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1.
FP Essent ; 486: 33-44, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31710456

RESUMO

Interstitial lung disease (ILD) includes approximately 100 separate conditions that fall into four main categories: conditions with known etiologies (eg, connective tissue disease), granulomatous diseases, idiopathic interstitial pneumonias, and miscellaneous conditions. Most patients report unexplained exertional dyspnea that develops insidiously over a variable period. Cough also is common. Because the clinical manifestations of ILD mimic those of other lung diseases, comprehensive testing almost always is required. Testing typically includes chest imaging, pulmonary function testing, and basic laboratory tests. If findings are not consistent with common diagnoses, such as chronic obstructive pulmonary disease, additional testing with high-resolution computed tomography scan and bronchoscopy or surgical lung biopsy can help confirm the diagnosis and type of ILD. Depending on the type, therapy can involve management of the underlying disease (eg, management of an autoimmune condition) or symptomatic treatment. Several drugs and interventions are available to help alleviate symptoms, slow progression, and, in some cases, reverse the condition. In cases of refractory disease, lung transplantation may be required. For patients with progressive disease and contraindications to transplantation, palliative care measures should be considered.


Assuntos
Doenças Pulmonares Intersticiais , Biópsia , Humanos , Pulmão/fisiopatologia , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/terapia , Testes de Função Respiratória
2.
BMJ Case Rep ; 12(8)2019 Aug 01.
Artigo em Inglês | MEDLINE | ID: mdl-31371277

RESUMO

A 70-year-old man presented with 1 month of haematuria and mild right-sided flank pain with no other symptoms. Diagnostic workup included serum studies which showed the presence of antimyeloperoxidase antibodies, a kidney biopsy which demonstrated necrotising crescentic glomerulonephritis with linear immunofluorescence of the basement membrane, and electron microscopy which exhibited thickening of the glomerular basement membrane. Incidentally, the patient was discovered to have a latent hepatitis B infection, which complicated immunosuppressive therapy. He was treated with a course of plasmapheresis and methylprednisolone, followed by entecavir for hepatitis B prophylaxis, and finally by rituximab. This case of glomerulonephritis was notable for its resemblance to the better known Goodpasture's disease. Typically, Goodpasture's syndrome exists on a spectrum from seronegative disease to double-positive disease that presents with both anti-glomerular basement membrane (anti-GBM) and cytoplasmic-antineutrophil cytoplasmic antibodies/antiproteinase 3 antibodies (c-ANCA/anti-PR3). However, this patient's glomerulonephritis was unique because he presented negative for anti-GBM antibodies and positive for perinuclear-antineutrophil cytoplasmic antibodies/antimyeloperoxidase antibodies (p-ANCA/anti-MPO).


Assuntos
Glomerulonefrite/diagnóstico , Doenças Pulmonares Intersticiais/diagnóstico , Peroxidase/imunologia , Idoso , Anticorpos Anticitoplasma de Neutrófilos/metabolismo , Autoanticorpos/metabolismo , Diagnóstico Diferencial , Glomerulonefrite/imunologia , Glomerulonefrite/terapia , Guanina/análogos & derivados , Guanina/uso terapêutico , Humanos , Doenças Pulmonares Intersticiais/imunologia , Doenças Pulmonares Intersticiais/terapia , Masculino , Metilprednisolona/uso terapêutico , Microscopia Eletrônica , Plasmaferese , Rituximab/uso terapêutico , Resultado do Tratamento
3.
J Assoc Physicians India ; 67(3): 28-33, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31304702

RESUMO

Introduction: Interstitial Lung diseases (ILD) are group of disorders wherein due to varied etiologies, interstitium goes into progressive inflammation or fibrosis. Although, the awareness has improved but the therapy is still facing challenges. Pulmonary Rehabilitation (PR) is a worthy modality, which not only supports but also imparts evident benefits in these patients. Material and Methods: The study is a retrospective observational study conducted over a period of 2 years at Pulmonary Rehabilitation center, a private clinic setup on patients with different restrictive lung diseases like interstitial lung diseases, neuromuscular disorders and post-surgical patients. A total of 100 patients were enrolled, out of which 21 patients were lost to follow up. The study population included 34% males and 66 % females with a mean age of 56.3 ± 14.2 years. 24 patients required oxygen support (where SpO2< 90% at baseline). Outcome measures were assessed in these patients at the time of enrollment into the program (0 week) and at the end of the program (8 weeks). Effect of PR programme was then analyzed with appropriate statistical methods. Results: Overall, statistically significant benefits were noted in 6 Minute walk distance (6MWD), muscle strength, dyspnea and Quality of life with 8 weeks. The mean 6 MWTD was 297.9 meters pre PR, which improved to 359.7 meters at the completion of 8 weeks post PR. Mean difference was 61.8 meters, which was found to be statistically significant (p value<0.001) Improvement in muscle strength of different upper and lower limb muscle groups were noted. Also, significant improvement in comprehensive score of Chronic Respiratory Diseases Questionnaire (CRDQ) scores was documented. Statistically significant improvement was found in the dyspnea, fatigue and emotional components. However, mastery components did not show statistically significant change. Conclusion: PR has proven to be a very useful modality in the management of restrictive lung diseases, especially with the known limitations of pharmacological options to treat this disabling chronic lung diseases, even with those with evident type I respiratory failure at the beginning.


