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1.
Mod Rheumatol Case Rep ; 5(1): 101-107, 2021 01.
Artigo em Inglês | MEDLINE | ID: mdl-33019894

RESUMO

Anti-melanoma differentiation-associated gene 5 juvenile dermatomyositis (anti-MDA5 JDM) is associated with high risk of developing rapidly progressive interstitial lung disease (RP-ILD). Here we report an 11-year-old girl with anti-MDA5 JDM and RP-ILD which led to a fatal outcome, further aggravated by SARS-CoV-2 infection. She was referred to our hospital after being diagnosed with anti-MDA5 JDM and respiratory failure due to RP-ILD. On admission, fibrobronchoscopy with bronchoalveolar lavage (BAL) revealed Pneumocystis jirovecii infection so treatment with intravenous trimethoprim-sulfamethoxazole was initiated. Due to RP-ILD worsening, immunosuppressive therapy was intensified using methylprednisolone pulses, cyclophosphamide, tofacitinib and intravenous immunoglobulin without response. She developed severe hypoxemic respiratory failure, pneumomediastinum and pneumothorax, further complicated with severe RP-ILD and cervical subcutaneous emphysema. Three real-time RT-PCR for SARS-CoV-2 were made with a negative result. In addition, she was complicated with a secondary hemophagocytic lymphohistiocytosis and a fourth real-time PCR for SARS-CoV-2 performed in BAS sample was positive. Despite aggressive treatment of RP-ILD due to anti-MDA5 JDM, there was no improvement of respiratory failure in the following days and patient developed refractory septic shock and died. Anti-MDA5 JDM patients with RP-ILD have a poor prognosis with a high mortality rate. For this reason, intensive immunosuppressive therapy is essential including the use of promising drugs such as tofacitinib. COVID-19 in children with underlying health conditions like anti-MDA5 JDM may still be at risk for disease and severe complications.


Assuntos
/complicações , Dermatomiosite/complicações , Imunossupressores/uso terapêutico , Doenças Pulmonares Intersticiais/complicações , Linfo-Histiocitose Hemofagocítica/etiologia , Pneumonia por Pneumocystis/complicações , Monofosfato de Adenosina/análogos & derivados , Monofosfato de Adenosina/uso terapêutico , Alanina/análogos & derivados , Alanina/uso terapêutico , Antibacterianos/uso terapêutico , Anticorpos Monoclonais Humanizados/uso terapêutico , Antivirais/uso terapêutico , Autoanticorpos/imunologia , Broncoscopia , Criança , Ciclofosfamida/uso terapêutico , Dermatomiosite/tratamento farmacológico , Dermatomiosite/imunologia , Progressão da Doença , Evolução Fatal , Feminino , Humanos , Hidroxicloroquina/uso terapêutico , Hospedeiro Imunocomprometido , Imunoglobulinas Intravenosas/uso terapêutico , Fatores Imunológicos/uso terapêutico , Helicase IFIH1 Induzida por Interferon/imunologia , Pulmão/diagnóstico por imagem , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Doenças Pulmonares Intersticiais/imunologia , Doenças Pulmonares Intersticiais/terapia , Linfo-Histiocitose Hemofagocítica/imunologia , Enfisema Mediastínico/etiologia , Metilprednisolona/uso terapêutico , Piperidinas/uso terapêutico , Pneumonia por Pneumocystis/imunologia , Pneumotórax/etiologia , Pirimidinas/uso terapêutico , Insuficiência Respiratória/etiologia , Insuficiência Respiratória/terapia , Choque Séptico/etiologia , Enfisema Subcutâneo/etiologia , Tomografia Computadorizada por Raios X , Combinação Trimetoprima e Sulfametoxazol/uso terapêutico
2.
Kyobu Geka ; 73(10): 840-844, 2020 Sep.
Artigo em Japonês | MEDLINE | ID: mdl-33130776

RESUMO

Interstitial lung diseases (ILDs) are associated with an increased risk of lung cancer, and pulmonary resection is well known to be associated with high postoperative morbidity and mortality in lung cancer patients. Postoperative mortality rate of acute exacerbation( AE) was reported 33.3~100%. Sex, CRP, KL-6, %vital capacity( VC), forced expiratory volume in 1 second( FEV1.0), history of AE, preoperative steroid use, and surgical procedures were identified as possible risk factors of AE in the univariate analyses by the data obtained from patients with non-small cell lung cancer who had undergone pulmonary resection and presented with a clinical diagnosis of ILD between January 2000 and December 2009 at 64 institutions throughout Japan. Multivariate analysis using these factors identified surgical procedures except for wedge resection, history of AE, KL-6, %VC, and male sex as independent risk factors. A score by risk prediction for AE was 5 X (history of AE)+4 X (CT:UIP pattern)+3 X (gender:male)+3 X (preoperative steroid use)+2 X (KL-6>1,000 U/ml)+1 X (VC≤80%). The predicted probability of risk score 15~22 is>0.25, and risk score 11~14 is 0.1~0.25. We can use a simple risk scoring system comprising seven parameters to identify high risk patients for AE, and provide essential information to support fair and objective clinical decision-making by thoracic surgeons.


