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https://www.readbyqxmd.com/read/28926468/rituximab-experience-in-patients-with-long-standing-systemic-sclerosis-associated-interstitial-lung-disease-a-series-of-14-patients
#1
Alper Sari, Denizcan Guven, Berkan Armagan, Abdulsamet Erden, Umut Kalyoncu, Omer Karadag, Sule Apras Bilgen, Ihsan Ertenli, Sedat Kiraz, Ali Akdogan
OBJECTIVES: The objective of this study was to report the experience with rituximab treatment in a case series of patients with long-standing systemic sclerosis-associated interstitial lung disease (SSc-ILD). METHODS: We reviewed the charts of 197 SSc patients. Fourteen patients who received rituximab for SSc-ILD participated in this analysis. Pulmonary function tests, high-resolution thorax computed tomography and modified Rodnan skin scores were evaluated at baseline and end of the follow-up...
September 19, 2017: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
https://www.readbyqxmd.com/read/28925574/radiographic-fibrosis-score-predicts-survival-in-systemic-sclerosis-associated-interstitial-lung-disease
#2
Reoto Takei, Machiko Arita, Shogo Kumagai, Yuhei Ito, Fumiaki Tokioka, Takashi Koyama, Rintaro Saito, Keisuke Nishimura, Hironobu Tokumasu, Tadashi Ishida
BACKGROUND AND OBJECTIVE: Interstitial lung disease (ILD) is a common pulmonary manifestation of systemic sclerosis (SSc). It is unknown whether radiographic fibrosis score predicts mortality in SSc-associated ILD (SSc-ILD). We retrospectively analysed patients with SSc-ILD to evaluate whether radiographic fibrosis score was a useful predictor of mortality. METHODS: We identified SSc-ILD patients evaluated at Kurashiki Central Hospital (Japan) from 2006 to 2016, and radiographic fibrosis scores based on the extent of reticulation and honeycombing on high-resolution computed tomography (HRCT) scanning were calculated by manually tracing around each fibrotic area...
September 19, 2017: Respirology: Official Journal of the Asian Pacific Society of Respirology
https://www.readbyqxmd.com/read/28924106/intravenous-immunoglobulin-monotherapy-for-granulomatous-lymphocytic-interstitial-lung-disease-in-common-variable-immunodeficiency
#3
Mizue Hasegawa, Fumikazu Sakai, Asako Okabayashi, Akitoshi Sato, Naoko Yokohori, Hideki Katsura, Chihiro Asano, Toshiko Kamata, Eitetsu Koh, Yasuo Sekine, Kenzo Hiroshima, Takashi Ogura, Tamiko Takemura
Common variable immunodeficiency (CVID) is a heterogeneous subset of immunodeficiency disorders. Recurrent bacterial infection is the main feature of CVID, but various non-infectious complications can occur. A 42-year-old woman presented with cough and abnormal chest X-ray shadows. Laboratory tests showed remarkable hypogammaglobulinemia. Computed tomography revealed multiple consolidation and nodules on the bilateral lung fields, systemic lymphadenopathy, and splenomegaly. A surgical lung biopsy specimen provided the final diagnosis of lymphoproliferative disease in CVID, which was grouped under the term granulomatous lymphocytic interstitial lung disease...
September 15, 2017: Internal Medicine
https://www.readbyqxmd.com/read/28923440/interstitial-lung-disease-in-the-connective-tissue-diseases-a-paradigm-shift-in-diagnosis-and-treatment
#4
Paul F Dellaripa
Interstitial lung disease (ILD) in the connective tissue diseases (CTD) is amongst the most challenging aspect of care of patients with rheumatic diseases and is the source of significant morbidity and mortality. While there has been progress in our understanding of the natural history of these complications, we still suffer from a limited reservoir of data to confidently determine which patients are at highest risk for disease and those who are at highest risk for disease progression. Treatment options until recently have been limited to anti-inflammatory therapies but with the emerging availability of anti-fibrotic therapies, a shift in strategy is emerging to target therapies based on the specific radiographic, histopathologic features and biomarker profiles that are unique to patients with rheumatic diseases and ILD...