Assuntos
Doenças Pulmonares Intersticiais/terapia , Qualidade de Vida , Adulto , Idoso , Tolerância ao Exercício , Feminino , Humanos , Índia , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Inquéritos e Questionários
4.
Medicine (Baltimore) ; 98(26): e16086, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31261523

RESUMO

Pleuroparenchymal fibroelastosis (PPFE) is a rare new interstitial lung disease (ILD) characterized by the fibrotic thickening of the visceral pleura and subadjacent parenchymal areas of the upper lobes This study reveals that patients with ILD-SSc associated with chest HRCT evidence of PPFE require close and recurrent follow-up with periodic evaluation of lung function parameters, DLCO and chest HRCT. Rheumatologists should be aware of this new radiological finding which is accompanied by a negative prognosis, especially when associated with a progressive course. Patients with this radiological pattern need to be monitored with particular attention.


Assuntos
Doenças Pulmonares Intersticiais/complicações , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Doenças Pleurais/complicações , Doenças Pleurais/diagnóstico por imagem , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/diagnóstico por imagem , Idoso , Feminino , Seguimentos , Humanos , Pulmão/diagnóstico por imagem , Doenças Pulmonares Intersticiais/terapia , Masculino , Doenças Pleurais/terapia , Prognóstico , Fibrose Pulmonar/complicações , Fibrose Pulmonar/diagnóstico por imagem , Fibrose Pulmonar/terapia , Estudos Retrospectivos , Reumatologistas , Escleroderma Sistêmico/terapia
5.
Trials ; 20(1): 355, 2019 Jun 13.
Artigo em Inglês | MEDLINE | ID: mdl-31196156

RESUMO

BACKGROUND: People living with chronic heart failure (CHF), chronic obstructive pulmonary disease (COPD), and interstitial lung disease (ILD) suffer impaired quality of life due to burdensome symptoms and depression. The Advancing Symptom Alleviation with Palliative Treatment (ADAPT) trial aims to determine the effect of a multidisciplinary, team-based intervention on quality of life in people with these common diseases. METHODS/DESIGN: The ADAPT trial is a two-site, patient-level randomized clinical trial that examines the effectiveness of the ADAPT intervention compared to usual care on patient-reported quality of life at 6 months in veterans with CHF, COPD or ILD with poor quality of life and increased risk for hospitalization or death. The ADAPT intervention involves a multidisciplinary team-a registered nurse, social worker, palliative care specialist, and primary care provider (with access to a pulmonologist and cardiologist)-who meet weekly to make recommendations and write orders for consideration by participants' individual primary care providers. The nurse and social worker interact with participants over six visits to identify and manage a primary bothersome symptom and complete a structured psychosocial intervention and advance care planning. The primary outcome is change in patient-reported quality of life at 6 months as measured by the Functional Assessment of Chronic Illness Therapy-General questionnaire. Secondary outcomes at 6 months include change in symptom distress, depression, anxiety, disease-specific quality of life hospitalizations, and advance care planning communication and documentation. Intervention implementation will be assessed using a mixed-methods approach including a qualitative assessment of participants' and intervention personnel experiences and a quantitative assessment of care delivery, resources, and cost. DISCUSSION: The ADAPT trial studies an innovative intervention designed to improve quality of life for veterans with common, burdensome illnesses by targeting key underlying factors-symptoms and depression-that impair quality of life but persist despite disease-specific therapies. Leveraging the skills of affiliate health providers with physician supervision will extend the reach of palliative care and improve quality of life for those with advanced disease within routine outpatient care. The hybrid effectiveness/implementation design of the ADAPT trial will shorten the time to broader dissemination if effective and create avenues for future research. TRIAL REGISTRATION: ClinicalTrials.gov, NCT02713347 . Registered March 19, 2016.


Assuntos
Insuficiência Cardíaca/terapia , Doenças Pulmonares Intersticiais/terapia , Cuidados Paliativos , Doença Pulmonar Obstrutiva Crônica/terapia , Qualidade de Vida , Ensaios Clínicos Controlados Aleatórios como Assunto , Planejamento Antecipado de Cuidados , Doença Crônica , Insuficiência Cardíaca/psicologia , Humanos , Doenças Pulmonares Intersticiais/psicologia , Equipe de Assistência ao Paciente , Doença Pulmonar Obstrutiva Crônica/psicologia
6.
Respir Investig ; 57(4): 399-403, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31054836

RESUMO

Transbronchial lung cryobiopsy (TBLC) is a useful and safe method for the diagnosis of interstitial lung disease (ILD). Herein, we describe the cases of two patients who developed ILD from anti-aminoacyl-tRNA synthetase syndrome with respiratory failure. TBLC was performed instead of surgical lung biopsy. There were no complications, and sufficient specimens were harvested to make the precise histopathological diagnosis. TBLC should be considered as a critical approach for the histopathological diagnosis of ILD in patients who cannot undergo surgical lung biopsy because of respiratory failure.


