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https://www.readbyqxmd.com/read/29229096/clinical-characteristics-of-patients-with-anti-aminoacyl-trna-synthetase-antibody-positive-idiopathic-interstitial-pneumonia
#1
Hirokazu Yura, Noriho Sakamoto, Minoru Satoh, Hiroshi Ishimoto, Tetsuya Hanaka, Chiyo Ito, Tomoko Hasegawa, Shin Tanaka, Takuto Miyamura, Shota Nakashima, Atsuko Hara, Tomoyuki Kakugawa, Keishi Oda, Takashi Kido, Yasushi Obase, Yuji Ishimatsu, Kazuhiro Yatera, Atsushi Kawakami, Hiroshi Mukae
BACKGROUND: Anti-aminoacyl-tRNA synthetase (ARS) antibodies have been detected in patients with polymyositis/dermatomyositis (PM/DM) and are especially correlated with interstitial lung disease (ILD). The aim of this study was to clarify the clinical features of patients with anti-ARS antibody positive idiopathic interstitial pneumonias (IIPs). METHODS: Patients were classified into three groups: 1) IIP with anti-ARS antibodies (ARS(+)IIP), 2) IIP without anti-ARS antibodies (ARS(-)IIP), and 3) PM/DM-associated ILD with anti-ARS antibodies (ARS(+)PM/DM-ILD)...
November 2017: Respiratory Medicine
https://www.readbyqxmd.com/read/29223948/-hypersensitivity-pneumonitis-a-series-of-nine-cases-with-surgical-lung-biopsy
#2
Ricardo A Gómez Tejada, Cora G Legarreta, Juan Ignacio Enghelmayer, Milagros Dianti, Silvana Acuña, Gloria Olmedo
In a series of nine patients with histopathological diagnosis of hypersensitivity pneumonitis, we retrospectively evaluated clinical data, exposure related factors, pulmonary function tests and chest computed tomography scans. A restrictive abnormality with reduction of diffusion capacity for carbon monoxide was mainly found. Chest scans showed fibrotic patterns in most cases; ground glass attenuation areas with mosaic pattern and consolidation in the rest. Exposure to avian antigens, cereal grains and air conditioners contaminated with fungi yeasts and bacteria, were suspected from clinical data in two-thirds of the cases...
2017: Medicina
https://www.readbyqxmd.com/read/29219887/short-term-automated-quantification-of-radiologic-changes-in-the-characterization-of-idiopathic-pulmonary-fibrosis-versus-nonspecific-interstitial-pneumonia-and-prediction-of-long-term-survival
#3
Federica De Giacomi, Sushravya Raghunath, Ronald Karwoski, Brian J Bartholmai, Teng Moua
PURPOSE: Fibrotic interstitial lung diseases presenting with nonspecific and overlapping radiologic findings may be difficult to diagnose without surgical biopsy. We hypothesized that baseline quantifiable radiologic features and their short-term interval change may be predictive of underlying histologic diagnosis as well as long-term survival in idiopathic pulmonary fibrosis (IPF) presenting without honeycombing versus nonspecific interstitial pneumonia (NSIP). MATERIALS AND METHODS: Forty biopsy-confirmed IPF and 20 biopsy-confirmed NSIP patients with available high-resolution chest computed tomography 4 to 24 months apart were studied...
December 6, 2017: Journal of Thoracic Imaging
https://www.readbyqxmd.com/read/29212838/long-term-macrolides-in-diffuse-interstitial-lung-diseases
#4
REVIEW
Paola Faverio, Francesco Bini, Adriano Vaghi, Alberto Pesci
In the present review we provide currently available evidence for the use of macrolides in the treatment of diffuse interstitial lung diseases (ILDs). Up to now, research on macrolides has mainly focused on three areas. First, macrolides have shown some promising results in cellular models and case reports as antifibrotic agents, by promoting autophagy and clearance of intracellular protein aggregates and acting as regulators of surfactant homeostasis. Secondly, macrolides have an immunomodulatory effect, which has been applied in some organising pneumonia cases...