September 15, 2017: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/28923086/usefulness-of-lung-ultrasound-b-lines-in-connective-tissue-disease-associated-interstitial-lung-disease-a-literature-review
#5
REVIEW
YuKai Wang, Luna Gargani, Tatiana Barskova, Dan E Furst, Marco Matucci Cerinic
Interstitial lung disease (ILD) is a major pulmonary manifestation of connective tissue disease (CTD), leading to significant morbidity and mortality. Chest high-resolution computed tomography (HRCT) is presently considered the diagnostic gold standard for pulmonary fibrosis diagnosis and quantification in the clinical arena. However, not negligible doses of ionizing radiation limit the use of HRCT, especially for serial follow-up in younger female patients. In the past decade, lung ultrasound (LUS) has been proposed to assess ILD by detecting and quantifying sonographic B-lines...
September 18, 2017: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/28921478/interstitial-pneumonia-with-autoimmune-features-an-additional-risk-factor-for-ards
#6
Giacomo Grasselli, Beatrice Vergnano, Maria Rosa Pozzi, Vittoria Sala, Gabriele D'Andrea, Vittorio Scaravilli, Marco Mantero, Alberto Pesci, Antonio Pesenti
BACKGROUND: Interstitial pneumonia with autoimmune features (IPAF) identifies a recently recognized autoimmune syndrome characterized by interstitial lung disease and autoantibodies positivity, but absence of a specific connective tissue disease diagnosis or alternative etiology. We retrospectively reviewed the clinical presentation, diagnostic workup and management of seven critically ill patients who met diagnostic criteria for IPAF. We compared baseline characteristics and clinical outcome of IPAF patients with those of the population of ARDS patients admitted in the same period...
September 18, 2017: Annals of Intensive Care
https://www.readbyqxmd.com/read/28918442/the-role-of-periostin-in-lung-fibrosis-and-airway-remodeling
#7
REVIEW
David N O'Dwyer, Bethany B Moore
Periostin is a protein that plays a key role in development and repair within the biological matrix of the lung. As a matricellular protein that does not contribute to extracellular matrix structure, periostin interacts with other extracellular matrix proteins to regulate the composition of the matrix in the lung and other organs. In this review, we discuss the studies exploring the role of periostin to date in chronic respiratory diseases, namely asthma and idiopathic pulmonary fibrosis. Asthma is a major health problem globally affecting millions of people worldwide with significant associated morbidity and mortality...
September 16, 2017: Cellular and Molecular Life Sciences: CMLS
https://www.readbyqxmd.com/read/28916165/muc1-deficiency-exacerbates-pulmonary-fibrosis-in-a-mouse-model-of-silicosis
#8
Kosuke Kato, Marina A Zemskova, Alec D Hanss, Marianne M Kim, Sandra J Gendler, Ross Summer, Kwang Chul Kim
BACKGROUND: MUC1 (MUC in human and Muc in animals) is a membrane-tethered mucin expressed on the apical surface of lung epithelial cells. However, in the lungs of patients with interstitial lung disease, MUC1 is aberrantly expressed in hyperplastic alveolar type II epithelial (ATII) cells and alveolar macrophages (AM), and elevated levels of extracellular MUC1 are found in bronchoalveolar lavage (BAL) fluid and the serum of these patients. While pro-fibrotic effects of extracellular MUC1 have recently been described in cultured fibroblasts, the contribution of MUC1 to the pathobiology of pulmonary fibrosis is unknown...
September 12, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/28915678/meta-analysis-of-the-incidence-and-risks-of-interstitial-lung-disease-and-qtc-prolongation-in-non-small-cell-lung-cancer-patients-treated-with-alk-inhibitors
#9
Liping Lin, Juanjuan Zhao, Ning Kong, Yan He, Jiazhu Hu, Fuxi Huang, Jianjun Han, Xiaolong Cao
BACKGROUND: To conduct a systematic review and meta-analysis to assess the overall incidence and risk of interstitial lung disease (ILD) and QTc prolongation associated with anaplastic lymphoma kinase (ALK)-tyrosine kinase inhibitors (-TKIs) in non-small-cell lung cancer (NSCLC) patients. RESULTS: A total of 1,770 patients from 8 clinical trials were included. The incidences of high-grade ILD and QTc prolongation was 2.5% (95% CI 1.7-3.6%), and 2.8% (95% CI 1.8-4...