Assuntos
Aminoacil-tRNA Sintetases/imunologia , Autoanticorpos , Biópsia/métodos , Broncoscopia/métodos , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/patologia , Insuficiência Respiratória/patologia , Idoso de 80 Anos ou mais , Ciclosporina/administração & dosagem , Feminino , Humanos , Imunossupressores/administração & dosagem , Pulmão/patologia , Doenças Pulmonares Intersticiais/imunologia , Doenças Pulmonares Intersticiais/terapia , Masculino , Pessoa de Meia-Idade , Oxigenoterapia , Prednisolona/administração & dosagem , Insuficiência Respiratória/imunologia , Insuficiência Respiratória/terapia , Síndrome
7.
Rev Med Liege ; 74(4): 197-203, 2019 Apr.
Artigo em Francês | MEDLINE | ID: mdl-30997969

RESUMO

Non-specific Interstitial Pneumonia (NSIP) is an anatomo-clinical entity within the group of Diffuse Infiltrative Pulmonary Diseases (DPID). It is very rarely found in pediatrics. Main symptoms are dry cough and dyspnea. Bronchoalveolar lavage and biology are non specific. The thoracic CT scan suspects the diagnosis, but histological examination of a lung biopsy remains the reference examination and makes the diagnosis highly probable according to the ATS / ERS criteria. An autoimmune assessment should be performed because NSIPs are often associated with connective tissue disease or may even be the first sign of connectivitive tissues diseases. The treatment of the acute phase is mainly based on the administration of corticosteroids and the prognosis is generally good. In this article, we describe the management of NSIP, based on a pediatric clinical case.


Assuntos
Doenças do Tecido Conjuntivo , Doenças Pulmonares Intersticiais , Adolescente , Biópsia , Criança , Doenças do Tecido Conjuntivo/complicações , Doenças do Tecido Conjuntivo/diagnóstico , Doenças do Tecido Conjuntivo/terapia , Dispneia/etiologia , Humanos , Pulmão , Doenças Pulmonares Intersticiais/complicações , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/terapia
8.
Rev Mal Respir ; 36(4): 508-518, 2019 Apr.
Artigo em Francês | MEDLINE | ID: mdl-31006579

RESUMO

INTRODUCTION: In 2015, the International Society for Heart and Lung Transplantation (ISHLT) published a consensus document for the selection of lung transplant candidates. In the absence of recent French recommendations, this guideline is useful in order to send lung transplant candidates to the transplantation centers and to list them for lung transplantation at the right time. BACKGROUND: The main indications for lung transplantation in adults are COPD and emphysema, idiopathic pulmonary fibrosis and interstitial diseases, cystic fibrosis and pulmonary arterial hypertension (PAH). The specific indications for each underlying disease as well as the general contraindications have been reviewed in 2015 by the ISHLT. For cystic fibrosis, the main factors are forced expiratory volume in one second, 6-MWD, PAH and clinical deterioration characterized by increased frequency of exacerbations; for emphysema progressive disease, the BODE score, hypercapnia and FEV1; for PAH progressive disease or the need of specific intravenous therapy and NYHA classification. Finally, the diagnosis of fibrosing interstitial lung disease is usually a sufficient indication for lung transplantation assessment. OUTLOOK AND CONCLUSION: These new recommendations, close to French practices, help clinicians to find the right time for referral of patients to transplantation centers. This is crucial for the prognosis of lung transplantation.


Assuntos
Transplante de Pulmão/métodos , Seleção de Pacientes , Adulto , Contraindicações , Fibrose Cística/epidemiologia , Fibrose Cística/terapia , França/epidemiologia , Transplante de Coração-Pulmão/efeitos adversos , Transplante de Coração-Pulmão/métodos , Transplante de Coração-Pulmão/normas , Humanos , Hipertensão Pulmonar/epidemiologia , Hipertensão Pulmonar/terapia , Fibrose Pulmonar Idiopática/epidemiologia , Fibrose Pulmonar Idiopática/terapia , Doenças Pulmonares Intersticiais/epidemiologia , Doenças Pulmonares Intersticiais/terapia , Transplante de Pulmão/efeitos adversos , Transplante de Pulmão/normas , Transplante de Pulmão/estatística & dados numéricos , Guias de Prática Clínica como Assunto/normas , Doença Pulmonar Obstrutiva Crônica/epidemiologia , Doença Pulmonar Obstrutiva Crônica/terapia , Enfisema Pulmonar/epidemiologia , Enfisema Pulmonar/terapia
9.
Rev Med Suisse ; 15(641): 536-541, 2019 Mar 06.
Artigo em Francês | MEDLINE | ID: mdl-30860324

