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interstitial pulmonary fibrosis

Baptiste Chéhère, Valérie Bougault, Cécile Chenivesse, Jean-Marie Grosbois, Benoit Wallaert
BACKGROUND: Pulmonary rehabilitation (PR) improves performance in the 6-min walk test (6MWT) in a subset of patients with fibrotic idiopathic interstitial pneumonia (f-IIP); however, a large proportion of patients do not respond to PR. AIM: To investigate the effects of a PR program on cardiorespiratory responses during a 6MWT and to identify the characteristics of patients who do not show improved performance after PR. DESIGN: An observational study...
June 14, 2018: European Journal of Physical and Rehabilitation Medicine
Estrella Fernández Fabrellas, Ricardo Peris Sánchez, Cristina Sabater Abad, Gustavo Juan Samper
Idiopathic pulmonary fibrosis (IPF), a devastating progressive interstitial lung disease (ILD) with no known cause, is the most common and deadly of the idiopathic interstitial pneumonias. With a median survival of 3⁻5 years following diagnosis, IPF is characterized by a progressive decline in lung function and quality of life in most patients. Prognostic factors recognized classically that influence mortality include functional, clinical and radiological parameters. However, in recent years, there has also been progress in the knowledge of genetic factors and biomarkers that may be useful in the prognostic evaluation of these patients...
June 14, 2018: Medical Sciences: Open Access Journal
Hiroshi Oiwa, Katsuhiro Ooi, Tetsu Oyama, Eiji Sugyama
There are conflicting data to date on the causal relationship between idiopathic interstitial pneumonia and antineutrophil cytoplasmic antibody (ANCA): (1) myeloperoxidase-ANCA may play a pathogenetic role in pulmonary fibrosis, or (2) pulmonary fibrosis may induce production of the antibody. In this article, we describe a case of an elderly female patient with microscopic polyangiitis that developed after positive conversion of myeloperoxidase-ANCA during the course of idiopathic interstitial pneumonia. Our experience as well as similar case reports may suggest that idiopathic interstitial pneumonia may induce production of ANCAs and even ANCA-associated vasculitis...
March 2018: Archives of Rheumatology
Smriti Mishra, Mohammad I Shah, Malay Sarkar, Nimisha Asati, Chittaranjan Rout
Interstitial lung diseases (ILDs) are a diverse group of ∼200 acute and chronic pulmonary disorders that are characterized by variable amounts of inflammation, fibrosis and architectural distortion with substantial morbidity and mortality. Inaccurate and delayed diagnoses increase the risk, especially in developing countries. Studies have indicated the significant roles of genetic elements in ILDs pathogenesis. Therefore, the first genetic knowledge resource, ILDgenDB, has been developed with an objective to provide ILDs genetic data and their integrated analyses for the better understanding of disease pathogenesis and identification of diagnostics-based biomarkers...
January 1, 2018: Database: the Journal of Biological Databases and Curation
Laurens J De Sadeleer, Stijn E Verleden, Els De Dycker, Jonas Yserbyt, Johny A Verschakelen, Eric K Verbeken, Benoit Nemery, Geert M Verleden, Frederik Hermans, Bart M Vanaudenaerde, Wim A Wuyts
BACKGROUND AND OBJECTIVE: Although idiopathic pulmonary fibrosis (IPF) patients experience a worse survival compared with chronic hypersensitivity pneumonitis (CHP), organic dust exposure is a known risk factor for both IPF and CHP. METHODS: We divided patients diagnosed with IPF, based on their exposure to moulds/birds (absent: group A; present: group B). We retrospectively compared pulmonary function and survival between groups A and B, and a separate CHP cohort (group C)...