August 22, 2017: Oncotarget
https://www.readbyqxmd.com/read/28914826/pulmonary-alveolar-proteinosis-a-case-report
#10
Luis Alberto Concepción-Urteaga, Luis Alejandro Rodríguez-Hidalgo, Jorge Luis Cornejo-Portella, Oscar Neri Alquizar-Horna, Daniel Anderson Aguilar-Villanueva, Marcio José Concepción-Zavaleta, Mario Gustavo Azañero-Luján
INTRODUCTION: Pulmonary alveolar proteinosis is a rare, diffuse interstitial lung disease, characterized by alveolar obstruction due to the accumulation of pulmonary surfactant. CLINICAL PRESENTATION: A 30-year-old male with progressively worsening dyspnea and non-productive cough for one year. He was a sugar cane plantation worker and had prior recurrent respiratory infections. Physical exam revealed cyanosis, and bilateral coarse and fine rales. Chest computed tomography showed diffuse crazy paving pattern...
September 14, 2017: Medwave
https://www.readbyqxmd.com/read/28914637/signal-transducer-and-activator-of-transcription-gain-of-function-primary-immunodeficiency-immunodysregulation-disorders
#11
Filippo Consonni, Laura Dotta, Francesca Todaro, Donatella Vairo, Raffaele Badolato
PURPOSE OF REVIEW: To describe primary immunodeficiencies caused by gain-of-function (GOF) mutations of signal transducer and activator of transcription (STAT) genes, a group of genetically determined disorders characterized by susceptibility to infections and, in many cases, autoimmune manifestations. RECENT FINDINGS: GOF mutations affecting STAT1 result in increased STAT tyrosine phosphorylation and secondarily increased response to STAT1-signaling cytokines, such as interferons...
September 13, 2017: Current Opinion in Pediatrics
https://www.readbyqxmd.com/read/28913747/primary-sj%C3%A3-gren-s-syndrome-extraglandular-manifestations-and-hydroxychloroquine-therapy
#12
J Demarchi, S Papasidero, M A Medina, D Klajn, R Chaparro Del Moral, O Rillo, V Martiré, G Crespo, A Secco, A Catalan Pellet, C Amitrano, C Crow, C Asnal, P Pucci, F Caeiro, N Benzanquen, J P Pirola, M Mayer, F Zazzetti, S Velez, J Barreira, N Tamborenea, L Santiago, L Raiti
The use of hydroxychloroquine (HCQ) in Primary Sjögren's Syndrome (pSS) has been assessed in different studies over the last years, with conflicting results regarding its efficacy in sicca syndrome and extraglandular manifestations (EGM). The goal of this study was to compare the incidence rate of EGM in pSS patients with and without HCQ therapy.We performed a multicenter retrospective study, including patients with pSS (European classification criteria) with at least 1 year of follow-up. Subjects with concomitant fibromyalgia, autoimmune hepatitis, primary biliary cirrhosis, and primary sclerosing cholangitis were excluded...
September 14, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28910817/the-lung-in-dysregulated-states-of-humoral-immunity
#13
Yurdagül Uzunhan, Florence Jeny, Marianne Kambouchner, Morgane Didier, Diane Bouvry, Hilario Nunes, Jean-François Bernaudin, Dominique Valeyre
In common variable immunodeficiency, lung manifestations are related to different mechanisms: recurrent pneumonias due to encapsulated bacteria responsible for diffuse bronchiectasis, diffuse infiltrative pneumonia with various patterns, and lymphomas, mostly B cell extranodal non-Hodgkin type. The diagnosis relies on significant serum Ig deficiency and the exclusion of any primary or secondary cause. Histopathology may be needed. Immunoglobulin (IgG) replacement is crucial to prevent infections and bronchiectasis...
September 15, 2017: Respiration; International Review of Thoracic Diseases
https://www.readbyqxmd.com/read/28910509/the-experience-of-palliative-care-service-provision-for-people-with-non-malignant-respiratory-disease-and-their-family-carers-an-all-ireland-qualitative-study
#14
Clare Mc Veigh, Joanne Reid, Philip Larkin, Sam Porter, Peter Hudson
AIM: To explore specialist and generalist palliative care provision for people with non-malignant respiratory disease, in rural and urban areas in the North and Republic of Ireland. BACKGROUND: Globally, palliative care is recommended as an appropriate healthcare option for people with advanced non- malignant lung disease. Yet, there is limited evidence regarding the integration of palliative care for this client group. DESIGN: Qualitative study...