RESUMO

Diffuse interstitial lung disease (ILD) is one of the most frequent extra-articular manifestation of rheumatoid arthritis (RA) and is an important factor of morbidity and mortality. However, the physiopathological mechanisms underlying RA-associated ILD remain poorly understood, and disease management is difficult in the absence of effective treatments and international guidelines. The recent identification of genetic variants and mutations similar to those observed in idiopathic pulmonary fibrosis (IPF), a disease affecting exclusively the lung, provides new insights into the understanding of RA-associated ILD. Furthermore, new antifibrotic drugs approved for the treatment of IPF, including pirfenidone and nintedanib, could also prove to be effective for RA-associated ILD. Studies are ongoing to confirm this hypothesis.


Assuntos
Artrite Reumatoide , Fibrose Pulmonar Idiopática , Doenças Pulmonares Intersticiais , Artrite Reumatoide/complicações , Artrite Reumatoide/genética , Artrite Reumatoide/terapia , Humanos , Fibrose Pulmonar Idiopática/genética , Fibrose Pulmonar Idiopática/terapia , Doenças Pulmonares Intersticiais/genética , Doenças Pulmonares Intersticiais/terapia , Resultado do Tratamento
10.
J Ayub Med Coll Abbottabad ; 31(1): 127-128, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30868797

RESUMO

Interstitial lung disease (ILD) is a pathology involving lower respiratory tract, causing damage to the alveolar walls and vascular bed, resulting in decreasing functional alveolar units. Two third cases of ILD are idiopathic and one third cases of ILD are related to connective tissue disease (CTD). Clinical course of ILD is chronic and progressive in nature, CTD-ILD has a better prognosis compared to idiopathic ILD. Acute exacerbations are life-threatening and require close monitoring and immediate treatment, though CTD-ILD initial clinical manifestation at first clinical encounter is rarely found to be life threatening or acute exacerbation of ILD. I am reporting a case of CTD-ILD presenting to the Emergency Room of Memon Medical Institute Hospital (MMIH) manifesting as acute exacerbation of underlying ILD.


Assuntos
Doenças do Tecido Conjuntivo/complicações , Serviços Médicos de Emergência , Doenças Pulmonares Intersticiais/complicações , Doenças Pulmonares Intersticiais/terapia , Adulto , Serviço Hospitalar de Emergência , Feminino , Humanos
11.
PLoS One ; 14(3): e0213444, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30861018

RESUMO

OBJECTIVES: To evaluate interstitial lung disease associated with systemic sclerosis (SSc-ILD) and its changes during treatment by using quantitative analysis (QA) compared to semi-quantitative analysis (semiQA) of chest computed tomography (CT) scans. To assess the prognostic value of QA in predicting functional changes. MATERIALS AND METHODS: We retrospectively selected 35 consecutive patients with SSc-ILD with complete pulmonary functional evaluation, Doppler-echocardiography, immunological tests, and chest CT scan at both baseline and follow-up after immunosuppressive therapy. CT images were analyzed by two chest radiologists for semiQA and by a computational platform for texture analysis of ILD patterns (CALIPER) for QA. Concordance between semiQA and QA was tested. Traction bronchiectasis severity was scored. Analysis of ROC curves was performed. RESULTS: Seventy CT scans were analyzed and QA failed in 4/70 scans. Thus, the final population included 31/35 patients (51.3±12.1 years). QA had a weak-to-good concordance with semiQA (ICC reticular:0.275; ICC ground-glass:0.667) and QA correlated better than semiQA (r = -0.3 to -0.74 vs r = -0.3 to -0.4) with functional parameters. Both methods correlated with traction bronchiectases score and pulmonary artery diameter at CT. A pulmonary artery diameter ≥29mm distinguished patients with lower lung volumes and ILD extent greater than 39% (p<0.001). Changes in QA patterns during treatment were not accurate (AUC: 0.50 to 0.70; p>0.05) in predicting disease progression as assessed by functional parameters, whereas variation in total lung volume at QA accurately predicted changes in the composite functional respiratory endpoint with FVC% and DLco% (AUC = 0.74; 95%CI: 0.54 to 0.93; p = 0.03). CONCLUSIONS: Pulmonary QA of CT images can objectively quantify specific patterns of ILD changes during treatment in patients with SSc-ILD. Changes in QA patterns do not correlate with functional changes, but variation in total lung volume at QA accurately predicted changes in the composite functional respiratory endpoint with FVC% and DLco%. Pulmonary artery diameter at CT reflects the interstitial involvement, identifying patients with more severe prognosis.