June 13, 2018: Respirology: Official Journal of the Asian Pacific Society of Respirology
Amy Dressen, Alexander R Abbas, Christopher Cabanski, Janina Reeder, Thirumalai R Ramalingam, Margaret Neighbors, Tushar R Bhangale, Matthew J Brauer, Julie Hunkapiller, Jens Reeder, Kiran Mukhyala, Karen Cuenco, Jennifer Tom, Amy Cowgill, Jan Vogel, William F Forrest, Harold R Collard, Paul J Wolters, Jonathan A Kropski, Lisa H Lancaster, Timothy S Blackwell, Joseph R Arron, Brian L Yaspan
BACKGROUND: Idiopathic pulmonary fibrosis (IPF) risk has a strong genetic component. Studies have implicated variations at several loci, including TERT, surfactant genes, and a single nucleotide polymorphism at chr11p15 (rs35705950) in the intergenic region between TOLLIP and MUC5B. Patients with IPF who have risk alleles at rs35705950 have longer survival from the time of IPF diagnosis than do patients homozygous for the non-risk allele, whereas patients with shorter telomeres have shorter survival times...
June 8, 2018: Lancet Respiratory Medicine
Junji Uchino, Akira Nakao, Nobuyo Tamiya, Yoshiko Kaneko, Tadaaki Yamada, Kenichi Yoshimura, Masaki Fujita, Koichi Takayama
BACKGROUND: Advances in epidermal growth factor receptor-tyrosine kinase inhibitor (EGFR-TKI) treatment led to research on the mechanism of the resistance have revealed that an occurrence of T790M gene mutation generated in exon 20 of the EGFR gene is associated with approximately 50% to 60% of observed resistance. Osimertinib, a 3rd-generation EGFR-TKI, has been shown to be effective against both EGFR tyrosine kinase inhibitor-sensitizing and T790M resistance mutations. In this study, we prospectively investigate the efficacy and safety of osimertinib in elderly patients aged ≥75 years, with ineffective prior EGFR-TKI treatment or with recurrence of EGFR-TKI mutation-positive or T790M mutation-positive nonsmall-cell lung cancer...
June 2018: Medicine (Baltimore)
Stefan Kopf, Jan B Groener, Zoltan Kender, Thomas Fleming, Maik Brune, Christin Riedinger, Nadine Volk, Esther Herpel, Dominik Pesta, Julia Szendrödi, Mark O Wielpütz, Hans-Ulrich Kauczor, Hugo A Katus, Michael Kreuter, Peter P Nawroth
BACKGROUND: Diabetes mellitus is a significant comorbidity of interstitial lung disease (ILD). OBJECTIVES: The aim of this study was to investigate the incidence of restrictive lung disease (RLD) and ILD in patients with prediabetes and type 2 diabetes (T2D). METHODS: Forty-eight nondiabetics, 68 patients with prediabetes, 29 newly diagnosed T2D, and 110 patients with long-term T2D were examined for metabolic control, diabetes-related complications, breathlessness, and lung function...
June 6, 2018: Respiration; International Review of Thoracic Diseases
Maysoon Eldoma, Janet Pope
Systemic sclerosis (SSc) is a rare autoimmune connective tissue disease (CTD) characterized by vascular dysfunction, fibrosis, inflammation and autoantibodies. The pathophysiology of SSc is not completely understood, and many patients acquire organ or tissue damage despite advances in treatment. Current treatments target affected organs with modest improvements. Areas covered: This review evaluates several treatment strategies for SSc based on involved organs including skin, pulmonary, cardiac, renal, musculoskeletal and gastrointestinal...
June 6, 2018: Expert Review of Clinical Immunology
Noriyuki Enomoto, Yoshiyuki Oyama, Yasunori Enomoto, Masashi Mikamo, Masato Karayama, Hironao Hozumi, Yuzo Suzuki, Masato Kono, Kazuki Furuhashi, Tomoyuki Fujisawa, Naoki Inui, Yutaro Nakamura, Takafumi Suda
BACKGROUND: Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) has an extremely poor prognosis. The role of ferritin in the pathogenesis of AE-IPF is not well known while serum ferritin is a key prognostic indicator for patients with clinically amyopathic dermatomyositis with rapidly progressive interstitial pneumonia. OBJECTIVE: To elucidate the clinical importance of serum ferritin in patients with AE-IPF. METHODS: Thirty-seven patients (48 episodes), who were diagnosed with AE-IPF and treated at our hospital between 1997 and 2015, were retrospectively studied...