Assuntos
Carcinoma Pulmonar de Células não Pequenas , Doenças Pulmonares Intersticiais , Neoplasias Pulmonares , Progressão da Doença , Humanos , Japão , Doenças Pulmonares Intersticiais/complicações , Neoplasias Pulmonares/complicações , Neoplasias Pulmonares/cirurgia , Masculino , Estudos Retrospectivos , Fatores de Risco , Tomografia Computadorizada por Raios X
3.
Rev Mal Respir ; 37(10): 829-832, 2020 Dec.
Artigo em Francês | MEDLINE | ID: mdl-33069501

RESUMO

INTRODUCTION: Methotrexate-induced pneumonitis is a rare but potentially fatal side effect. It is a diagnosis of exclusion. There are early and late forms and different cell patterns in the bronchoalveolar lavage (BAL). CASE REPORT: We present a case of acute interstitial lung disease in a 54-year-old patient who had been taking methotrexate for a year and a half for rheumatoid arthritis. After excluding other causes and based on the diagnostic criteria of Searles and McKendry, we could reasonably identify methotrexate as the cause of the lung disease. It was of late onset and the BAL showed neutrophilia and eosinophilia. CONCLUSION: Methotrexate-induced pneumonitis is a diagnosis of exclusion. A late onset combined with the predominance of neutrophils and eosinophils in BAL is rare in the literature, demonstrating the wide heterogeneity of methotrexate-related interstitial lung disease.


Assuntos
Eosinofilia/induzido quimicamente , Leucocitose/induzido quimicamente , Doenças Pulmonares Intersticiais/induzido quimicamente , Metotrexato/efeitos adversos , Doença Aguda , Líquido da Lavagem Broncoalveolar , Eosinofilia/diagnóstico , Eosinofilia/patologia , Feminino , Humanos , Leucocitose/complicações , Leucocitose/diagnóstico , Doenças Pulmonares Intersticiais/complicações , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/patologia , Pessoa de Meia-Idade , Neutrófilos/patologia , Febre Reumática/tratamento farmacológico , Febre Reumática/patologia , Tomografia Computadorizada por Raios X
4.
Medicine (Baltimore) ; 99(41): e22453, 2020 Oct 09.
Artigo em Inglês | MEDLINE | ID: mdl-33031276

RESUMO

BACKGROUND: The objective of this meta-analysis was to summarize and identify the available evidence from studies to estimate the clinical value of traditional Chinese medicine (TCM) in the treatment of rheumatoid arthritis with interstitial lung disease (RA-ILD). And provides clinicians with evidence on which to base their clinical decision making. METHODS: This review will include all studies comparing clinical efficacy of TCM in the treatment of RA-ILD. The search strategy will be performed in 9 databases. We will not establish any limitations to language and publication status, published from inception to the August 2020. Two reviewers will screen, select studies, extract data, and assess quality independently. Outcome is lung function, number of swelling joints, number of painful joints, duration of morning stiffness, VAS score, adverse effects, quality of life, ESR, CRP, rheumatoid factor and safety. The methodological quality including the risk of bias of the included studies will be evaluated. We will carry out statistical analysis using RevMan 5.3 software. RESULTS: This study will summarize current evidence to assess the efficacy and safety of TCM in the treatment of RA-ILD. CONCLUSION: The findings of this study will provide helpful evidence for the clinician, and will promote further studies, as well as studying the value of TCM. REGISTRATION NUMBER: INPLASY202080108 (DOI number: 10.37766/inplasy2020.8.0108).


Assuntos
Artrite Reumatoide/terapia , Doenças Pulmonares Intersticiais/terapia , Medicina Tradicional Chinesa , Artrite Reumatoide/complicações , Humanos , Doenças Pulmonares Intersticiais/complicações , Metanálise como Assunto , Projetos de Pesquisa , Revisões Sistemáticas como Assunto
5.
Yonsei Med J ; 61(11): 958-964, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-33107239