Assuntos
Doenças Pulmonares Intersticiais/diagnóstico por imagem , Escleroderma Sistêmico/diagnóstico por imagem , Adulto , Bronquiectasia/diagnóstico por imagem , Feminino , Humanos , Estudos Longitudinais , Doenças Pulmonares Intersticiais/fisiopatologia , Doenças Pulmonares Intersticiais/terapia , Masculino , Pessoa de Meia-Idade , Prognóstico , Artéria Pulmonar/diagnóstico por imagem , Interpretação de Imagem Radiográfica Assistida por Computador , Testes de Função Respiratória , Estudos Retrospectivos , Rituximab/uso terapêutico , Escleroderma Sistêmico/fisiopatologia , Escleroderma Sistêmico/terapia , Tomografia Computadorizada por Raios X/estatística & dados numéricos
12.
Arthritis Rheumatol ; 71(8): 1339-1349, 2019 08.
Artigo em Inglês | MEDLINE | ID: mdl-30762947

RESUMO

OBJECTIVE: Systemic sclerosis (SSc) is associated with interstitial lung disease (ILD) and pulmonary hypertension (PH). This study was undertaken to determine the prevalence, characteristics, treatment, and outcomes of PH in a cohort of patients with SSc-associated ILD. METHODS: Patients with SSc-associated ILD on high-resolution computed tomography (HRCT) were included in a prospective observational cohort. Patients were screened for PH based on a standardized screening algorithm and underwent right-sided heart catheterization (RHC) if indicated. PH classification was based on hemodynamic findings and the extent of ILD on HRCT. Summary statistics and survival using the Kaplan-Meier method were calculated. RESULTS: Of the 93 patients with SSc-associated ILD included in the study, 76% were women and 65.6% had diffuse cutaneous SSc. The mean age was 54.9 years, and the mean SSc disease duration was 8 years. Twenty-nine patients (31.2%) had RHC-proven PH; of those 29 patients, 24.1% had PAH, 55.2% had World Health Organization (WHO) Group III PH, 34.5% had WHO Group III PH with pulmonary vascular resistance >3.0 Wood units, 48.3% had a PH diagnosis within 7 years of SSc onset, 82.8% received therapy for ILD, and 82.8% received therapy for PAH. The survival rate 3 years after SSc-associated ILD diagnosis for all patients was 97%. The survival rate 3 years after PH diagnosis for those with SSc-associated ILD and PH was 91%. CONCLUSION: In a large cohort of patients with SSc-associated ILD, a significant proportion of patients had coexisting PH, which often occurs early after SSc diagnosis. Most patients were treated with ILD and PAH therapies, and survival was good. Patients with SSc-associated ILD should be evaluated for coexisting PH.


Assuntos
Hipertensão Pulmonar/mortalidade , Doenças Pulmonares Intersticiais/mortalidade , Escleroderma Sistêmico/mortalidade , Adulto , Cateterismo Cardíaco , Comorbidade , Feminino , Humanos , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/terapia , Estimativa de Kaplan-Meier , Doenças Pulmonares Intersticiais/etiologia , Doenças Pulmonares Intersticiais/terapia , Masculino , Pessoa de Meia-Idade , Prevalência , Estudos Prospectivos , Escleroderma Sistêmico/complicações , Taxa de Sobrevida , Tomografia Computadorizada por Raios X , Resultado do Tratamento
13.
BMC Pulm Med ; 19(1): 50, 2019 Feb 26.
Artigo em Inglês | MEDLINE | ID: mdl-30808321

RESUMO

OBJECTIVE: Long-term oxygen therapy (LTOT) during 15 h/day or more prolongs survival in patients with chronic obstructive pulmonary disease (COPD) and severe hypoxemia. No randomized controlled trial has evaluated the net effects (benefits or harms) from LTOT 24 h/day compared with 15 h/day or the effect in conditions other than COPD. We describe a multicenter, national, phase IV, non-superiority, registry-based, randomized controlled trial (R-RCT) of LTOT prescribed 24 h/day compared with 15 h/day. The primary endpoint is all-cause-mortality at 1 year. Secondary endpoints include cause-specific mortality, hospitalizations, health-related quality of life, symptoms, and outcomes in interstitial lung disease. METHODS/DESIGN: Patients qualifying for LTOT are randomized to LTOT 24 h/day versus 15 h/day during 12 months using the Swedish Register for Respiratory Failure (Swedevox). Planned sample size in this pragmatic study is 2126 randomized patients. Clinical follow-up and concurrent treatments are according to routine clinical practice. Mortality, hospitalizations, and incident diseases are assessed using national Swedish registries with expected complete follow-up. Patient-reported outcomes are assessed using postal questionnaire at 3 and 12 months. DISCUSSION: The R-RCT approach combines the advantages of a prospective randomized trial and large clinical national registries for enrollment, allocation, and data collection, with the aim of improving the evidence-based use of LTOT. TRIAL REGISTRATION: Clinical Trial registered with www.clinicaltrials.gov , Title: REgistry-based Treatment Duration and Mortality in Long-term OXygen Therapy (REDOX); ID: NCT03441204.