September 14, 2017: Journal of Advanced Nursing
https://www.readbyqxmd.com/read/28898549/filamin-a-flna-mutation-a-newcomer-to-the-childhood-interstitial-lung-disease-child-classification
#15
Susan C Shelmerdine, Thomas Semple, Colin Wallis, Paul Aurora, Shahin Moledina, Michael T Ashworth, Catherine M Owens
AIM: Interstitial lung disease (ILD) in infants represents a rare and heterogenous group of disorders, distinct from those occurring in adults. In recent years a new entity within this category is being recognized, namely filamin A (FLNA) mutation related lung disease. Our aims are to describe the clinical and radiological course of patients with this disease entity to aid clinicians in the prognostic counseling and management of similar patients they may encounter. METHOD: A retrospective case note review was conducted of all patients treated at our institution (a specialist tertiary referral childrens' center) for genetically confirmed FLNA mutation related lung disease...
September 12, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28895301/early-leaflet-thrombosis-complicating-transcatheter-implantation-of-a-sapien-3-valve-in-a-native-right-ventricular-outflow-tract
#16
Mounir Riahi, Philipp Blanke, John Webb, Ronald G Carere
A 59-year-old female with Tetralogy of Fallot had a previous complete repair with RVOT patch enlargement. She developed subsequent severe symptomatic (NYHA III) pulmonary regurgitation with severe RV dilatation. She had a concomitant interstitial lung disease secondary to hypersensitivity pneumonitis that precluded her from cardiac surgery. After preprocedural assessment using computed tomography, echocardiography and invasive angiography we decided to implant a 29 mm Edwards Sapien 3 valve without pre-stenting...
September 12, 2017: Catheterization and Cardiovascular Interventions
https://www.readbyqxmd.com/read/28891101/the-interstitial-lung-disease-multidisciplinary-meeting-a-position-statement-from-the-thoracic-society-of-australia-and-new-zealand-and-the-lung-foundation-australia
#17
Jyotika D Prasad, Annabelle Mahar, Jane Bleasel, Samantha J Ellis, Daniel C Chambers, Fiona Lake, Peter M A Hopkins, Tamera J Corte, Heather Allan, Ian N Glaspole
Interstitial lung diseases (ILD) are a diverse group of pulmonary diseases for which accurate diagnosis is critical for optimal treatment outcomes. Diagnosis of ILD can be challenging and a multidisciplinary approach is recommended in international guidelines. The purpose of this position paper is to review the evidence for the use of the multidisciplinary meeting (MDM) in ILD and suggest an approach to its governance and constitution, in an attempt to provide a standard methodology that could be applied across Australia and New Zealand...
September 10, 2017: Respirology: Official Journal of the Asian Pacific Society of Respirology
https://www.readbyqxmd.com/read/28888691/exercise-training-improves-exercise-capacity-and-quality-of-life-in-people-with-interstitial-lung-disease-synopsis
#18
Tania Janaudis-Ferreira
No abstract text is available yet for this article.
September 6, 2017: Journal of Physiotherapy
https://www.readbyqxmd.com/read/28888689/exercise-training-improves-exercise-capacity-and-quality-of-life-in-people-with-interstitial-lung-disease-commentary
#19
Lisa Wickerson
No abstract text is available yet for this article.
September 6, 2017: Journal of Physiotherapy
https://www.readbyqxmd.com/read/28886193/estimating-the-incidence-of-interstitial-lung-diseases-in-the-cree-of-eeyou-istchee-northern-qu%C3%A3-bec
#20
Matthieu Storme, Alexandre Semionov, Deborah Assayag, Michael Lefson, Darlene Kitty, David Dannenbaum, Jill Torrie, Pierre Lejeune, Elizabeth Robinson, Faiz Ahmad Khan
BACKGROUND: Little is known about the epidemiology of interstitial lung disease (ILD) amongst Canada's Indigenous populations. Clinicians working in Eeyou Istchee (the Cree territory of the James Bay region of Québec, population 17, 956) suspected that ILD was more common in this area. We sought to identify all prevalent and incident cases of ILD in Eeyou Istchee between 2006 and 2013, to describe characteristics of affected patients, distribution of subtypes, and estimate disease incidence...
2017: PloS One
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