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https://www.readbyqxmd.com/read/28728932/the-complex-restrictive-pulmonary-function-pattern-clinical-and-radiological-analysis-of-a-common-but-previously-undescribed-restrictive-pattern
#1
Ryan D Clay, Vivek N Iyer, Dereddi Raja Reddy, Brittany Siontis, Paul D Scanlon
BACKGROUND: Most patients with restriction have a PFT pattern in which TLC, FVC and FEV1 are reduced to a similar degree. We call this pattern 'simple' restriction (SR). In contrast, we commonly observe a pattern in which FVC percent predicted (pp) is disproportionately reduced relative to TLCpp. We term this pattern 'complex restriction' (CR) and attempted to characterize its clinical, radiologic and physiologic features. METHODS: We reviewed PFTs of patients tested between November 2009 and June 2013 who had restriction (TLC<LLN)...
July 17, 2017: Chest
https://www.readbyqxmd.com/read/28727155/mucosal-associated-invariant-mait-cells-are-deficient-in-systemic-sclerosis
#2
Arsène Mekinian, Thibaut Mahevas, Mohamad Mohty, Vincent Jachiet, Sébastien Rivière, Olivier Fain, Béatrice Gaugler
OBJECTIVES: Systemic sclerosis (SSc) is an autoimmune disease, characterized by fibrosis of the skin and other organs, vascular impairment and deficient immune responses. Mucosal associated invariant T cells (MAIT) cells have been involved in various inflammatory and autoimmune diseases. The aim of this study was to determine the frequencies of MAIT cells in the blood of patients with systemic sclerosis (SSc), and to compare their distribution in the different types of SSc. PATIENTS AND METHODS: Blood samples from SSc patients and healthy controls were examined by flow cytometer to analyze the frequencies of MAIT and γδ T cells...
July 18, 2017: Scandinavian Journal of Immunology
https://www.readbyqxmd.com/read/28724562/the-role-of-macrophages-in-interstitial-lung-diseases-number-3-in-the-series-pathology-for-the-clinician-edited-by-peter-dorfm%C3%A3-ller-and-alberto-cavazza
#3
Giulio Rossi, Alberto Cavazza, Paolo Spagnolo, Salvatore Bellafiore, Elisabetta Kuhn, Pierpaolo Carassai, Laura Caramanico, Gloria Montanari, Gaia Cappiello, Alessandro Andreani, Francesca Bono, Nazarena Nannini
The finding of collections of macrophages/histiocytes in lung biopsy and bronchoalveolar lavage is relatively common in routine practice. This morphological feature in itself is pathological, but the exact clinical significance and underlying disease should be evaluated together with clinical data, functional respiratory and laboratory tests and imaging studies.Morphological characteristics of macrophages and their distribution along the different pulmonary structures should be examined carefully by pathologists...
September 30, 2017: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/28724445/sustained-benefit-from-combined-plasmapheresis-and-allogeneic-mesenchymal-stem-cells-transplantation-therapy-in-systemic-sclerosis
#4
Huayong Zhang, Jun Liang, Xiaojun Tang, Dandan Wang, Xuebing Feng, Fan Wang, Bingzhu Hua, Hong Wang, Lingyun Sun
BACKGROUND: Systemic sclerosis (SSc) is an autoimmune disease involving the skin and several internal organs. Most therapies available for this disease are symptomatic. Given the difficulty in treating SSc, we conducted this study to investigate the effect of combined plasmapheresis (PE) and allogeneic mesenchymal stem cells transplantation (MSCT) therapy on SSc. METHODS: Fourteen patients underwent three repeated PE treatments with subsequent pulse cyclophosphamide on days 1, 3 and 5...
July 19, 2017: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/28720095/fibrocyte-measurement-in-peripheral-blood-correlates-with-number-of-cultured-mature-fibrocytes-in-vitro-and-is-a-potential-biomarker-for-interstitial-lung-disease-in-rheumatoid-arthritis
#5
Søren Andreas Just, Hanne Lindegaard, Eva Kildall Hejbøl, Jesper Rømhild Davidsen, Niels Bjerring, Søren Werner Karlskov Hansen, Henrik Daa Schrøder, Inger Marie Jensen Hansen, Torben Barington, Christian Nielsen
BACKGROUND: Interstitial lung disease (ILD) can be a severe extra-articular disease manifestation in Rheumatoid Arthritis (RA). A potential role of fibrocytes in RA associated ILD (RA-ILD) has not previously been described. We present a modified faster method for measuring circulating fibrocytes, without intracellular staining. The results are compared to the traditional culture method, where the number of monocytes that differentiate into mature fibrocytes in vitro are counted. The results are following compared to disease activity in patients with severe asthma, ILD, RA (without diagnosed ILD) and RA with verified ILD (RA-ILD)...