Assuntos
Doenças Pulmonares Intersticiais/terapia , Oxigenoterapia/métodos , Doença Pulmonar Obstrutiva Crônica/terapia , Sistema de Registros , Nível de Saúde , Hospitalização/estatística & dados numéricos , Humanos , Doenças Pulmonares Intersticiais/mortalidade , Doenças Pulmonares Intersticiais/fisiopatologia , Doença Pulmonar Obstrutiva Crônica/mortalidade , Doença Pulmonar Obstrutiva Crônica/fisiopatologia , Qualidade de Vida , Método Simples-Cego , Taxa de Sobrevida , Suécia , Fatores de Tempo , Resultado do Tratamento
14.
Jpn J Clin Oncol ; 49(5): 458-464, 2019 May 01.
Artigo em Inglês | MEDLINE | ID: mdl-30793176

RESUMO

INTRODUCTION: Although chemoradiotherapy for locally advanced lung cancer has the potential for cure, treatment is avoided in patients with interstitial lung disease because of the risk for severe radiation pneumonitis. Interstitial lung abnormalities (ILA) can be evaluated using high-resolution computed tomography (HRCT) to assess interstitial changes. In this study, we retrospectively examined the feasibility and efficacy of chemoradiotherapy for locally advanced lung cancer patients with ILA. METHODS: Patients who underwent chemoradiotherapy for locally advanced lung cancer at Okayama University Hospital between 2012 and 2015 were reviewed retrospectively. HRCT prior to treatment was evaluated by one pulmonologist and two radiologists using a sequential reading method. RESULTS: Of the 77 patients enrolled in this study, ILA was present in 25 (32.5%) and indeterminate ILA in 24 patients; 28 patients did not have ILA. Desaturation at rest (SpO2 < 95%) and honeycombing on HRCT were not observed in ILA patients. Only one patient with ILA had a low vital capacity (%VC < 80%). Severe radiation pneumonitis (≥Grade 2) occurred in 36.0% of the patients with ILA, but it was controllable; Grade 4 or 5 was not observed. Multivariate analysis showed that >25% of the lung volume receiving >20 Gy was risk factors of severe radiation pneumonitis, but ILA was not. The 2-year survival rates of patients with and without ILA were 56.8% and 74.1%, respectively, but the difference was not significant (P = 0.33). CONCLUSIONS: Chemoradiotherapy was feasible and effective in some patient population with ILA without desaturation, low VC and honeycombing on HRCT.


Assuntos
Quimiorradioterapia , Doenças Pulmonares Intersticiais/complicações , Doenças Pulmonares Intersticiais/terapia , Neoplasias Pulmonares/complicações , Neoplasias Pulmonares/terapia , Adulto , Idoso , Idoso de 80 Anos ou mais , Quimiorradioterapia/efeitos adversos , Estudos de Viabilidade , Feminino , Humanos , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Neoplasias Pulmonares/diagnóstico por imagem , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Pneumonite por Radiação/etiologia , Estudos Retrospectivos , Fatores de Risco , Tomografia Computadorizada por Raios X , Resultado do Tratamento
15.
J Photochem Photobiol B ; 191: 123-127, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30616036

RESUMO

Selinium nanoparticles (SeNPs) with minimal toxicity and efficient antioxidant properties were reported earlier for their anti-carcinogenic influence against various types of cancers, thus elevating its potential. In the present study, the anti-carcinogenic effect of selenium nanoparticles against lung cancer was studied. Selenium nanoparticles were biosynthesized and were characterized using UV- Vis absorption spectroscopy. A decrease in the absorption intensity was recorded with the increase in time, which represented the protein consumption during the reduction of SeO32- to Se0. The calculated average crystalline size from XRD studies of the synthesized selenium nanoparticles was found to be 88.89 nm which was in accordance with the TEM analysis while the SAED pattern has disclosed hexagonal ring structure with diffraction ring pattern.MTT assay was performed to evaluate the radio-sensitizing effect of selenium nanoparticles under the X-ray influence against cancer as well as healthy cell lines. SeNPs showed potent cytotoxicity effect in cancer cells whereas it showed relatively less toxic effect in normal healthy cells. However, caspase-3 activity was even more elevated when subjected to X-ray exposure than in the absence. These findings apparently revealed the cytotoxic potential of SeNPs + X-ray combination in the lung cancer cell lines.