December 31, 2017: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/29212510/factors-affecting-treatment-outcome-in-patients-with-idiopathic-nonspecific-interstitial-pneumonia-a-nationwide-cohort-study
#5
Sang Hoon Lee, Moo Suk Park, Song Yee Kim, Dong Soon Kim, Young Whan Kim, Man Pyo Chung, Soo Taek Uh, Choon Sik Park, Sung Woo Park, Sung Hwan Jeong, Yong Bum Park, Hong Lyeol Lee, Jong Wook Shin, Eun Joo Lee, Jin Hwa Lee, Yangin Jegal, Hyun Kyung Lee, Yong Hyun Kim, Jin Woo Song, Jong Sun Park
BACKGROUND: The effects of corticosteroid-based therapy in patients with idiopathic nonspecific interstitial pneumonia (iNSIP), and factors affecting treatment outcome, are not fully understood. We aimed to investigate the long-term treatment response and factors affecting the treatment outcome in iNSIP patients from a multi-center study in Korea. METHODS: The Korean interstitial lung disease (ILD) Study Group surveyed ILD patients from 2003 to 2007. Patients were divided into two groups to compare the treatment response: response group (forced vital capacity (FVC) improves ≥10% after 1 year) and non-response group (FVC <10%)...
December 6, 2017: Respiratory Research
https://www.readbyqxmd.com/read/29209910/efficacy-and-safety-of-nintedanib-for-the-treatment-of-idiopathic-pulmonary-fibrosis-an-update
#6
José Antonio Rodríguez-Portal
Idiopathic pulmonary fibrosis is a fatal form of progressive fibrosing interstitial pneumonia with limited treatment options. In recent years, its management has been transformed with the approval of two new antifibrotic drugs: nintedanib and pirfenidone. Nintedanib is a tyrosine kinase inhibitor that efficiently slows idiopathic pulmonary fibrosis progression and has an acceptable tolerability profile. This article reviews new available evidence on the long-term efficacy and safety of nintedanib in patients with idiopathic pulmonary fibrosis...
December 5, 2017: Drugs in R&D
https://www.readbyqxmd.com/read/29206632/unclassifiable-interstitial-lung-disease-outcome-prediction-using-ct-and-functional-indices
#7
Joseph Jacob, Brian J Bartholmai, Srinivasan Rajagopalan, Ryoko Egashira, Anne Laure Brun, Maria Kokosi, Arjun Nair, Simon L F Walsh, Ronald Karwoski, Andrew G Nicholson, David M Hansell, Athol U Wells
BACKGROUND: Unclassifiable-interstitial lung disease (uILD) represents a heterogeneous collection of pathologies encompassing those fibrosing lung diseases which do not fulfill current diagnostic criteria. We evaluated baseline and longitudinal functional and CT (visual and quantitative computer [CALIPER] analysis) variables to identify outcome predictors in uILD. METHODS: Consecutive patients with uILD on multidisciplinary review (n = 95) had baseline functional (FVC, DLco, CPI [composite physiologic index]) and CT features (visual evaluation: CT pattern, fibrosis extent, honeycombing presence, traction bronchiectasis severity, pulmonary artery (PA) diameter; CALIPER evaluation: fibrosis extent, pulmonary vessel volume (PVV)) examined in univariate and multivariate Cox regression models...
