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Idiopathic interstitial pneumonias

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https://www.readbyqxmd.com/read/28919004/stereotactic-body-radiotherapy-for-lung-cancer-patients-with-idiopathic-interstitial-pneumonias
#1
Yuichiro Tsurugai, Atsuya Takeda, Naoko Sanuki, Tatsuji Enomoto, Takeshi Kaneko, Yu Hara, Tomikazu Mizuno, Noriyuki Saeki, Yousuke Aoki, Yohei Oku, Takeshi Akiba, Etsuo Kunieda
PURPOSE: To compare toxicity and survival after stereotactic body radiotherapy (SBRT) between lung cancer patients with or without idiopathic interstitial pneumonias (IIPs), and to investigate the potential value of SBRT for the patients. METHODS: Among lung cancer patients receiving SBRT between 2005 and 2016, we evaluated those treated with a total dose of 40-60Gy in five fractions with curative intent who either were staged as cT1-4N0M0 or experienced postoperative isolated local recurrence...
September 14, 2017: Radiotherapy and Oncology: Journal of the European Society for Therapeutic Radiology and Oncology
https://www.readbyqxmd.com/read/28886713/increased-activated-regulatory-t-cells-proportion-correlate-with-the-severity-of-idiopathic-pulmonary-fibrosis
#2
Ziliang Hou, Qiao Ye, Meihua Qiu, Yu Hao, Junyan Han, Hui Zeng
BACKGROUND: Regulatory T cells (Tregs) are crucial in maintaining immune tolerance and immune homeostasis, but their role in idiopathic pulmonary fibrosis (IPF) is unclear. This study was designed to explore the role of Tregs in IPF. METHODS: Percentages of Tregs and their subpopulations in peripheral blood (PB) and bronchoalveolar lavage (BAL) samples were determined by flow cytometry in 29 patients with IPF, 19 patients with primary Sjögren's syndrome-related interstitial pneumonia (pSS-IP), and 23 healthy controls (HCs)...
September 8, 2017: Respiratory Research
https://www.readbyqxmd.com/read/28886193/estimating-the-incidence-of-interstitial-lung-diseases-in-the-cree-of-eeyou-istchee-northern-qu%C3%A3-bec
#3
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
https://www.readbyqxmd.com/read/28877715/exploring-efficacy-and-safety-of-oral-pirfenidone-for-progressive-non-ipf-lung-fibrosis-relief-a-randomized-double-blind-placebo-controlled-parallel-group-multi-center-phase-ii-trial
#4
Jürgen Behr, Petra Neuser, Antje Prasse, Michael Kreuter, Klaus Rabe, Carmen Schade-Brittinger, Jasmin Wagner, Andreas Günther
BACKGROUND: Pirfenidone is currently approved in the EU for the treatment of mild to moderate idiopathic pulmonary fibrosis (IPF) and offers a beneficial risk-benefit profile. However, there are several other, progressive fibrotic lung diseases, in which conventional anti-inflammatory therapy is not sufficiently effective and antifibrotic therapies may offer a novel treatment option. METHODS/DESIGN: We designed a study protocol for inclusion of patients with progressive fibrotic lung disease despite conventional anti-inflammatory therapy (EudraCT 2014-000861-32)...
September 6, 2017: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/28877057/pleuroparenchymal-fibroelastosis-a-review-of-histopathologic-features-and-the-relationship-between-histologic-parameters-and-survival
#5
Reena Khiroya, Claudio Macaluso, Maria A Montero, Athol U Wells, Felix Chua, Maria Kokosi, Toby M Maher, Anand Devaraj, Alexandra Rice, Elisabetta A Renzoni, Andrew G Nicholson
Pleuroparenchymal fibroelastosis (PPFE) is now a defined clinicopathologic entity in the updated 2013 ATS/ERS classification of idiopathic interstitial pneumonias (IIPs), which has led to a significant increase in cases being diagnosed at our institution. We have therefore reviewed 43 PPFE cases (58 biopsies in total) to assess whether any clinical or histopathologic features provide prognostic information. A semiquantatitive grading system was used to assess extent of fibroblastic foci, intra-alveolar fibroelastosis, visceral pleural fibrosis, chronic inflammation in areas of fibrosis, vascular fibrointimal thickening, and presence of granulomas...
