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https://www.readbyqxmd.com/read/29327616/nintedanib-for-the-treatment-of-idiopathic-pulmonary-fibrosis
#1
Francesco Varone, Giacomo Sgalla, Bruno Iovene, Teresa Bruni, Luca Richeldi
Idiopathic Pulmonary Fibrosis (IPF) is an interstitial lung disease characterized by the progressive loss of pulmonary function, ultimately leading to respiratory failure and death. Two novel compounds, nintedanib and pirfenidone, have shown efficacy in reducing the rate of decline of lung function in IPF patients. The multiple tyrosine kinase inhibitor nintedanib has extensively being studied as a potential angiogenesis inhibitor in clinical against various neoplastic disorders. Afterwards, this compound was successfully tested in IPF...
January 12, 2018: Expert Opinion on Pharmacotherapy
https://www.readbyqxmd.com/read/29327393/pulmonary-hypertension-in-interstitial-lung-disease-limitations-of-echocardiography-compared-to-cardiac-catheterization
#2
Gregory J Keir, S John Wort, Maria Kokosi, Peter M George, Simon L F Walsh, Joseph Jacob, Laura Price, Simon Bax, Elisabetta A Renzoni, Toby M Maher, Peter MacDonald, David M Hansell, Athol U Wells
BACKGROUND AND OBJECTIVE: In interstitial lung disease (ILD), pulmonary hypertension (PH) is a major adverse prognostic determinant. Transthoracic echocardiography (TTE) is the most widely used tool when screening for PH, although discordance between TTE and right heart catheter (RHC) measured pulmonary haemodynamics is increasingly recognized. We evaluated the predictive utility of the updated European Society of Cardiology/European Respiratory Society (ESC/ERS) TTE screening recommendations against RHC testing in a large, well-characterized ILD cohort...
January 12, 2018: Respirology: Official Journal of the Asian Pacific Society of Respirology
https://www.readbyqxmd.com/read/29325675/mycophenolate-mofetil-as-a-therapeutic-agent-for-interstitial-lung-diseases-in-systemic-sclerosis
#3
REVIEW
Takahiro Ueda, Takuro Sakagami, Toshiaki Kikuchi, Toshinori Takada
Systemic sclerosis (SSc) is an intractable disease that causes fibrosis in all organs. Approximately 40% of patients with SSc have some degree of interstitial lung disease (ILD). One third of patients with SSc and ILD, approximately 15% of all patients, have pulmonary lesions, which slowly progress to respiratory failure resistant to corticosteroid and other treatments. A randomized controlled trial conducted in the United States indicated that one year of treatment with oral cyclophosphamide in patients with SSc-ILD had a significant but modest beneficial effect on lung function, dyspnea, thickening of the skin, and health-related quality of life...
January 2018: Respiratory Investigation
https://www.readbyqxmd.com/read/29325580/the-predictive-prognostic-factors-for-polymyositis-dermatomyositis-associated-interstitial-lung-disease
#4
Yumiko Sugiyama, Ryusuke Yoshimi, Maasa Tamura, Mitsuhiro Takeno, Yosuke Kunishita, Daiga Kishimoto, Yuji Yoshioka, Kouji Kobayashi, Kaoru Takase-Minegishi, Toshiyuki Watanabe, Naoki Hamada, Hideto Nagai, Naomi Tsuchida, Yutaro Soejima, Hiroto Nakano, Reikou Kamiyama, Takeaki Uehara, Yohei Kirino, Akiko Sekiguchi, Atsushi Ihata, Shigeru Ohno, Shouhei Nagaoka, Hideaki Nakajima
BACKGROUND: Interstitial lung disease (ILD) is the principal cause of death in polymyositis/dermatomyositis (PM/DM). Here we investigated prognostic factors for death and serious infection in PM/DM-ILD using the multicenter database. METHODS: We retrospectively reviewed baseline demographic, clinical and laboratory findings, treatment regimens and outcomes in patients with PM/DM-ILD. The distribution of ILD lesions was evaluated in four divided lung zones of high-resolution computed tomography images...