RESUMO

PURPOSE: Recent history of malignancy without 5-year disease-free interval is an absolute contraindication for lung transplantation (LTx). However, in rare cases, lung cancer may be incidentally diagnosed in the explanted lung of recipients. We evaluated the prevalence, 5-year survival, and prognosis of incidental lung cancer after LTx. MATERIALS AND METHODS: Medical records of patients who underwent LTx at Severance Hospital between January 1, 2012 and June 30, 2019 were reviewed. Patients with incidental lung cancer were included, and those with histologically proven pre-transplant lung cancer were excluded. RESULTS: Of the 247 patients who underwent LTx, 6 (2.4%) were diagnosed with incidental lung cancer. Interstitial lung disease (ILD) was the underlying lung disease in all patients. The median interval from the last preoperative computed tomography (CT) screening to LTx was 26 days. The most common histological type of incidental lung cancer was adenocarcinoma (n=4, 66.7%). All Stage IV cases were misdiagnosed as fibrosis on preoperative chest CT. Patients with incidental lung cancer showed lower 5-year survival than those without malignancy (median survival: 8.5 months vs. not reached, p=0.047, respectively). Patients with Stage III or IV demonstrated lower 5-year survival than those with Stage I or II and those without malignancy (median survival: 5 months, 19 months, and not reached, respectively, p=0.011). CONCLUSION: Multidisciplinary preoperative screening and serial imaging studies within short intervals are required to differentiate lung malignancy from fibrotic foci. Furthermore, active pathologic examination of suspicious lung lesions is required in patients at high risk for lung cancer.


Assuntos
Neoplasias Pulmonares/patologia , Neoplasias Pulmonares/terapia , Transplante de Pulmão/efeitos adversos , Pulmão/diagnóstico por imagem , Adenocarcinoma/mortalidade , Adenocarcinoma/patologia , Adulto , Feminino , Seguimentos , Humanos , Incidência , Achados Incidentais , Pulmão/patologia , Doenças Pulmonares Intersticiais/complicações , Doenças Pulmonares Intersticiais/cirurgia , Neoplasias Pulmonares/mortalidade , Masculino , Pessoa de Meia-Idade , Pneumonectomia , Prevalência , Prognóstico , Estudos Retrospectivos , Doadores de Tecidos , Tomografia Computadorizada por Raios X , Transplantados , Resultado do Tratamento
6.
Medicine (Baltimore) ; 99(43): e22443, 2020 Oct 23.
Artigo em Inglês | MEDLINE | ID: mdl-33120739

RESUMO

The objectives of this study were to describe the prevalence and characteristics of radiographic lesions of the hands, and calcifications of the spine on computer tomography scans (CT-scans), and to investigate the relationships between radiographic and CT-scan abnormalities and clinical features in a population of patients with systemic sclerosis (SSc).Subjects underwent X-ray examination of the hands, and thoracic or thoraco-abdominal and pelvic CT scan or lumbar CT scan in the year. Structural lesions on hand X ray was scored and spinal calcifications were evaluated in the anterior, intracanal and posterior segments. Intra and inter-reliability was tested for radiography and CT- scan. Prognostic factors considered were interstitial pulmonary lesions on the CT scan, pulmonary arterial hypertension (PAH) and death.This study involved 77 SSc patients, 58 (75%) with limited cutaneous SSc (lcSSc) and 19 (25%) with diffuse SSc (dSSc). The prevalences of radiographic lesions of the hand were 28.6% for periarticular calcifications and 26% for calcinosis. On CT scan, 64 (83%) patients exhibited at least 1 calcification. Spine calcifications were depicted in 80.5%, 27.3%, and 35.1% at the anterior, intracanal and posterior segments respectively. Calcifications were mainly localized on thoracic spine. Inter reader reliabilities were good for hands and moderate for spine respectively. Spine calcifications and periarticular calcifications in the hands were associated (P = .012). Calcinosis in the hands was related to PAH (P = .02). Posterior calcification segment and foraminal calcifications were associated with interstitial lung disease (ILD) (P = .029) and death (P = .001).More than 80% of systemic sclerosis patients presented spine calcifications. A significant association between hands and spinal calcifications were confirmed and some localization in the posterior segment considered as a bad prognostic factor.


Assuntos
Calcinose/diagnóstico por imagem , Ossos da Mão/diagnóstico por imagem , Escleroderma Sistêmico/complicações , Doenças da Coluna Vertebral/diagnóstico por imagem , Coluna Vertebral/diagnóstico por imagem , Fatores Etários , Feminino , Humanos , Hipertensão Pulmonar/complicações , Doenças Pulmonares Intersticiais/complicações , Masculino , Pessoa de Meia-Idade , Prognóstico , Radiografia , Reprodutibilidade dos Testes , Estudos Retrospectivos , Escleroderma Sistêmico/mortalidade , Tomografia Computadorizada por Raios X
7.
Health Qual Life Outcomes ; 18(1): 334, 2020 Oct 08.
Artigo em Inglês | MEDLINE | ID: mdl-33032602