June 5, 2018: Clinical Respiratory Journal
Khadija Ayed, Raja Serairi Beji, Saloua Jameleddine
Idiopathic pulmonary fibrosis is the most common of the idiopathic interstitial pneumonias. The role of inflammation in idiopathic pulmonary fibrosis (IPF) is controversial. If inflammation were critical to the disease process, lung pathology would demonstrate an influx of inflammatory cells, and that the disease would respond to immunosuppression. The classic pathology does not display substantial inflammation, and no modulation of the immune system is effective as treatment. Recent data suggest that the pathophysiology of the disease is more a product of fibroblast dysfunction than of dysregulated inflammation...
August 2017: La Tunisie Médicale
Vanessa Martins, Walcy Rosolia Teodoro, Ana Paula Pereira Velosa, Priscila Andrade, Cecília Farhat, Alexandre Todorovic Fabro, Vera Luiza Capelozzi
Anomalous histoarchitecture with increased levels of type-V collagen (Col V) in lungs of human idiopathic pulmonary fibrosis (IPF) and bleomycin (BLM) airway-centered interstitial fibrosis suggest that this collagen can be a possible trigger involved in the pathogenesis of these diseases. Butylated hydroxytoluene (BHT) injury model revealed a distal involvement of lung parenchyma with significant endothelial injury and fibrotic response, contrasting with the BLM airway-centered insult. We undertook this study to analyze whether BHT alters distal airway/alveolar epithelial cells (AECs) and extracellular matrix (ECM) signaling involved in the initiation and progression of pulmonary fibrosis in a different pathway concerning overexpression of Col V...
June 5, 2018: Histology and Histopathology
Samuel Y Ash, Rola Harmouche, James C Ross, Alejandro A Diaz, Farbod N Rahaghi, Gonzalo Vegas Sanchez-Ferrero, Rachel K Putman, Gary M Hunninghake, Jorge Onieva Onieva, Fernando J Martinez, Augustine M Choi, Russell P Bowler, David A Lynch, Hiroto Hatabu, Surya P Bhatt, Mark T Dransfield, J Michael Wells, Ivan O Rosas, Raul San Jose Estepar, George R Washko
Purpose To determine if interstitial features at chest CT enhance the effect of emphysema on clinical disease severity in smokers without clinical pulmonary fibrosis. Materials and Methods In this retrospective cohort study, an objective CT analysis tool was used to measure interstitial features (reticular changes, honeycombing, centrilobular nodules, linear scar, nodular changes, subpleural lines, and ground-glass opacities) and emphysema in 8266 participants in a study of chronic obstructive pulmonary disease (COPD) called COPDGene (recruited between October 2006 and January 2011)...
June 5, 2018: Radiology
Li Li, Lin Cai, Linxin Zheng, Yujie Hu, Weifeng Yuan, Zhenhui Guo, Weifeng Li
Pulmonary fibrosis (PF) is a life-threatening interstitial lung disease. In this study, we tried to reveal the model of action between high-mobility group box 1 (HMGB1) and α -smooth muscle actin ( α -SMA) and the protective role of gefitinib in pulmonary fibrosis induced by the administration of bleomycin aerosol in mice. For the mechanism study, lung tissues were harvested two weeks after modeling to detect the coexpression of HMGB1 and α -SMA by immunohistochemistry and immunofluorescence staining. Protein-DNA interactions were analyzed using a pulldown assay to study the relationship between HMGB1 and α -SMA...
2018: Oxidative Medicine and Cellular Longevity
Jingyao Zhang, Ruixia Cui, Yang Feng, Weiman Gao, Jianbin Bi, Zeyu Li, Chang Liu
Background: Pulmonary fibrosis is a chronic progressive fibrosis interstitial lung disease that is characterized by inflammatory infiltration and fibrotic changes. 5-Hydroxytryptamine (5-HT) is an important regulatory factor in inflammation, immunomodulation, and fibrosis. The aim of this study was to investigate the role of 5-HT in bleomycin- (BLM-) induced pulmonary fibrosis through wild-type C57BL/6 (WT) and TPH1 knockout (KO) mouse experiments. Methods: The mice were grouped as follows: WT control group, KO control group, WT BLM group, and KO BLM group...