September 4, 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28876508/the-effects-of-pirfenidone-in-patients-with-an-acute-exacerbation-of-interstitial-pneumonia
#6
Takuma Matsumura, Kenji Tsushima, Mitsuhiro Abe, Kenichi Suzuki, Kazutaka Yamagishi, Akane Matsumura, Yasunori Ichimura, Jun Ikari, Jiro Terada, Koichiro Tatsumi
Introdcution: The prognosis of patients with an acute exacerbation of interstitial pneumonia (AE-IP) is poor. Pirfenidone (PFD) reduces the disease progression in idiopathic pulmonary fibrosis. OBJECTIVES: The purpose of this study was evaluating whether the administration of PFD improved the outcomes of AE-IP. METHODS: We conducted a retrospective study of 31 patients with AE-IP who did not recover between 7 and 14 days after an initial treatment. Fourteen patients received PFD within 2 weeks (PFD group) of the AE, while 17 patients were treated without PFD (Non-PFD group)...
September 6, 2017: Clinical Respiratory Journal
https://www.readbyqxmd.com/read/28865842/lung-fibrosis-associated-soluble-mediators-and-bronchoalveolar-lavage-from-idiopathic-pulmonary-fibrosis-patients-promote-the-expression-of-fibrogenic-factors-in-subepithelial-lung-myofibroblasts
#7
Evangelos Bouros, Eirini Filidou, Konstantinos Arvanitidis, Dimitrios Mikroulis, Pashalis Steiropoulos, George Bamias, Demosthenes Bouros, George Kolios
Idiopathic pulmonary fibrosis (IPF) is characterized by infiltration of inflammatory cells, excessive collagen production and accumulation of myofibroblasts. We explored the possible role of subepithelial lung myofibroblasts (SELMs) in the development of fibrosis in IPF. SELMs, isolated from surgical specimens of healthy lung tissue, were cultured with pro-inflammatory factors or bronchoalveolar lavage fluid (BALF) from patients with IPF or idiopathic non-specific interstitial pneumonia (iNSIP) and their fibrotic activity was assessed...
August 30, 2017: Pulmonary Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/28858871/unilateral-upper-lung-field-pulmonary-fibrosis-radiologically-consistent-with-pleuroparenchymal-fibroelastosis-after-thoracotomy-a-new-disease-entity-related-to-thoracotomy
#8
Akimasa Sekine, Hiroaki Satoh, Tae Iwasawa, Kentaro Matsui, Eriko Ikeya, Satoshi Ikeda, Hideaki Yamakawa, Ryo Okuda, Hideya Kitamura, Takeshi Shinohara, Tomohisa Baba, Shigeru Komatsu, Terufumi Kato, Eri Hagiwara, Takashi Ogura
BACKGROUND: Pleuroparenchymal fibroelastosis (PPFE) is a rare bilateral idiopathic interstitial pneumonia defined by pleural-parenchymal involvement. In clinical practice, we encountered patients with upper lung field pulmonary fibrosis (Upper-PF), which was radiologically consistent with PPFE, but apparently limited to the unilateral lung. OBJECTIVES: The purpose of the study was to clarify the clinical characteristics in those patients. METHODS: We examined the medical records of all the consecutive patients from 2012 to 2016 to see whether there were patients having unilateral Upper-PF...