RESUMO

BACKGROUND: Health-related quality of life (HRQL) in interstitial lung disease (ILD) patients is impaired. We aimed to identify baseline predictors for HRQL decline within a 12-month observation period. METHODS: We analyzed 194 ILD patients from two German ILD-centers in the observational HILDA study. We employed the disease-specific King's Brief Interstitial Lung Disease questionnaire (K-BILD) with the subdomains 'psychological impact', 'chest symptoms' and 'breathlessness and activities', and the generic EQ-5D Visual Analog Scale (VAS). We evaluated how many patients experienced a clinically meaningful decline in HRQL. Subsequently, we investigated medical and sociodemographic factors as potential predictors of HRQL deterioration. RESULTS: Within the study population (34.0% male, Ø age 61.7) mean HRQL scores hardly changed between baseline and follow up (K-BILD: 52.8 vs. 52.5 | VAS: 60.0 vs. 57.3). On the intra-individual level, 30.4% (n = 59) experienced a clinically relevant deterioration in K-BILD total score and 35.4% (n = 68) in VAS. Lower baseline forced vital capacity (FVC) % predicted determined HRQL decline in K-BILD total score (ß-coefficient: - 0.02, p = 0.007), VAS (ß-coefficient: - 0.03, p < 0.0001), and in the subdomain 'psychological impact' (ß-coefficient: - 0.02, p = 0.014). Lower baseline diffusing capacity of carbon monoxide (DLCO) % predicted determined deterioration in 'breathlessness and activities' (ß-coefficient: - 0.04, p = 0.003) and 'chest symptoms' (ß-coefficient: - 0.04, p = 0.002). Additionally, increasing age predicted decline in 'psychological impact' (ß-coefficient: 0.06, p < 0.007). CONCLUSION: Around a third of ILD patients experienced a clinically relevant HRQL deterioration in a 12-month period, which was associated with baseline lung function values in all K-BILD domains. As lung function values are time-dependent variables with possible improvements, in contrast to age and ILD subtype, it, thus, seems important to improve lung function and prevent its decline in order to maintain HRQL on the possibly highest level.


Assuntos
Progressão da Doença , Doenças Pulmonares Intersticiais/psicologia , Qualidade de Vida , Idoso , Dispneia/etiologia , Feminino , Humanos , Doenças Pulmonares Intersticiais/complicações , Doenças Pulmonares Intersticiais/fisiopatologia , Masculino , Pessoa de Meia-Idade , Inquéritos e Questionários , Escala Visual Analógica
8.
Acta Biomed ; 91(3): ahead of print, 2020 08 10.
Artigo em Inglês | MEDLINE | ID: mdl-32921749

RESUMO

Platypnea-orthodeoxia syndrome (POS) is a clinical entity characterized by positional dyspnoea (platypnea) and arterial desaturation (orthodeoxia) that occurs when sitting or standing up and usually resolves by lying down. POS may result from some cardiopulmonary disorders or from other miscellaneous aetiologies. We report a case of POS in a patient after fibrotic evolution of SARS-CoV-2 interstitial pneumonia associated with pulmonary embolism. The patient did not have any evidence of an intracardiac/intrapulmonary shunt.


Assuntos
Betacoronavirus , Infecções por Coronavirus/complicações , Dispneia/etiologia , Doenças Pulmonares Intersticiais/complicações , Pulmão/diagnóstico por imagem , Pneumonia Viral/complicações , Idoso , Infecções por Coronavirus/diagnóstico , Dispneia/diagnóstico , Feminino , Humanos , Doenças Pulmonares Intersticiais/diagnóstico , Pandemias , Pneumonia Viral/diagnóstico , Tomografia Computadorizada por Raios X
9.
Am J Case Rep ; 21: e926781, 2020 Sep 21.
Artigo em Inglês | MEDLINE | ID: mdl-32952147

RESUMO

BACKGROUND Coronavirus disease 2019 (COVID-19) is caused by a novel coronavirus, SARS-CoV-2, and is associated with severe respiratory disease. There are extensive publications on the chest computed tomography (CT) findings of COVID-19 pneumonia, with ground-glass opacities (GGO) and mixed GGO and consolidation being the most common findings. Those with interstitial thickening manifesting as reticular opacities typically show superimposed ground-glass opacities, giving a crazy-paving pattern. CASE REPORT We report the case of a 77-year-old man with a background of asthma-chronic obstructive pulmonary disease (COPD) overlap syndrome (ACOS) who presented with progressive cough and shortness of breath for 2 days. He was in close contact with a confirmed COVID-19 case. Reverse-transcription polymerase chain reaction analysis of a nasopharyngeal swab was positive for SARS-CoV-2. The initial chest radiograph was negative for lung consolidation and ground-glass opacities. During admission, he had worsening shortness of breath with desaturation, prompting a chest CT examination, which was performed on day 14 of illness. The chest CT revealed an atypical finding of predominant focal subpleural interstitial thickening in the right lower lobe. He was provided supportive treatment along with steroid and antibiotics. He recovered well and subsequently tested negative for 2 consecutive swabs. He was discharged after 34 days. CONCLUSIONS Interstitial thickening or reticular pattern on CT has been described in COVID-19 pneumonia, but largely in association with ground-glass opacity or consolidation. This case demonstrates an atypical predominance of interstitial thickening on chest CT in COVID-19 pneumonia on day 14 of illness, which is the expected time of greatest severity of the disease.