September 2017: Respiratory Medicine
https://www.readbyqxmd.com/read/29202589/interstitial-lung-disease-in-systemic-autoimmune-rheumatic-diseases-a-comprehensive-review
#8
Fabiola Atzeni, Maria Chiara Gerardi, Giuseppe Barilaro, Ignazio Francesco Masala, Maurizio Benucci, Piercarlo Sarzi-Puttini
Interstitial lung diseases (ILDs) are among the most serious complications associated with systemic rheumatic diseases, and lead to significant morbidity and mortality; they may also be the first manifestation of connective tissue diseases (CTDs). The aim of this narrative review is to summarise the data concerning the pathogenesis of CTD/ILD and its distinguishing features in different rheumatic diseseas. Areas covered: The pathogenesis, clinical aspects and treatment of ILD associated with rheumatic systemic diseases and CTDs were reviewed by searching the PubMed, Medline, and Cochrane Library databases for papers published between 1995 and February 2017 using combinations of words or terms...
December 5, 2017: Expert Review of Clinical Immunology
https://www.readbyqxmd.com/read/29194717/validity-of-ultrasound-lung-comets-for-assessment-of-the-severity-of-interstitial-pneumonia
#9
Mariko Asano, Hiroyuki Watanabe, Kazuhiro Sato, Yuji Okuda, Sho Sakamoto, Yukiyasu Hasegawa, Kazuhisa Sudo, Masahide Takeda, Masaaki Sano, Satoshi Kibira, Hiroshi Ito
OBJECTIVES: Ultrasound (US) lung comets are often observed in patients with interstitial lung disease or congestive heart failure, but few studies have explored the clinical importance of US lung comets in patients with the former condition. We explored whether the US lung comet number could be used to assess the severity of interstitial pneumonia. METHODS: Forty stable patients with interstitial pneumonia were examined. Lung comets evident on transthoracic US imaging in 12 selected regions of the posterior chest wall were analyzed...
November 30, 2017: Journal of Ultrasound in Medicine: Official Journal of the American Institute of Ultrasound in Medicine
https://www.readbyqxmd.com/read/29186838/role-of-micrornas-in-tgf-%C3%AE-signaling-pathway-mediated-pulmonary-fibrosis
#10
REVIEW
Hara Kang
Pulmonary fibrosis is the most common form of interstitial lung disease. The transforming growth factor-β (TGF-β) signaling pathway is extensively involved in the development of pulmonary fibrosis by inducing cell differentiation, migration, invasion, or hyperplastic changes. Accumulating evidence indicates that microRNAs (miRNAs) are dysregulated during the initiation of pulmonary fibrosis. miRNAs are small noncoding RNAs functioning as negative regulators of gene expression at the post-transcriptional level...
November 25, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29185086/interstitial-lung-disease-in-south-africans-with-systemic-sclerosis
#11
Philippa Ashmore, Mohammed Tikly, Michelle Wong, Claudia Ickinger
To investigate the frequency, severity and predictors of interstitial lung disease (ILD) in a cohort of South Africans with systemic sclerosis (SSc). Retrospective record review of SSc patients attending a tertiary Connective Tissue Diseases Clinic. Patients with ILD, defined by a combination of clinical findings, imaging, and lung function tests were compared to patients without ILD in terms of demographics, clinical features and autoantibodies. The majority (86.8%) of the 151 patients included were of Black ethnicity, 40% had ILD, of whom 39% had moderate-severe lung disease...
November 28, 2017: Rheumatology International
https://www.readbyqxmd.com/read/29172671/pulmonary-quantitative-ct-imaging-in-focal-and-diffuse-disease-current-research-and-clinical-applications
#12
Mario Silva, Gianluca Milanese, Valeria Seletti, Alarico Ariani, Nicola Sverzellati
The frenetic development of imaging technology - both hardware and software - provides exceptional potential for investigation of the lung. In the last two decades, computed tomography (CT) was exploited for detailed characterization of pulmonary structures and description of respiratory disease. The introduction of volumetric acquisition allowed increasingly sophisticated analysis of CT data by means of computerized algorithm, namely quantitative computed tomography (QCT). Hundreds of thousands of CTs have been analyzed for characterization of focal and diffuse disease of the lung...
