programmes for fibrotic ILD. Timely identification and accurate diagnosis of patients with fibrotic ILD poses several substantial clinical challenges, but could potentially improve outcomes through early initiation of appropriate management.


Assuntos
Doenças Pulmonares Intersticiais/complicações , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Fibrose Pulmonar/complicações , Fibrose Pulmonar/diagnóstico por imagem , Tomografia Computadorizada por Raios X/métodos , Diagnóstico Precoce , Humanos , Pulmão/diagnóstico por imagem
20.
Am J Case Rep ; 22: e932660, 2021 Jul 31.
Artigo em Inglês | MEDLINE | ID: mdl-34330886

RESUMO

BACKGROUND There is growing concern about the clinical course of certain diseases in patients who are simultaneously infected by SARS-CoV-2. This report is of a 34-year-old woman from Brazil with a recent diagnosis of pulmonary lymphangioleiomyomatosis (LAM) diagnosed by raised serum VEGF-D levels and the finding of lung cysts on computed tomography (CT) imaging, who presented with COVID-19 pneumonia. CASE REPORT Five months after the diagnosis of pulmonary LAM, which was based on the presence of diffuse and bilateral cystic lesions on CT scan associated with high serum VEGF-D levels, the patient presented with worsening dyspnea, drop in peripheral oxygen oxygenation, fever, and diffuse myalgia. She was using Sirolimus because it inhibits the development of LAM cells. A worsening of lung abnormalities was demonstrated in a chest CT examination, with the appearance of areas of consolidation and ground-glass abnormalities. A nasal swab sample tested positive for SARS-CoV-2 infection using reverse-transcription polymerase chain reaction. Thus, Sirolimus was suspended because of concern about its immunosuppressive action. She received hospital support following the institutional protocol in force at the time, without the need for invasive mechanical ventilation. After 2 weeks, she was discharged from the hospital, with supplemental oxygen at home and return of Sirolimus. CONCLUSIONS This report has described the presentation of COVID-19 pneumonia due to SARS-CoV-2 infection in a 34-year-old woman with a recent diagnosis of LAM involving the lungs.


Assuntos
COVID-19 , Cistos , Doenças Pulmonares Intersticiais , Linfangioleiomiomatose , Adulto , Brasil , Feminino , Humanos , Pulmão/diagnóstico por imagem , Linfangioleiomiomatose/diagnóstico por imagem , SARS-CoV-2 , Tomografia Computadorizada por Raios X , Fator D de Crescimento do Endotélio Vascular
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