Assuntos
Infecções por Coronavirus/diagnóstico , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Tomografia Computadorizada Multidetectores/métodos , Pneumonia Viral/diagnóstico , Intensificação de Imagem Radiográfica , Síndrome Respiratória Aguda Grave/diagnóstico por imagem , Corticosteroides/administração & dosagem , Idoso , Antibacterianos/administração & dosagem , Técnicas de Laboratório Clínico , Meios de Contraste , Infecções por Coronavirus/complicações , Tosse/diagnóstico , Tosse/etiologia , Progressão da Doença , Dispneia/diagnóstico , Dispneia/etiologia , Seguimentos , Humanos , Unidades de Terapia Intensiva , Tempo de Internação , Doenças Pulmonares Intersticiais/complicações , Doenças Pulmonares Intersticiais/terapia , Masculino , Pandemias , Pneumonia Viral/complicações , Pneumonia Viral/diagnóstico por imagem , Medição de Risco , Síndrome Respiratória Aguda Grave/virologia , Resultado do Tratamento
10.
Ann Rheum Dis ; 79(10): 1305-1309, 2020 10.
Artigo em Inglês | MEDLINE | ID: mdl-32737115

RESUMO

OBJECTIVES: The genetic background of rheumatoid arthritis-interstitial lung disease (RA-ILD) has been evaluated in Europeans, but little knowledge has been obtained in non-Europeans. This study aimed to elucidate genome-wide risk of RA-ILD in non-Europeans. METHODS: We performed an initial genome-wide association study (GWAS) of RA-ILD in the Japanese population. By conducting the meta-analysis of the three GWAS datasets of the RA cohorts and biobank of Japanese, our study included 358 RA-ILD cases and 4550 RA subjects without ILD. We then conducted the stratified analysis of the effect of the GWAS risk allele in each CT image pattern. RESULTS: We identified one novel RA-ILD risk locus at 7p21 that satisfied the genome-wide significance threshold (rs12702634 at RPA3-UMAD1, OR=2.04, 95% CI 1.59 to 2.60, p=1.5×10-8). Subsequent stratified analysis based on the CT image patterns demonstrated that the effect size of the RA-ILD risk allele (rs12702634-C) was large with the UIP pattern (OR=1.86, 95% CI 0.97 to 3.58, p=0.062) and the probable UIP pattern (OR=2.26, 95% CI 1.36 to 3.73, p=0.0015). CONCLUSION: We revealed one novel genetic association with RA-ILD in Japanese. The RA-ILD risk of the identified variant at RPA3-UMAD1 was relatively high in the CT image patterns related to fibrosis. Our study should contribute to elucidation of the complicated aetiology of RA-ILD.


Assuntos
Artrite Reumatoide/genética , Proteínas de Ligação a DNA/genética , Doenças Pulmonares Intersticiais/genética , Artrite Reumatoide/complicações , Grupo com Ancestrais do Continente Asiático/genética , Estudo de Associação Genômica Ampla , Genótipo , Humanos , Doenças Pulmonares Intersticiais/complicações , RNA Antissenso/genética
11.
Geriatr Gerontol Int ; 20(10): 878-884, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32770645

RESUMO

AIM: Spontaneous pneumothorax shows a bimodal age distribution, with the secondary peak including patients aged ≥50 years. The purpose of this study was to clarify the etiology and prognosis of spontaneous pneumothorax in the elderly. METHODS: Patients aged ≥50 years who were admitted to a tertiary university hospital between 2006 and 2016 due to spontaneous pneumothorax were retrospectively investigated. RESULTS: Among 136 consecutive patients aged ≥50 years with spontaneous pneumothorax (mean age, 70 years; 114 men), 124 (91%) had underlying lung diseases, including pulmonary emphysema (42%) and interstitial pneumonia (27%). The median period of thoracic drainage was longer (14 days) in the cases with interstitial pneumonia than in the cases of primary pneumothorax (4 days; P < 0.001) and emphysema (9 days; P < 0.005). Eighteen patients (13%) died within 180 days after the onset of pneumothorax. The mortality rate was highest in the cases with interstitial pneumonia (27%) and was mostly associated with infectious complications. Death or worsened respiratory failure within 180 days from admission was associated with older age, systemic corticosteroid use and interstitial pneumonia in multivariate logistic regression analysis. CONCLUSIONS: Pulmonary emphysema is the most common underlying disease associated with spontaneous pneumothorax in the elderly population. Pneumothorax associated with interstitial pneumonia is less frequent, but it requires prolonged tube thoracostomy and demonstrates higher mortality and morbidity, particularly in those receiving systemic corticosteroids. Different treatment strategies are warranted for patients with interstitial pneumonia-related pneumothorax. Geriatr Gerontol Int 2020; 20: 878-884.