September 1, 2017: Respiration; International Review of Thoracic Diseases
https://www.readbyqxmd.com/read/28852519/fatal-diffuse-alveolar-haemorrhage-mimicking-acute-exacerbation-in-idiopathic-pulmonary-fibrosis-treated-with-nintedanib
#9
Keishi Sugino, Yasuhiko Nakamura, Muneyuki Sekiya, Hiroshi Kobayashi, Kazutoshi Shibuya, Sakae Homma
A 75-year-old man was referred to our hospital with a 1-year history of persistent dry cough and progressive dyspnoea on exertion. He was treated with aspirin due to thrombosis of internal carotid artery. He was diagnosed with idiopathic pulmonary fibrosis (IPF)/usual interstitial pneumonia (UIP), and started on inhaled N-acetylcysteine therapy and pirfenidone. Since his clinical condition progressively deteriorated after 6 months, he was switched from pirfenidone to nintedanib. As a result, his general condition worsened rapidly...
November 2017: Respirology Case Reports
https://www.readbyqxmd.com/read/28842969/pleuroparenchymal-fibroelastosis-in-heterogeneous-clinical-conditions-clinicopathologic-analysis-of-7-cases
#10
Yoon Jin Cha, Joungho Han, Man Pyo Chung, Tae Jung Kim, Sumin Shin
BACKGROUND AND AIMS: Pleuroparenchymal fibroelastosis (PPFE) is classified as a rare idiopathic interstitial pneumonia, and its clinicopathologic features remain unclear. We sought to investigate the clinical, radiologic, and pathologic features of patients with histologically proven PPFE. METHODS: Seven patients who had histologic features of PPFE that were definitive or consistent with PPFE were included. Clinical data and radiologic findings of chest computed tomography were obtained...
August 26, 2017: Clinical Respiratory Journal
https://www.readbyqxmd.com/read/28838377/a-pilot-trial-of-nivolumab-treatment-for-advanced-non-small-cell-lung-cancer-patients-with-mild-idiopathic-interstitial-pneumonia
#11
Daichi Fujimoto, Takeshi Morimoto, Jiro Ito, Yuki Sato, Munehiro Ito, Shunsuke Teraoka, Kojiro Otsuka, Kazuma Nagata, Atsushi Nakagawa, Keisuke Tomii
INTRODUCTION: Nivolumab has demonstrated efficacy against metastatic non-small cell lung cancer (NSCLC). However, immune-related adverse events can occur, among which pneumonitis is relatively common. Lung cancer patients with idiopathic interstitial pneumonia (IIP) have a higher risk of pneumonitis associated with anticancer therapy. We hypothesized that the benefit of nivolumab may outweigh the risks of pneumonitis in patients with NSCLC who have mild IIP. We performed a pilot trial to evaluate the safety of nivolumab in NSCLC patients with mild IIP...
September 2017: Lung Cancer: Journal of the International Association for the Study of Lung Cancer
https://www.readbyqxmd.com/read/28821283/gene-expression-profiling-of-idiopathic-interstitial-pneumonias-iips-identification-of-potential-diagnostic-markers-and-therapeutic-targets
#12
Yasushi Horimasu, Nobuhisa Ishikawa, Masaya Taniwaki, Kakuhiro Yamaguchi, Kosuke Hamai, Hiroshi Iwamoto, Shinichiro Ohshimo, Hironobu Hamada, Noboru Hattori, Morihito Okada, Koji Arihiro, Yuji Ohtsuki, Nobuoki Kohno
BACKGROUND: Chronic fibrosing idiopathic interstitial pneumonia (IIP) is characterized by alveolar epithelial damage, activation of fibroblast proliferation, and loss of normal pulmonary architecture and function. This study aims to investigate the genetic backgrounds of IIP through gene expression profiling and pathway analysis, and to identify potential biomarkers that can aid in diagnosis and serve as novel therapeutic targets. METHODS: RNA extracted from lung specimens of 12 patients with chronic fibrosing IIP was profiled using Illumina Human WG-6 v3 BeadChips, and Ingenuity Pathway Analysis was performed to identify altered functional and canonical signaling pathways...