November 27, 2017: British Journal of Radiology
https://www.readbyqxmd.com/read/29170036/mucins-mucus-and-goblet-cells
#13
REVIEW
Jonathan Ma, Bruce K Rubin, Judith A Voynow
The respiratory epithelium is lined by mucus, a gel consisting of water, ions, proteins and macromolecules. The major macromolecular components of mucus are the mucin glycoproteins, which are critical for local defense of the airway. There are three classes of mucins in the airways: those that are secreted but do not polymerize (MUC7), those that are secreted and polymerize to form gels (MUC5AC, MUC5B), and those that have transmembrane domains and are cell surface associated (MUC1, MUC4, MUC16, MUC20). The mucins are regulated at the transcriptional, post-transcriptional and epigenetic level, and post-translational modifications play an important role in mucin binding and clearance of microbes and pollutants...
November 20, 2017: Chest
https://www.readbyqxmd.com/read/29168054/cardiac-function-in-patients-with-polymyositis-or-dermatomyositis-a-three-dimensional-speckle-tracking-echocardiography-study
#14
Yue Zhong, Wenjuan Bai, Qibing Xie, Jianhong Sun, Hong Tang, Li Rao
Cardiac event is a major cause of death in patients with idiopathic inflammatory myopathies (IIM). The most frequent IIMs are polymyositis (PM) and dermatomyositis (DM). The purpose of this study was to analyze cardiac involvement by three-dimensional speckle-tracking echocardiography (3D STE) in patients with PM or DM, and to identify the relationship of cardiac injury with clinical characteristics and disease-specific parameters. 60 PM/DM patients with preserved left ventricular ejection fraction and 30 matched healthy controls were assessed by conventional echocardiography, 3D STE with biventricular strain analysis and electrocardiogram...
November 22, 2017: International Journal of Cardiovascular Imaging
https://www.readbyqxmd.com/read/29164837/serum-surfactant-protein-d-and-exhaled-nitric-oxide-as-biomarkers-of-early-lung-damage-in-systemic-sclerosis
#15
Alida Benfante, Riccardo Messina, Alessandra Paternò, Nicola Scichilone
BACKGROUND: Interstitial lung disease (ILD) complicates the course of systemic sclerosis (SSc), representing the main cause of death in these patients. The identification of parameters that can predict the early onset and progression of ILD in SSc represents an unmet need in clinical practice. The study was designed to explore whether the surfactant proteins (SP) A and D may be used as noninvasive tools for the early identification of ILD in SSc. Alveolar exhaled nitric oxide (NO) was investigated as a surrogate marker of distal inflammation...
November 21, 2017: Minerva Medica
https://www.readbyqxmd.com/read/29164429/red-blood-cell-distribution-width-as-a-related-factor-of-pulmonary-arterial-hypertension-in-patients-with-systemic-sclerosis
#16
Jiuliang Zhao, Hongnan Mo, Xiaoxiao Guo, Qian Wang, Dong Xu, Yong Hou, Zhuang Tian, Yongtai Liu, Hui Wang, Jinzhi Lai, Mengtao Li, Xiaofeng Zeng
The aim of this study was to investigate the utility of red blood cell distribution width (RDW) as a simple and readily available marker of occurrence of pulmonary arterial hypertension (PAH) in patients with systemic sclerosis (SSc). One hundred and forty-five consecutive patients with SSc were recruited to the single-center cross-sectional study. Demographic characteristics, hematological parameters, Modified Rodnan Skin Score, and World Health Organization functional classification were determined. Diagnosis of PAH was based on screening by echocardiography and was confirmed by right heart catheterization...