Assuntos
Enfisema/complicações , Doenças Pulmonares Intersticiais/complicações , Pneumotórax/etiologia , Pneumotórax/terapia , Idoso , Idoso de 80 Anos ou mais , Feminino , Hospitalização , Humanos , Tempo de Internação , Pneumopatias/complicações , Masculino , Pessoa de Meia-Idade , Pneumotórax/mortalidade , Prognóstico , Doença Pulmonar Obstrutiva Crônica/complicações , Insuficiência Respiratória , Estudos Retrospectivos , Fatores de Risco , Toracostomia , Resultado do Tratamento
12.
J Infect Dis ; 222(10): 1596-1600, 2020 10 13.
Artigo em Inglês | MEDLINE | ID: mdl-32745172

RESUMO

Using a reliable primate model is critical for developing therapeutic advances to treat humans infected with severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2). Here, we exposed macaques to high titers of SARS-CoV-2 via combined transmission routes. We observed acute interstitial pneumonia with endotheliitis in the lungs of all infected macaques. All macaques had a significant loss of total lymphocytes during infection, which were restored over time. These data show that SARS-CoV-2 causes a coronavirus disease 2019 (COVID-19)-like disease in macaques. This new model could investigate the interaction between SARS-CoV-2 and the immune system to test therapeutic strategies.


Assuntos
Betacoronavirus/genética , Infecções por Coronavirus/complicações , Modelos Animais de Doenças , Doenças Pulmonares Intersticiais/complicações , Linfopenia/complicações , Doenças dos Macacos/virologia , Pneumonia Viral/complicações , Animais , Infecções por Coronavirus/patologia , Infecções por Coronavirus/virologia , Feminino , Doenças Pulmonares Intersticiais/patologia , Linfopenia/patologia , Macaca fascicularis , Macaca mulatta , Masculino , Doenças dos Macacos/patologia , Pandemias , Pneumonia Viral/patologia , Pneumonia Viral/virologia , Reação em Cadeia da Polimerase Via Transcriptase Reversa
14.
PLoS One ; 15(7): e0235624, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32634173

RESUMO

BACKGROUND: Spontaneous pneumothorax is a complication that occurs in patients with connective tissue disease-associated interstitial lung disease (CTD-ILD); however, few studies on the clinical implications of pneumothorax for patients with CTD-ILD have been performed. OBJECTIVES: This study aimed to investigate the incidence and prognostic significance of pneumothorax and the risk factors for its onset in patients with CTD-ILD. METHODS: This study included 140 consecutive patients with CTD-ILD. Clinical characteristics, laboratory findings, pulmonary function test results, and chest high-resolution computed tomography (HRCT) images were retrospectively evaluated. RESULTS: A total of 18 patients (12.9%) developed pneumothorax during their clinical course. The cumulative incidence of pneumothorax from the time of CTD-ILD diagnosis was 6.5%, 8.7%, and 11.3% at 1, 3, and 5 years, respectively. The 10-year survival rate was significantly lower in patients with pneumothorax (29.6%) than that in those without pneumothorax (81.3%). The development of pneumothorax was significantly associated with poor prognosis (HR 22.0; p < 0.010). Furthermore, a lower body mass index, greater extent of reticular abnormalities on HRCT, and administration of methylprednisolone pulse therapy were significantly associated with the development of pneumothorax. CONCLUSION: Pneumothorax is a serious complication in the clinical course of patients with CTD-ILD and the onset of pneumothorax predicts a poor outcome.


Assuntos
Doenças do Tecido Conjuntivo/diagnóstico , Doenças Pulmonares Intersticiais/diagnóstico , Pneumotórax/diagnóstico , Idoso , Anti-Inflamatórios/uso terapêutico , Doenças do Tecido Conjuntivo/complicações , Doenças do Tecido Conjuntivo/tratamento farmacológico , Doenças do Tecido Conjuntivo/mortalidade , Feminino , Humanos , Incidência , Estimativa de Kaplan-Meier , Doenças Pulmonares Intersticiais/complicações , Doenças Pulmonares Intersticiais/tratamento farmacológico , Doenças Pulmonares Intersticiais/mortalidade , Masculino , Metilprednisolona/uso terapêutico , Pessoa de Meia-Idade , Pneumotórax/complicações , Pneumotórax/epidemiologia , Prognóstico , Modelos de Riscos Proporcionais , Testes de Função Respiratória , Estudos Retrospectivos , Fatores de Risco , Tomografia Computadorizada por Raios X , Resultado do Tratamento , Capacidade Vital
15.
J Card Surg ; 35(8): 2077-2080, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32652726

RESUMO

Heart-lung transplant (HLT) is a widely accepted modality for certain patients with advanced and refractory cardiopulmonary disease. Some of these patients are critically ill on the transplant waiting list, and venoarterial extracorporeal membrane oxygenation (VA-ECMO) can be used as a bridge to transplantation. Although the experience with ECMO as a bridge to lung transplant is promising, there is limited evidence to use ECMO as a bridge to HLT. Femoral cannulation remains a concern for ambulation given the risk of bleeding and cannula complications despite studies reporting its safety. We present a case of a 56-year-old male with interstitial lung disease and severe secondary pulmonary hypertension, who was successfully bridged to HLT with ambulatory femoral VA-ECMO.