August 18, 2017: BMC Medical Genetics
https://www.readbyqxmd.com/read/28820502/oligonucleotides-targeting-periostin-ameliorates-pulmonary-fibrosis
#13
A Tomaru, T Kobayashi, J A Hinneh, P B Tonto, C N D' Alessandro-Gabazza, H Fujimoto, K Fujiwara, Y Takahashi, M Ohnishi, T Yasuma, K Nishihama, M Yoshino, K Takao, M Toda, T Totoki, Y Takei, K Yoshikawa, O Taguchi, E C Gabazza
Idiopathic pulmonary fibrosis (IPF) is a fatal disease with a median survival of 3 to 4 years after diagnosis. It is the most frequent form of a group of interstitial pneumonias of unknown etiology. Current available therapies prevent deterioration of lung function but no therapy has shown to improve survival. Periostin is a matricellular protein of the fasciclin 1 family. There is increased deposition of periostin in lung fibrotic tissues. Here, we evaluated whether small interfering RNA or antisense oligonucleotide against periostin inhibit lung fibrosis by direct administration into the lung by intranasal route...
August 18, 2017: Gene Therapy
https://www.readbyqxmd.com/read/28817393/increased-expiratory-computed-tomography-density-reveals-possible-abnormalities-in-radiologically-preserved-lung-parenchyma-in-idiopathic-pulmonary-fibrosis
#14
Valentina Petroulia, Manuela Funke, Pascal Zumstein, Sabina Berezowska, Lukas Ebner, Thomas Geiser, Nenad Torbica, Johannes Heverhagen, Alexander Poellinger
OBJECTIVES: Idiopathic pulmonary fibrosis (IPF) is a progressive lethal chronic lung disease with unclear pathogenesis. Radiological hallmark is the pattern of usual interstitial pneumonia accentuated in peripheral and basal areas with otherwise preserved lung structure. One hypothesis is that alveolar collapse and consequent induration lead to fibrotic transformation of lung tissue. The aim of the study was to investigate normal-appearing tissue during expiration for signs of collapsibility and differences from other diseases or controls...
August 16, 2017: Investigative Radiology
https://www.readbyqxmd.com/read/28800589/prognostic-factors-and-outcomes-in-japanese-lung-transplant-candidates-with-interstitial-lung-disease
#15
Kohei Ikezoe, Tomohiro Handa, Kiminobu Tanizawa, Toyofumi F Chen-Yoshikawa, Takeshi Kubo, Akihiro Aoyama, Hideki Motoyama, Kyoko Hijiya, Shinsaku Tokuda, Yoshinari Nakatsuka, Yuko Yamamoto, Ayako Oshima, Shin-Ichi Harashima, Sonoko Nagai, Toyohiro Hirai, Hiroshi Date, Kazuo Chin
OBJECTIVE: Young patients with advanced interstitial lung disease (ILD) are potential candidates for cadaveric lung transplantation. This study aimed to examine clinical features, outcomes, and prognostic factors in Japanese ILD patients awaiting lung transplantation. METHODS: We investigated the clinical features and outcomes of 77 consecutive candidates with ILD who were referred to Kyoto University Hospital and subsequently actively listed for lung transplant in the Japan Organ Transplant Network between 2010 and 2014...
2017: PloS One
https://www.readbyqxmd.com/read/28796717/utility-of-flexible-bronchoscopic-cryobiopsy-for-diagnosis-of-diffuse-parenchymal-lung-diseases
#16
Robert J Lentz, Trevor M Taylor, Jonathan A Kropski, Kim L Sandler, Joyce E Johnson, Timothy S Blackwell, Fabien Maldonado, Otis B Rickman
BACKGROUND: Initial reports of transbronchial cryobiopsy for diffuse parenchymal lung disease (DPLD) suggest the diagnostic yield approaches that of surgical lung biopsy (SLB) with an excellent safety profile. Centers performing cryobiopsy differ significantly in procedure technique; an optimal technique minimizing complications but still capable of diagnosing a wide range of DPLDs has not been established. We evaluated our practice of flexible bronchoscopic cryobiopsy in a primarily outpatient setting for patients who required a tissue diagnosis for DPLD of uncertain etiology...