January 11, 2018: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/29325094/functional-rescue-of-misfolding-abca3-mutations-by-small-molecular-correctors
#5
Susanna Kinting, Stefanie Höppner, Ulrike Schindlbeck, Maria E Forstner, Jacqueline Harfst, Thomas Wittmann, Matthias Griese
ABCA3, a phospholipid transporter in lung lamellar bodies (LB), is essential for the assembly of pulmonary surfactant and LB biogenesis. Mutations in the ABCA3 gene are an important genetic cause for respiratory distress syndrome in neonates and interstitial lung disease in children and adults, for which there is currently no cure.The aim of this study was to prove that disease causing misfolding ABCA3 mutations can be corrected in vitro and to investigate available options for correction.We stably expressed HA-tagged wild type ABCA3 or variants p...
January 9, 2018: Human Molecular Genetics
https://www.readbyqxmd.com/read/29323413/extracorporeal-membrane-oxygenation-for-refractory-severe-respiratory-failure-in-acute-interstitial-pneumonia
#6
Gabriela Gonçalves-Venade, Nuno Lacerda-Príncipe, Roberto Roncon-Albuquerque, José Artur Paiva
Acute interstitial pneumonia (AIP) is a rare idiopathic interstitial lung disease with rapid progressive respiratory failure and high mortality. In the present report, three cases of AIP complicated by refractory respiratory failure supported with extracorporeal membrane oxygenation (ECMO) are presented. One male and two female patients (ages 27-59) were included. Venovenous ECMO support was provided using miniaturized systems, with two-site femoro-jugular circuit configuration. Despite lung protective ventilation, prone position and neuromuscular blockade, refractory respiratory failure of unknown etiology supervened (ratio of arterial oxygen partial pressure to fractional inspired oxygen 46-130) and ECMO was initiated after 3-7 days of mechanical ventilation...
January 11, 2018: Artificial Organs
https://www.readbyqxmd.com/read/29322341/interstitial-lung-disease-in-systemic-sclerosis-data-from-the-spanish-scleroderma-study-group
#7
D Sánchez-Cano, N Ortego-Centeno, J L Callejas, V Fonollosa Plá, R Ríos-Fernández, C Tolosa-Vilella, G Espinosa-Garriga, D Colunga-Argüelles, M V Egurbide-Arberas, M Rubio-Rivas, M Freire, J J Ríos-Blanco, L Trapiella-Martínez, M Rodríguez-Carballeira, A Marín-Ballvé, X Pla-Salas, C P Simeón-Aznar
OBJECTIVES: To evaluate the clinical characteristics of patients with interstitial lung disease (ILD) in the setting of a large cohort of systemic sclerosis (SSc) patients, and to analyse the differences according to the SSc subtype (following the modification of classification criteria of the American College of Rheumatology for SSc proposed by LeRoy and Medsger), factors are associated with moderate-to-severe impairment of lung function, as well as mortality and causes of death. METHODS: A descriptive study was performed, using the available data from the Spanish Scleroderma Study Group...
January 10, 2018: Rheumatology International
https://www.readbyqxmd.com/read/29321510/a-model-of-human-lung-fibrogenesis-for-the-assessment-of-anti-fibrotic-strategies-in-idiopathic-pulmonary-fibrosis
#8
Katy M Roach, Amanda Sutcliffe, Laura Matthews, Gill Elliott, Chris Newby, Yassine Amrani, Peter Bradding
Idiopathic pulmonary fibrosis (IPF) is a progressive interstitial lung disease with limited therapeutic options. KCa3.1 ion channels play a critical role in TGFβ1-dependent pro-fibrotic responses in human lung myofibroblasts. We aimed to develop a human lung parenchymal model of fibrogenesis and test the efficacy of the selective KCa3.1 blocker senicapoc. 2 mm3 pieces of human lung parenchyma were cultured for 7 days in DMEM ± TGFβ1 (10 ng/ml) and pro-fibrotic pathways examined by RT-PCR, immunohistochemistry and collagen secretion...
January 10, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29320838/-advances-in-autoantibodies-of-idiopathic-inflammatory-myopathy-and-interstitial-lung-disease
#9
M M Xie, H R Cai
No abstract text is available yet for this article.