2018: Mediators of Inflammation
Paolo Spagnolo, Joyce C Lee, Nicola Sverzellati, Giulio Rossi, Vincent Cottin
Interstitial lung disease (ILD) is an increasingly recognized complication of rheumatoid arthritis (RA) and is associated with significant morbidity and mortality. In addition, approximately one-third of patients have subclinical disease with varying degrees of functional impairment. While risk factors for RA-ILD are well established (e.g., older age, male gender, ever smoking history and seropositivity to rheumatoid factor and anti-cyclic citrullinated peptide antibodies) little is known about optimal disease assessment, treatment and monitoring, particularly in patients with progressive disease...
May 27, 2018: Arthritis & Rheumatology
Xiaoru Xia, Caijun Dai, Hua Yu, Xiaoying Huang, Ali Chen, Yingxia Tan, Liangxing Wang
Interstitial lung disease is the most common complication of systemic sclerosis (SSc) and is associated with a high rate of mortality. Due to the complex pathogenesis of SSc, the therapies currently available remain limited. In the present study, the effect of asiatic acid (AA) on SSc-associated pulmonary fibrosis (PF) and its association with the transforming growth factor-β1 (TGF-β1)/Smad2/3 signaling pathway were evaluated. A hypochlorous acid (HOCl)-induced model of SSc was used to evaluate the therapeutic effect of AA on PF in SSc, where AA was administered to SSc mice by gavage...
June 2018: Oncology Letters
Keishi Sugino, Takayuki Kabuki, Kazutoshi Shibuya, Sakae Homma
A 65-year-old woman with a 35-year history of limited cutaneous systemic scleroderma was admitted to our hospital complaining of a 3-month history of progressive dyspnoea on exertion. High-resolution CT images of the chest revealed diffuse reticular opacities and traction bronchiectasis predominantly in the bilateral lower lobes of the lung. Specimens obtained during video-assisted thoracic surgery were consistent with fibrocellular non-specific interstitial pneumonia and accompanied by accumulation of lymph follicles within areas of fibrosis...
May 26, 2018: BMJ Case Reports
Boglárka Lilla Szentes, Michael Kreuter, Thomas Bahmer, Surinder S Birring, Martin Claussen, Julia Waelscher, Reiner Leidl, Larissa Schwarzkopf
BACKGROUND: Patients with interstitial lung diseases (ILD) have impaired health-related quality of life (HRQL). Little is known about the applicability of the disease-specific King's Brief Interstitial Lung Disease questionnaire (K-BILD) and the generic EQ-5D-5L in a German setting. METHODS: We assessed disease-specific (K-BILD) and generic HRQL (EQ-5D experience based value set (EBVS) and Visual Analog Scale (VAS)) in 229 patients with different ILD subtypes in a longitudinal observational study (HILDA)...
May 25, 2018: Respiratory Research
Ganesh Raghu, Bernt van den Blink, Mark J Hamblin, A Whitney Brown, Jeffrey A Golden, Lawrence A Ho, Marlies S Wijsenbeek, Martina Vasakova, Alberto Pesci, Danielle E Antin-Ozerkis, Keith C Meyer, Michael Kreuter, Hugues Santin-Janin, Geert-Jan Mulder, Brian Bartholmai, Renu Gupta, Luca Richeldi
Importance: Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic lung disease with poor prognosis. Approved therapies do not halt disease progression. Objective: To determine the effect of recombinant human pentraxin 2 vs placebo on change from baseline to week 28 in mean forced vital capacity (FVC) percentage of predicted value. Design, Setting, and Participants: Phase 2, randomized, double-blind, placebo-controlled trial conducted at 18 sites in 7 countries of eligible patients with IPF (N = 117; aged 40-80 years; FVC ≥50% and ≤90% predicted; ratio of forced expiratory volume in the first second/FVC >0...
May 20, 2018: JAMA: the Journal of the American Medical Association
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