July 18, 2017: Respiratory Research
https://www.readbyqxmd.com/read/28699237/evaluation-of-visual-and-computer-based-ct-analysis-for-the-identification-of-functional-patterns-of-obstruction-and-restriction-in-hypersensitivity-pneumonitis
#6
Joseph Jacob, Brian J Bartholmai, Anne Laure Brun, Ryoko Egashira, Srinivasan Rajagopalan, Ronald Karwoski, Vasileios Kouranos, Maria Kokosi, David M Hansell, Athol U Wells
BACKGROUND AND OBJECTIVE: To determine whether computer-based quantification (CALIPER software) is superior to visual computed tomography (CT) scoring in the identification of CT patterns indicative of restrictive and obstructive functional indices in hypersensitivity pneumonitis (HP). METHODS: A total of 135 consecutive HP patients had CT parenchymal patterns evaluated quantitatively by both visual scoring and CALIPER. Results were evaluated against: forced vital capacity (FVC), total lung capacity (TLC), diffusing capacity for carbon monoxide (DLCO ) and a composite physiological index (CPI) to identify which CT scoring method better correlated with functional indices...
July 11, 2017: Respirology: Official Journal of the Asian Pacific Society of Respirology
https://www.readbyqxmd.com/read/28690212/focal-adhesions-in-the-skin-lessons-learned-from-skin-fragility-disorders
#7
Cristina Has, Yinghong He
Focal adhesions are large multiprotein cell-matrix adhesion complexes, which regulate multiple cellular functions, such as adhesion and migration. Their biological significance in skin is underscored by two genetic disorders, the Kindler syndrome and the interstitial lung disease, nephrotic syndrome and epidermolysis bullosa, in which mutations affect focal adhesion proteins, kindlin-1 and the integrin α3 subunit, respectively. Here we provide an overview of what we learned from the study of the molecular mechanisms of these diseases...
June 1, 2017: European Journal of Dermatology: EJD
https://www.readbyqxmd.com/read/28689173/current-understanding-and-management-of-pulmonary-langerhans-cell-histiocytosis
#8
Robert Vassallo, Sergio Harari, Abdellatif Tazi
Pulmonary Langerhans cell histiocytosis (PLCH) is a diffuse lung disease that usually affects young adult smokers. PLCH affects different lung compartments; bronchiolar, interstitial and pulmonary vascular dysfunction may coexist to varying extents, resulting in diverse phenotypes. Analyses of PLCH tissues have identified activating mutations of specific mitogen-activated protein kinases (BRAF(V600E) and others). The current consensus is that PLCH represents a myeloid neoplasm with inflammatory properties: the myeloid tumour cells exhibit surface CD1a expression and up to 50% of the cells harbour activating BRAF or other MAPK mutations...
July 8, 2017: Thorax
https://www.readbyqxmd.com/read/28688028/novel-assessment-of-interstitial-lung-disease-using-the-computer-aided-lung-informatics-for-pathology-evaluation-and-rating-caliper-software-system-in-idiopathic-inflammatory-myopathies
#9
Patompong Ungprasert, Katelynn M Wilton, Floranne C Ernste, Sanjay Kalra, Cynthia S Crowson, Srinivasan Rajagopalan, Brian J Bartholmai
PURPOSE: To evaluate the correlation between measurements from quantitative thoracic high-resolution CT (HRCT) analysis with "Computer-Aided Lung Informatics for Pathology Evaluation and Rating" (CALIPER) software and measurements from pulmonary function tests (PFTs) in patients with idiopathic inflammatory myopathies (IIM)-associated interstitial lung disease (ILD). METHODS: A cohort of patients with IIM-associated ILD seen at Mayo Clinic was identified from medical record review...
July 7, 2017: Lung
https://www.readbyqxmd.com/read/28679612/functional-and-prognostic-effects-when-emphysema-complicates-idiopathic-pulmonary-fibrosis
#10
Joseph Jacob, Brian J Bartholmai, Srinivasan Rajagopalan, Maria Kokosi, Toby M Maher, Arjun Nair, Ronald Karwoski, Elisabetta Renzoni, Simon L F Walsh, David M Hansell, Athol U Wells
This study aimed to investigate whether the combination of fibrosis and emphysema has a greater effect than the sum of its parts on functional indices and outcome in idiopathic pulmonary fibrosis (IPF), using visual and computer-based (CALIPER) computed tomography (CT) analysis.Consecutive patients (n=272) with a multidisciplinary IPF diagnosis had the extent of interstitial lung disease (ILD) scored visually and by CALIPER. Visually scored emphysema was subcategorised as isolated or mixed with fibrotic lung...