November 12, 2017: Chinese Journal of Tuberculosis and Respiratory Diseases
https://www.readbyqxmd.com/read/29320807/overlap-of-interstitial-pneumonia-with-autoimmune-features-with-undifferentiated-connective-tissue-disease-and-contribution-of-uip-to-mortality
#10
Bryan T Kelly, Teng Moua
BACKGROUND AND OBJECTIVE: Criteria for interstitial pneumonia with autoimmune features (IPAF) were recently established for research purposes in a joint statement from the European Respiratory Society (ERS) and American Thoracic Society (ATS). We reviewed the utility of these criteria in patients previously diagnosed as broadly defined undifferentiated connective tissue disease (UCTD) and noted overlapping IPAF findings. Additional review was given to IPAF patients with usual interstitial pneumonia (UIP) on histopathology or radiology in terms of survival and outcome...
January 10, 2018: Respirology: Official Journal of the Asian Pacific Society of Respirology
https://www.readbyqxmd.com/read/29320550/longitudinal-micro-ct-as-an-outcome-measure-of-interstitial-lung-disease-in-tnf-transgenic-mice
#11
Richard D Bell, Christopher Rudmann, Ronald W Wood, Edward M Schwarz, Homaira Rahimi
INTRODUCTION: Rheumatoid arthritis associated interstitial lung disease (RA-ILD) is a debilitating condition with poor survival prognosis. High resolution computed tomography (CT) is a common clinical tool to diagnose RA-ILD, and is increasingly being adopted in pre-clinical studies. However, murine models recapitulating RA-ILD are lacking, and CT outcomes for inflammatory lung disease have yet to be formally validated. To address this, we validate μCT outcomes for ILD in the tumor necrosis factor transgenic (TNF-Tg) mouse model of RA...
2018: PloS One
https://www.readbyqxmd.com/read/29320306/disease-staging-and-sub-setting-of-interstitial-lung-disease-associated-with-systemic-sclerosis-impact-on-therapy
#12
Peter M George, Athol U Wells
Interstitial lung disease (ILD) is the most serious complication of systemic sclerosis (SSc). There is no accepted guidance as to which clinical, radiological or physiological thresholds should prompt initiation or changes in treatment. Furthermore, some patients with extensive disease remain stable without the need for intervention whilst others with limited disease at the outset, experience a precipitous decline. Areas Covered: In this article, evidence for the integration of a number of disease-specific and patient-related domains are discussed and proposed...
January 10, 2018: Expert Review of Clinical Immunology
https://www.readbyqxmd.com/read/29319036/the-use-of-adaptive-intensity-modulated-radiotherapy-in-the-treatment-of-small-cell-carcinoma-lung-refractory-to-chemotherapy-in-a-patient-with-preexisting-interstitial-lung-disease
#13
Sarthak Tandon, Munish Gairola, Manoj Pal, Archana Aggarwal, Kanika Sharma, Ahmad Masroor Karimi, Avik Mandal, Rajiv Goyal, Ullas Batra, Inderjit Kaur
This is a case report of a 60-year-old diabetic, hypertensive male with a good performance status and a history of bilateral interstitial lung disease with a left upper lobe lung mass diagnosed to be a Stage IIB mixed small-cell/squamous cell carcinoma which was refractory to carboplatin- and etoposide-based chemotherapy. The patient was then taken up for adaptive intensity-modulated radiotherapy with tighter margin under image guidance with a mid-treatment replanning done at 25#. Acute toxicities were assessed weekly and showed no Grade 3 or more reactions...
January 2018: Lung India: Official Organ of Indian Chest Society
https://www.readbyqxmd.com/read/29316368/rash-fever-and-pulmonary-hypertension-in-a-6-year-old-female
#14
David Buchbinder, Gina A Montealegre Sanchez, Raphaela Goldbach-Mansky, Hermine Brunner, Andrew I Shulman
A previously healthy 2-year-old Guatemalan female with an undiagnosed chronic illness characterized by fever and rash had presented with anorexia, weight loss, periorbital edema, abdominal pain and distention. A chest radiograph documented cardiomegaly. An echocardiogram demonstrated a pericardial effusion, dilated right atrium, right ventricle, and main pulmonary artery as well as diminished right ventricular systolic function and pulmonary hypertension. Right ventricular systolic pressure was estimated at 90 mmHg...