Assuntos
Oxigenação por Membrana Extracorpórea/métodos , Transplante de Coração-Pulmão , Hipertensão Pulmonar/terapia , Doenças Pulmonares Intersticiais/terapia , Transplante de Pulmão , Artéria Femoral , Humanos , Hipertensão Pulmonar/etiologia , Doenças Pulmonares Intersticiais/complicações , Masculino , Pessoa de Meia-Idade , Índice de Gravidade de Doença , Resultado do Tratamento , Listas de Espera
16.
Pneumologie ; 74(11): 780-786, 2020 Nov.
Artigo em Alemão | MEDLINE | ID: mdl-32663889

RESUMO

Clinically amyopathic dermatomyositis (CADM) is a rare entity of dermatomyositis. As a rule, CADM presents without muscular involvement. Thus, the level of creatine kinase is most commonly within the normal range. Dermal manifestations of CADM are Gottron's papules and mechanic's hands. In the case of melanoma differentiation-associated gene 5 intracellular pathogen sensor (MDa5 antibodies), CADM is often associated with a rapidly progressive and severe form of interstitial lung disease. Pulmonary function tests reveal restriction and hypoxemia of varying degree. Features of ground-glass opacities, reticulations and consolidations are detected in high-resolution CT scan. Lymphocytes are sometimes predominant in bronchioloalveolar lavage. Pathologists see a picture similar to non-specific interstitial lung disease or organizing pneumonia. Pronounced immunosuppression is the most common therapy. However, sometimes a combination of different immunosuppressive therapies is necessary. A novel strategy to treat CADM with rapidly progressive interstitial lung disease is the Janus kinase inhibitor tofacitinib. Treatment can be monitored with the level of ferritin and MDa5-antibody titer. Mortality is as high as 84 %.


Assuntos
Dermatomiosite/complicações , Helicase IFIH1 Induzida por Interferon/imunologia , Doenças Pulmonares Intersticiais/complicações , Autoanticorpos/sangue , Creatina Quinase/sangue , Dermatomiosite/diagnóstico , Dermatomiosite/imunologia , Humanos , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/imunologia , Inibidores de Proteínas Quinases
17.
Expert Opin Pharmacother ; 21(16): 2041-2056, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-32674612

RESUMO

INTRODUCTION: Systemic sclerosis (SSc) is a multi-dimensional connective tissue disease of unknown etiology. Given the immense clinical complexity of SSc, the treatment of this condition is not standardized and considerable heterogeneity exists in SSc management approaches. The purpose of this article is to highlight novel therapeutic strategies and new medications under development for the treatment of systemic sclerosis (SSc). AREAS COVERED: Herein, the authors focus primarily on recently completed clinical trials and phase 3 and 4 clinical trials of therapeutic agents that show promise in SSc. This review is organized by the clinical complications that occur in SSc, for which novel treatment strategies are under study. EXPERT OPINION: Combining therapies to address the individual manifestations of SSc is a cornerstone to the comprehensive management of this condition. Therapeutic strategies must take into account the organs involved, the level of disease activity in each area, and the disease stage. Controlling the complex biological network, progressive vasculopathy and fibrosis, as well as manifestations of end-organ dysfunction are all critical considerations when determining the best treatment approach for SSc.


Assuntos
Drogas em Investigação/uso terapêutico , Doenças Pulmonares Intersticiais/tratamento farmacológico , Escleroderma Sistêmico/tratamento farmacológico , Autoanticorpos/sangue , Ensaios Clínicos como Assunto , Ciclofosfamida/administração & dosagem , Ciclofosfamida/uso terapêutico , Drogas em Investigação/administração & dosagem , Humanos , Doenças Pulmonares Intersticiais/complicações , Doenças Pulmonares Intersticiais/imunologia , Ácido Micofenólico/administração & dosagem , Ácido Micofenólico/uso terapêutico , Piridonas/administração & dosagem , Piridonas/uso terapêutico , Rituximab/administração & dosagem , Rituximab/uso terapêutico , Esclerodermia Difusa/complicações , Esclerodermia Difusa/tratamento farmacológico , Esclerodermia Difusa/imunologia , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/imunologia , Resultado do Tratamento
18.
Sci Rep ; 10(1): 10906, 2020 07 02.
Artigo em Inglês | MEDLINE | ID: mdl-32616807