Assuntos
Doenças Pulmonares Intersticiais/terapia , Neoplasias Pulmonares/terapia , Nanopartículas/química , Selênio/uso terapêutico , Antioxidantes/farmacologia , Caspase 3/metabolismo , Linhagem Celular , Humanos , Doenças Pulmonares Intersticiais/tratamento farmacológico , Doenças Pulmonares Intersticiais/radioterapia , Neoplasias Pulmonares/radioterapia , Nanopartículas/uso terapêutico , Radiossensibilizantes/química , Radiossensibilizantes/uso terapêutico , Raios X
16.
Respir Res ; 20(1): 13, 2019 Jan 18.
Artigo em Inglês | MEDLINE | ID: mdl-30658650

RESUMO

BACKGROUND: Systemic sclerosis (SSc) is a rare connective tissue disease with a heterogeneous clinical course. Interstitial lung disease (ILD) is a common manifestation of SSc and a leading cause of death. MAIN BODY: All patients newly diagnosed with SSc should receive a comprehensive clinical evaluation, including assessment of respiratory symptoms, a high-resolution computed tomography (HRCT) scan of the chest, and pulmonary function tests. ILD can develop in any patient with SSc, including those with pulmonary hypertension, but the risk is increased in those with diffuse (rather than limited) cutaneous SSc, those with anti-Scl-70/anti-topoisomerase I antibody, and in the absence of anti-centromere antibody. While it can occur at any time, the risk of developing ILD is greatest early in the course of SSc, so patients should be monitored closely in the first few years after diagnosis. An increased extent of lung fibrosis on HRCT and a low forced vital capacity (FVC) are predictors of early mortality. While not all patients will require treatment, current approaches to the treatment of progressive SSc-ILD focus on immunosuppressant therapies, including cyclophosphamide and mycophenolate mofetil. In patients with severe and/or rapidly progressive disease, both haematopoietic stem cell transplantation (HSCT) and lung transplantation have been successfully used. A number of medications, including the two drugs approved for the treatment of idiopathic pulmonary fibrosis (IPF), are under active investigation as potential new therapies for SSc-ILD. CONCLUSIONS: Physicians managing patients with SSc should maintain a high level of suspicion and regularly monitor for ILD, particularly in the first few years after diagnosis.


Assuntos
Doenças Pulmonares Intersticiais/fisiopatologia , Doenças Pulmonares Intersticiais/terapia , Escleroderma Sistêmico/fisiopatologia , Escleroderma Sistêmico/terapia , Anti-Inflamatórios/uso terapêutico , Transplante de Células-Tronco Hematopoéticas/métodos , Transplante de Células-Tronco Hematopoéticas/tendências , Humanos , Doenças Pulmonares Intersticiais/epidemiologia , Transplante de Pulmão/métodos , Transplante de Pulmão/tendências , Ensaios Clínicos Controlados Aleatórios como Assunto/métodos , Escleroderma Sistêmico/epidemiologia
18.
Clin Rheumatol ; 38(3): 803-815, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30392161

RESUMO

OBJECTIVE: To study prognostic factors in different types of idiopathic inflammatory myopathies (IIM) associated with interstitial lung disease (ILD). PATIENTS AND METHODS: Multicenter retrospective study of a Spanish cohort of patients diagnosed with IIM. Patients were classified into four categories: polymyositis (PM), dermatomyositis (DM), antisynthetase syndrome (ASS), and overlap myositis (OM). Sociodemographic data, clinical characteristics, antibodies, and treatments were collected. Cox regression models were calculated to identify factors associated with mortality, the necessity for long-term oxygen therapy (LTOT), and deterioration in respiratory function tests (RFT). RESULTS: The number of patients included was 478, of whom 112 (23.4%) suffered from ILD: 17% PM, 16% DM, 45% ASS, and 22% OM. Factors associated with mortality in the multivariate analysis were clinically meaningful progression of ILD after 3 months (CMP 3m) (hazard ratio (HR) 9.48, p = 0.005), severe infections (HR 6.41, p = 0.016), heliotrope erythema (HR 31.1, p = 0.002), delay in diagnosis (HR 1.29; p = 0.011), and Raynaud's phenomenon (HR 11.9, p = 0.007). However, being female (HR 0.19, p = 0.044) and positivity solely for ANAs (HR 0.08, p = 0.008) presented a protective effect. CMP 3m (HR 22.7, p = 0.027) was associated with the need for LTOT, while basal aldolase (HR 0.90; p = 0.049) had a protective effect. Likewise, joint manifestations (HR 0.04, p = 0.034) were shown to reduce risk of deterioration in RFT. CONCLUSIONS: CMP 3m, severe infections, delay in diagnosis, heliotrope erythema, and Raynaud's phenomenon were identified as factors of poor prognosis in different IIM associated with ILD.