July 2017: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/28668422/effects-of-pure-and-hybrid-iterative-reconstruction-algorithms-on-high-resolution-computed-tomography-in-the-evaluation-of-interstitial-lung-disease
#11
Masaki Katsura, Jiro Sato, Masaaki Akahane, Yoko Mise, Kaoru Sumida, Osamu Abe
OBJECTIVES: To compare image quality characteristics of high-resolution computed tomography (HRCT) in the evaluation of interstitial lung disease using three different reconstruction methods: model-based iterative reconstruction (MBIR), adaptive statistical iterative reconstruction (ASIR), and filtered back projection (FBP). METHODS: Eighty-nine consecutive patients with interstitial lung disease underwent standard-of-care chest CT with 64-row multi-detector CT...
August 2017: European Journal of Radiology
https://www.readbyqxmd.com/read/28648837/integrative-characterization-of-chronic-cigarette-smoke-induced-cardiopulmonary-comorbidities-in-a-mouse-model
#12
Ágnes Kemény, Kata Csekő, István Szitter, Zoltán V Varga, Péter Bencsik, Krisztina Kiss, Róbert Halmosi, László Deres, Krisztián Erős, Anikó Perkecz, László Kereskai, Terézia László, Tamás Kiss, Péter Ferdinandy, Zsuzsanna Helyes
Cigarette smoke-triggered inflammatory cascades and consequent tissue damage are the main causes of chronic obstructive pulmonary disease (COPD). There is no effective therapy and the key mediators of COPD are not identified due to the lack of translational animal models with complex characterization. This integrative chronic study investigated cardiopulmonary pathophysiological alterations and mechanisms with functional, morphological and biochemical techniques in a 6-month-long cigarette smoke exposure mouse model...
June 22, 2017: Environmental Pollution
https://www.readbyqxmd.com/read/28621173/evaluation-and-management-approaches-for-scleroderma-lung-disease
#13
Sara R Schoenfeld, Flavia V Castelino
Interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH) are leading causes of morbidity and mortality in systemic sclerosis (SSc). As symptoms are often under-reported in SSc, early screening of ILD and PAH is of paramount importance, and early treatment may be associated with better clinical outcomes. Serologies are particularly helpful in identifying patients at risk for pulmonary involvement. Pulmonary function testing, high-resolution computed tomography of the chest and echocardiography are important tools in the initial screening of these patients...
August 2017: Therapeutic Advances in Respiratory Disease
https://www.readbyqxmd.com/read/28597132/lung-involvement-in-stable-undifferentiated-connective-tissue-diseases-a-rheumatology-perspective
#14
Antonella Riccardi, Rosaria Irace, Ilaria Di Stefano, Michele Iudici, Serena Fasano, Marialuisa Bocchino, Annalisa Capaccio, Alessandro Sanduzzi, Gabriele Valentini
Previous studies of the occurrence of interstitial lung disease (ILD) in undifferentiated connective tissue diseases (UCTD) were conducted in patients admitted to Respiratory Medicine Units. The aim of the present prospective study was to investigate lung involvement in UCTD patients admitted to a Rheumatology Unit. Eighty-one consecutive UCTD patients were enrolled in the study. Each patient underwent history and physical examination, routine laboratory investigations, antinuclear antibody (ANA) profiling, B-mode echocardiography, and lung function study according to previously reported methods...
August 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28595790/trace-metals-in-fluids-lining-the-respiratory-system-of-patients-with-idiopathic-pulmonary-fibrosis-and-diffuse-lung-diseases
#15
REVIEW
Elena Bargagli, Federico Lavorini, Massimo Pistolesi, Elisabetta Rosi, Antje Prasse, Emilia Rota, Luca Voltolini
Idiopathic pulmonary fibrosis (IPF) is an interstitial lung disease with a poor prognosis and an undefined etiopathogenesis. Oxidative stress contributes to alveolar injury and fibrosis development and, because transition metals are essential to the functioning of most proteins involved in redox reactions, a better knowledge of metal concentrations and metabolism in the respiratory system of IPF patients may provide a valuable complementary approach to prevent and manage a disease which is often misdiagnosed or diagnosed in later stages...