August 7, 2017: Journal of Bronchology & Interventional Pulmonology
https://www.readbyqxmd.com/read/28777013/investigational-drugs-for-idiopathic-pulmonary-fibrosis
#17
REVIEW
Francesco Varone, Giuliano Montemurro, Francesco Macagno, Mariarosaria Calvello, Emanuele Conte, Enrica Intini, Bruno Iovene, Paolo Maria Leone, Pier-Valerio Mari, Luca Richeldi
IPF is a specific form of chronic fibrosing interstitial pneumonia of unknown cause, characterized by progressive worsening in lung function and an unfavorable prognosis. Current concepts on IPF pathogenesis are based on a dysregulated wound healing response, leading to an over production of extracellular matrix. Based on recent research however, several other mechanisms are now proposed as potential targets for novel therapeutic strategies. Areas covered: This review analyzes the current investigational strategies targeting extracellular matrix deposition, tyrosine-kinase antagonism, immune and autoimmune response, and cell-based therapy...
September 2017: Expert Opinion on Investigational Drugs
https://www.readbyqxmd.com/read/28775045/prevalence-and-incidence-of-interstitial-lung-diseases-in-a-multi-ethnic-county-of-greater-paris
#18
Boris Duchemann, Isabella Annesi-Maesano, Camille Jacobe de Naurois, Shreosi Sanyal, Pierre-Yves Brillet, Michel Brauner, Marianne Kambouchner, Sophie Huynh, Jean Marc Naccache, Raphael Borie, Jacques Piquet, Arsène Mekinian, Jerôme Virally, Yurdagul Uzunhan, Jacques Cadranel, Bruno Crestani, Olivier Fain, Francois Lhote, Robin Dhote, Nathalie Saidenberg-Kermanac'h, Paul-André Rosental, Dominique Valeyre, Hilario Nunes
The objective of the study was to estimate the prevalence and incidence of interstitial lung diseases (ILDs) in Seine-Saint-Denis, a multi-ethnic county of Greater Paris, France.Patients with ILDs were identified between January and December 2012 by using several sources; all potentially involved medical specialists from public and private hospitals, community-based pulmonologists and general practitioners, and the Social Security system. Diagnoses were validated centrally by an expert multidisciplinary discussion...
August 2017: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/28774636/sleep-as-a-new-target-for-improving-outcomes-in-idiopathic-pulmonary-fibrosis
#19
REVIEW
Charalampos Mermigkis, Izolde Bouloukaki, Sophia E Schiza
Idiopathic pulmonary fibrosis (IPF) is the most common type of interstitial pneumonia but remains a disease with a poor outcome. Two drugs, pirfenidone and nintedanib, have shown promising results at stalling disease progression; however, the interplay of sleep disruption or sleep disorders overall and in relation to medication effectiveness remains understudied. In the past, there was limited interest in the role of sleep in patients with IPF. Treating physicians tended to address only the daily disabling symptoms while disregarding the possible significant role of sleep alterations or coexisting sleep disorders...
August 1, 2017: Chest
https://www.readbyqxmd.com/read/28770888/spectrum-of-interstitial-lung-disease-from-a-tertiary-care-hospital-in-karachi
#20
Ali Bin Sarwar Zubairi, Maryam Hassan, Talha Shahzad, Sajjad Sarwar, Aamir Abbas, Huzaifa Ahmad, Muhammad Irfan
OBJECTIVE: To determine the clinical features and patterns of interstitial lung disease. METHODS: This retrospective study was conducted at the Aga Khan University Hospital, Karachi, and comprised record of patients diagnosed with interstitial lung disease from January 2005 to December 2015. All patients aged 16 years and above diagnosed with interstitial lung disease on the basis of clinical features, radiological features on high-resolution computed tomography of the chest, and lung biopsies were included...
July 2017: JPMA. the Journal of the Pakistan Medical Association
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