Assuntos
Doenças Pulmonares Intersticiais/fisiopatologia , Mortalidade , Miosite/fisiopatologia , Oxigenoterapia/estatística & dados numéricos , Adolescente , Adulto , Idoso , Anticorpos Antinucleares/imunologia , Diagnóstico Tardio/estatística & dados numéricos , Dermatomiosite/epidemiologia , Dermatomiosite/imunologia , Dermatomiosite/fisiopatologia , Progressão da Doença , Eritema/epidemiologia , Feminino , Frutose-Bifosfato Aldolase/metabolismo , Humanos , Estimativa de Kaplan-Meier , Estudos Longitudinais , Doenças Pulmonares Intersticiais/epidemiologia , Doenças Pulmonares Intersticiais/terapia , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Miosite/epidemiologia , Miosite/imunologia , Polimiosite/epidemiologia , Polimiosite/imunologia , Polimiosite/fisiopatologia , Prognóstico , Modelos de Riscos Proporcionais , Fatores de Proteção , Capacidade de Difusão Pulmonar , Doença de Raynaud/epidemiologia , Testes de Função Respiratória , Estudos Retrospectivos , Fatores de Risco , Fatores Sexuais , Espanha/epidemiologia , Capacidade Vital , Adulto Jovem
19.
Eur J Pediatr ; 178(2): 121-129, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30547349

RESUMO

The filamin A gene (FLNA) on Xq28 encodes the filamin A protein. Mutation in FLNA causes a wide spectrum of disease including skeletal dysplasia, neuronal migration abnormality, cardiovascular malformation, intellectual disability and intestinal obstruction. Recently, childhood-onset interstitial lung disease associated with a range of FLNA mutations has been recognised and reported. We document our personal experience of this emerging disorder and compile a comprehensive overview of clinical features and molecular changes in all identifiable published cases. Reviewing the emerging dataset, we underline this unanticipated phenotypic consequence of pathogenic FLNA mutation-associated pulmonary disease.Conclusion: From the emerging data, we suggest that while reviewing complex cases with a sustained oxygen requirement against a clincial background of cardiac concerns or intestinal obstruction to have a high index of suspicion for FLNA related pathology and to instigate early MRI brain scan and FLNA mutation analysis. What is Known: • FLNA gene on Xq28 encodes the filamin A protein and mutation therein is associated with variable phenotypes depending on its nature of mutation. • Loss-of-function mutation of filamin A is associated with X-linked inherited form of periventricular nodular heterotopia with or without epilepsy with most individuals affected being female. There is a recently recognised associated respiratory phenotype. What is New: • The respiratory phenotype in the form of childhood interstitial lung disease is a recently recognised clinical consequence of loss-of-function FLNA mutation. • Rare male patients with loss-of-function FLNA mutation-associated lung disease with residual protein function can survive into infancy with a severe form of the phenotype.


Assuntos
Filaminas/genética , Doenças Pulmonares Intersticiais/genética , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Pulmão/patologia , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/terapia , Imagem por Ressonância Magnética , Masculino , Mutação , Heterotopia Nodular Periventricular/genética , Fenótipo , Tomografia Computadorizada por Raios X , Ultrassonografia Doppler Transcraniana
20.
Eur Respir Rev ; 27(150)2018 Dec 31.
Artigo em Inglês | MEDLINE | ID: mdl-30578331

RESUMO

Acute exacerbation of interstitial lung disease (ILD) is associated with a poor prognosis and high mortality. Numerous studies have documented acute exacerbation in idiopathic pulmonary fibrosis (IPF), but less is known about these events in other ILDs that may present a progressive-fibrosing phenotype. We propose defining acute exacerbation as an acute, clinically significant respiratory deterioration, typically less than 1 month in duration, together with computerised tomography imaging showing new bilateral glass opacity and/or consolidation superimposed on a background pattern consistent with fibrosing ILDs. Drawing on observations in IPF, it is suspected that epithelial injury or proliferation and autoimmunity are risk factors for acute exacerbation in ILDs that may present a progressive-fibrosing phenotype, but further studies are required. Current acute exacerbation management strategies are based on recommendations in IPF, but no randomised controlled trials of acute exacerbation management have been performed. Although there are no formal strategies to prevent the development of acute exacerbation, possible approaches include antifibrotic drugs (such as nintedanib and pirfenidone), and minimising exposure to infection, airborne irritants and pollutants. This review discusses the current knowledge of acute exacerbation of ILDs that may present a progressive-fibrosing phenotype and acknowledges limitations of the data available.


Assuntos
Doenças Pulmonares Intersticiais/terapia , Pulmão/efeitos dos fármacos , Fibrose Pulmonar/terapia , Progressão da Doença , Feminino , Humanos , Pulmão/diagnóstico por imagem , Pulmão/fisiopatologia , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Doenças Pulmonares Intersticiais/mortalidade , Doenças Pulmonares Intersticiais/fisiopatologia , Masculino , Pessoa de Meia-Idade , Fenótipo , Prognóstico , Fibrose Pulmonar/diagnóstico por imagem , Fibrose Pulmonar/mortalidade , Fibrose Pulmonar/fisiopatologia , Medição de Risco , Fatores de Risco
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