July 2017: Journal of Trace Elements in Medicine and Biology
https://www.readbyqxmd.com/read/28586311/mesenchymal-stem-cells-in-idiopathic-pulmonary-fibrosis
#16
REVIEW
Xiaohong Li, Shaojie Yue, Ziqiang Luo
Idiopathic pulmonary fibrosis (IPF) is a major cause of respiratory failure in critically ill patients and common outcome of various lung interstitial diseases. Its mortality remains high, and no effective pharmacotherapy, in addition to artificial ventilation and transplantation, exists. As such, the administration of mesenchymal stem or stromal cells (MSCs) is currently investigated as a new therapeutic method for pulmonary fibrosis. Clinical trials on MSC-based therapy as a potential treatment for lung injury and fibrosis are also performed...
May 23, 2017: Oncotarget
https://www.readbyqxmd.com/read/28585060/rheumatoid-arthritis-related-interstitial-lung-disease-ra-ild-methotrexate-and-the-severity-of-lung-disease-are-associated-to-prognosis
#17
Jorge Rojas-Serrano, Denisse Herrera-Bringas, Diana I Pérez-Román, Renzo Pérez-Dorame, Heidegger Mateos-Toledo, Mayra Mejía
Interstitial lung disease (ILD) is a severe rheumatoid arthritis (RA) manifestation. The worst survival has been associated with usual interstitial pneumonia (UIP) definitive pattern in high-resolution chest tomography (HRCT) scans. Moreover, the use of methotrexate in RA-ILD is controversial. Our aim was to evaluate prognostic factors including methotrexate in an RA-ILD cohort and their association with survival. RA-ILD patients referred for medical evaluation and treatment at a single center were included...
June 6, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28576149/survival-and-quality-of-life-in-incident-systemic-sclerosis-related-pulmonary-arterial-hypertension
#18
Kathleen Morrisroe, Wendy Stevens, Molla Huq, David Prior, Jo Sahhar, Gene-Siew Ngian, David Celermajer, Jane Zochling, Susanna Proudman, Mandana Nikpour
BACKGROUND: Pulmonary arterial hypertension (PAH) is a leading cause of mortality in systemic sclerosis (SSc). We sought to determine survival, predictors of mortality, and health-related quality of life (HRQoL) related to PAH in a large SSc cohort with PAH. METHODS: We studied consecutive SSc patients with newly diagnosed (incident) World Health Organization (WHO) Group 1 PAH enrolled in a prospective cohort between 2009 and 2015. Survival methods were used to determine age and sex-adjusted standardised mortality ratio (SMR) and years of life lost (YLL), and to identify predictors of mortality...
June 2, 2017: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/28570100/serum-matrix-metalloproteinase-7-respiratory-symptoms-and-mortality-in-community-dwelling-adults-the-multi-ethnic-study-of-atherosclerosis
#19
Hilary F Armstrong, Anna J Podolanczuk, R Graham Barr, Elizabeth C Oelsner, Steven M Kawut, Eric A Hoffman, Russell Tracy, Naftali Kaminski, Robyn L McClelland, David J Lederer
RATIONALE: Matrix metalloproteinase-7 (MMP-7) has been implicated in ILD pathobiology and proposed as a diagnostic and prognostic biomarker of IPF. OBJECTIVES: To test associations between serum MMP-7 and lung function, respiratory symptoms, interstitial lung abnormalities (ILA), and all-cause mortality in community-dwelling adults sampled without regard to respiratory symptoms or disease. METHODS: We measured serum MMP-7 in 1,227 participants in the Multi Ethnic Study of Atherosclerosis at basline...
June 1, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28549020/lung-density-and-pulmonary-artery-diameter-are-predictors-of-pulmonary-hypertension-in-systemic-sclerosis
#20
Margreet E Bakker, Maarten K Ninaber, Jan Stolk, Lucia J M Kroft, Anne A Schouffoer, Jeska K de Vries Bouwstra, Suzanne E van Wijngaarden, Berend C Stoel
PURPOSE: The aim was to evaluate computed tomography (CT)-measured pulmonary artery diameter (PAD) and lung density as predictors of pulmonary hypertension (PH) in subjects with systemic sclerosis (SSc). We compared these PAD values with normal values and between SSc subgroups with PH and/or interstitial lung disease (ILD). We investigated whether PAD predicts PH and whether lung densitometry, by using the 85th percentile density value (Perc85) as a measure for ILD, can predict PH. MATERIALS AND METHODS: PAD and Perc85 were measured in axial CT scans and compared between 54 SSc and 76 control subjects...
May 25, 2017: Journal of Thoracic Imaging
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