RESUMO

Bronchiolitis manifests as a variety of histological features that explain the complex clinical profiles and imaging aspects. In the period between January 2011 and June 2015, patients with a cryobiopsy diagnosis of bronchiolitis were retrospectively retrieved from the database of our institution. Clinical profiles, imaging features and histologic diagnoses were analysed to identify the role of cryobiopsy in the diagnostic process. Twenty-three patients with a multidisciplinary diagnosis of small airway disease were retrieved (14 females, 9 males; age range 31-74 years old; mean age 54.2 years old). The final MDT diagnoses were post-infectious bronchiolitis (n = 5), constrictive bronchiolitis (n = 3), DIPNECH (n = 1), idiopathic follicular bronchiolitis (n = 3), Sjogren's disease (n = 1), GLILD (n = 1), smoking-related interstitial lung disease (n = 6), sarcoid with granulomatous bronchiolar disorder (n = 1), and subacute hypersensitivity pneumonitis (n = 2). Complications reported after the cryobiopsy procedure consisted of two cases of pneumothorax soon after the biopsy (8.7%), which were successfully managed with the insertion of a chest tube. Transbronchial cryobiopsy represents a robust and mini-invasive method in the characterization of small airway diseases, allowing a low percentage of complications and good diagnostic confidence.


Assuntos
Biópsia/métodos , Bronquiolite/patologia , Adulto , Idoso , Remodelação das Vias Aéreas , Alveolite Alérgica Extrínseca/complicações , Bronquiolite/diagnóstico , Bronquiolite/etiologia , Temperatura Baixa , Feminino , Humanos , Doenças Pulmonares Intersticiais/complicações , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Sarcoidose Pulmonar/complicações , Síndrome de Sjogren/complicações , Fumar/efeitos adversos
19.
PLoS One ; 15(6): e0234090, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32479560

RESUMO

OBJECTIVE: To investigate whether leucine-rich α2-glycoprotein (LRG) can be a biomarker for the disease activity, progression, and prognosis of interstitial pneumonia (IP) in patients with dermatomyositis (DM). METHODS: Correlations between the clinical findings and serum LRG levels were investigated in 46 patients with DM-IP (33 with acute/subacute IP [A/SIP] and 13 patients with chronic IP [CIP], including 10 fatal cases of IP). RESULTS: The median serum LRG level of 18.4 (14.6-25.2) µg/mL in DM-IP patients was higher than that in healthy control subjects. The median levels of serum LRG at baseline and at 2 and 4 weeks after the initiation of treatment in the patients who died were significantly higher than those in the surviving patients (P = 0.026, 0.029, and 0.008, respectively). The median level of serum LRG in the DM-A/SIP patients was significantly higher than that in the DM-CIP patients (P = 0.0004), and that in the anti-MDA5-Ab-positive group was slightly higher than that in the anti-ARS-Ab-positive group. The serum LRG levels correlated significantly with the serum levels of LDH, C-reactive protein, ferritin, AaDO2, %DLco, and total ground-glass opacity score. The survival rate after 24 weeks in patients with an initial LRG level ≥ 17.6 µg/mL (survival rate: 40%) was significantly lower than that in patients with an initial LRG level < 17.6 µg/mL (100%) (P = 0.0009). CONCLUSION: The serum LRG level may be a promising marker of disease activity, progression, and prognosis in patients with DM-IP.


Assuntos
Dermatomiosite/patologia , Glicoproteínas/sangue , Doenças Pulmonares Intersticiais/patologia , Idoso , Autoanticorpos/sangue , Biomarcadores/sangue , Gasometria , Proteína C-Reativa/análise , Estudos de Casos e Controles , Dermatomiosite/complicações , Progressão da Doença , Feminino , Ferritinas/sangue , Humanos , Helicase IFIH1 Induzida por Interferon/imunologia , Doenças Pulmonares Intersticiais/complicações , Doenças Pulmonares Intersticiais/mortalidade , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Taxa de Sobrevida
20.
Intern Med ; 59(16): 2029-2034, 2020 Aug 15.
Artigo em Inglês | MEDLINE | ID: mdl-32493848

RESUMO

In the 2013 updated classification of the American Thoracic Society/European Respiratory Society, airway-centered interstitial fibrosis (ACIF) is included as a bronchiolocentric pattern of interstitial pneumonia (IP) among idiopathic IPs. We encountered a case of severe pulmonary hypertension (PH) with chronic IP. The patient initially presented with shortness of breath and often lost consciousness due to PH, and seven years after his first visit, he ultimately died. An autopsy revealed ACIF and usual IP. In particular, the ACIF comprised non-atypical smooth muscle hyperplasia, and pulmonary hypertensive vascular degeneration was detected. This case may represent a new pathological feature of ACIF.


Assuntos
Broncopatias/complicações , Hipertensão Pulmonar/complicações , Doenças Pulmonares Intersticiais/complicações , Idoso , Broncopatias/patologia , Dispneia/patologia , Fibrose , Humanos , Hiperplasia/patologia , Hipertensão Pulmonar/patologia , Pulmão/patologia , Doenças Pulmonares Intersticiais/patologia , Masculino , Músculo Liso/patologia
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