November 21, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/29139195/phenotypic-characteristics-associated-with-slow-gait-speed-in-idiopathic-pulmonary-fibrosis
#17
Claire M Nolan, Matthew Maddocks, Toby M Maher, Jane L Canavan, Sarah E Jones, Ruth E Barker, Suhani Patel, Joseph Jacob, Paul Cullinan, William D-C Man
BACKGROUND AND OBJECTIVE: Usual gait speed over 4 m (4MGS) is an established functional performance measure in older adults that consistently predicts adverse health outcomes, but few data exist in idiopathic pulmonary fibrosis (IPF). We assessed the reliability of 4MGS, its relationship with established outcome measures and its responsiveness to pulmonary rehabilitation. METHODS: In four prospective IPF cohorts, 4MGS inter-observer (n = 46) and test-retest (n = 46) reliability, concurrent validity (n = 65 and n = 62) and responsiveness (n = 60) were determined...
November 14, 2017: Respirology: Official Journal of the Asian Pacific Society of Respirology
https://www.readbyqxmd.com/read/29138863/therapeutic-effect-of-ulinastatin-on-pulmonary-fibrosis-via-downregulation-of-tgf%C3%A2-%C3%AE-1-tnf%C3%A2-%C3%AE-and-nf%C3%A2-%C3%AE%C2%BAb
#18
Dejun Li, Hongsheng Ji, Bao Zhao, Chunyang Xu, Wenjun Xia, Lihui Han, Dongqing Yu, Yuanrong Ju, Changjun Jin
Pulmonary fibrosis is a chronic, progressive, lethal lung disease characterized by alveolar cell necrosis and dysplasia of interstitial fibrotic tissue, resulting in loss of lung function and eventual respiratory failure. Previously, glucocorticoid drugs were used to treat this lung disorder. However, positive responses were recorded in less than half of treated patients and the cytotoxicity caused by high dosage treatment is still a concern. The present study investigated whether ulinastatin, a typical urinary trypsin inhibitor that mitigates numerous inflammatory responses, could be a treatment option for lung fibrosis...
November 14, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/29137621/copa-syndrome-in-an-icelandic-family-caused-by-a-recurrent-missense-mutation-in-copa
#19
Brynjar O Jensson, Sif Hansdottir, Gudny A Arnadottir, Gerald Sulem, Ragnar P Kristjansson, Asmundur Oddsson, Stefania Benonisdottir, Hakon Jonsson, Agnar Helgason, Jona Saemundsdottir, Olafur T Magnusson, Gisli Masson, Gudmundur A Thorisson, Adalbjorg Jonasdottir, Aslaug Jonasdottir, Asgeir Sigurdsson, Ingileif Jonsdottir, Vigdis Petursdottir, Jon R Kristinsson, Daniel F Gudbjartsson, Unnur Thorsteinsdottir, Reynir Arngrimsson, Patrick Sulem, Gunnar Gudmundsson, Kari Stefansson
BACKGROUND: Rare missense mutations in the gene encoding coatomer subunit alpha (COPA) have recently been shown to cause autoimmune interstitial lung, joint and kidney disease, also known as COPA syndrome, under a dominant mode of inheritance. CASE PRESENTATION: Here we describe an Icelandic family with three affected individuals over two generations with a rare clinical presentation of lung and joint disease and a histological diagnosis of follicular bronchiolitis...
November 14, 2017: BMC Medical Genetics
https://www.readbyqxmd.com/read/29135399/-investigation-of-parameters-related-to-prognosis-in-diffuse-parenchymal-lung-diseases-prognosis-in-interstitial-lung-diseases
#20
Coşkun Canıvar, Züleyha Bingöl, Zeki Kılıçaslan, Tülin Çağatay, N Gülfer Okumuş
Introduction: Parameters related to prognosis in diffuse parenchymal lung disease (DPLD) have a decisive influence on treatment and follow-up processes. We aimed to define baseline characteristics and factors that effect the mortality of the group of patients with DPLD and to determine distinctions between subgroups. Materials and Methods: Demographic characteristics, complaints, comorbidity, treatment, pulmonary function tests, echocardiographic findings, six minute walking test (6MWT), arterial blood gases analysis, radiological findings and survival time were collected from outpatient clinics database...
September 2017: Tüberküloz Ve Toraks
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