January 5, 2018: Arthritis Care & Research
https://www.readbyqxmd.com/read/29316328/anti-fibrotic-effects-of-pirfenidone-by-interference-with-the-hedgehog-signalling-pathway-in-patients-with-systemic-sclerosis-associated-interstitial-lung-disease
#15
Hua Xiao, Guang-Feng Zhang, Xiang-Ping Liao, Xiao-Jie Li, Jian Zhang, Haobo Lin, Zhe Chen, Xiao Zhang
AIM: To determine whether pirfenidone attenuates lung fibrosis by interfering with the hedgehog (Hh) signalling pathway in patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD). METHODS: Twenty-five SSc-ILD patients (20 first visit, five who underwent pirfenidone treatment for 6 months) and 10 healthy controls were recruited. Lung tissues were obtained by open-chest surgery, and primary lung fibroblasts were isolated, cultured and stimulated with pirfenidone...
January 7, 2018: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/29308284/an-unusual-flare-of-anti-synthetase-syndrome-during-concurrent-trastuzumab-therapy-given-for-recurrent-breast-cancer
#16
Timothy D Reynolds, Vivek Mohan, Matthew Roy, Nathan Manghat, Huzaifa Adamali, Harsha Gunawardena
We present the case of a patient with relapsing anti-synthetase syndrome (ASS) that may have been triggered by monoclonal antibody trastuzumab therapy given for breast cancer. A 52-year-old female with a history of anti-Jo1-associated ASS went into remission with glucocorticoids and mycophenolate mofetil. Her past history included invasive ductal carcinoma of the right breast that was fully treated six years prior to the onset of ASS. She subsequently developed recurrent right-sided breast cancer that was treated with right mastectomy and six cycles of cyclophosphamide-docetaxel chemotherapy...
December 2017: European Journal of Rheumatology
https://www.readbyqxmd.com/read/29303281/-idiopathic-pulmonary-fibrosis-can-we-always-diagnose-and-treat-it-right
#17
Martina Vašáková
Idiopathic pulmonary fibrosis (IPF) is a severe pulmonary disease characterized by fibrotisation of lung tissue based on pathological healing of alveolar lesions. The disease is initiated in individuals of middle and older age who have a genetical disposition to fibroproliferation. Clinical presentation is not uniform. The patients can have a rapidly progressive disease, eventually severe acute exacerbations, however some of them can have relatively benign course of the disease. Problems in diagnosis are usually caused by atypical radiologic findings and no or nondiagnostic lung biopsy...
2018: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/29297433/interstitial-lung-disease-and-risk-of-mortality-11-year-nationwide-population-based-study
#18
W-I Choi, S H Park, S Dauti, B-J Park, C W Lee
BACKGROUND: Patients with interstitial lung disease (ILD) constitute a substantial disease burden. Although ILD outcomes have been investigated, the risk of death due to ILD has not been studied in the light of confounders and comorbidities. In this nationwide, 11-year longitudinal, population-based study, we aimed to discover if ILD is an independent risk factor for mortality. DESIGN: Data on 1 031 392 (2.2%) randomly selected subjects from 47 279 373 Korean residents were collected from the 2002 Korean National Health Insurance database...
January 1, 2018: International Journal of Tuberculosis and Lung Disease
https://www.readbyqxmd.com/read/29297420/the-rise-of-interstitial-lung-diseases
#19
Rogelio Pérez-Padilla, Moisés Selman
No abstract text is available yet for this article.
January 1, 2018: International Journal of Tuberculosis and Lung Disease
https://www.readbyqxmd.com/read/29297205/cyclophosphamide-for-connective-tissue-disease-associated-interstitial-lung-disease
#20
REVIEW
Hayley Barnes, Anne E Holland, Glen P Westall, Nicole Sl Goh, Ian N Glaspole
BACKGROUND: Approximately one-third of individuals with interstitial lung disease (ILD) have associated connective tissue disease (CTD). The connective tissue disorders most commonly associated with ILD include scleroderma/systemic sclerosis (SSc), rheumatoid arthritis, polymyositis/dermatomyositis, and Sjögren's syndrome. Although many people with CTD-ILD do not develop progressive lung disease, a significant proportion do progress, leading to reduced physical function, decreased quality of life, and death...
January 3, 2018: Cochrane Database of Systematic Reviews
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