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Idiopathic pulmonary disease

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https://www.readbyqxmd.com/read/28743305/prognosis-and-longitudinal-changes-of-physical-activity-in-idiopathic-pulmonary-fibrosis
#1
Thomas Bahmer, Anne-Marie Kirsten, Benjamin Waschki, Klaus F Rabe, Helgo Magnussen, Detlef Kirsten, Marco Gramm, Simone Hummler, Eva Brunnemer, Michael Kreuter, Henrik Watz
BACKGROUND: Physical activity (PA) is associated with disease severity in idiopathic pulmonary fibrosis (IPF), but longitudinal studies evaluating its prognostic value and changes over time are lacking. METHODS: We measured PA (steps per day, SPD) in a cohort of 46 IPF-patients (mean age, 67 years; mean FVC, 76.1%pred.) by accelerometry at baseline, recorded survival status during 3 years follow-up and repeated measurements in survivors. We compared the prognostic value of PA to established mortality predictors including lung function (FVC, DLCO) and 6-min walking-distance (6MWD)...
July 25, 2017: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/28737730/sildenafil-in-infants-and-children
#2
REVIEW
Larisa Simonca, Robert Tulloh
Pulmonary arterial hypertension (PAH) management has been transformed in recent times with the advent of cheap and effective diagnostic tools and therapy. Sildenafil, a phosphodiesterase-V inhibitor, has been at the centre of this treatment, and its success in treating PAH has led to its widespread uptake in adult and paediatric pulmonary hypertension (PH), as a first line treatment choice. This might apply to persistent pulmonary hypertension of the newborn (PPHN) or bronchopulmonary dysplasia, as well as to more complex diseases, such as idiopathic pulmonary hypertension...
July 24, 2017: Children
https://www.readbyqxmd.com/read/28732835/idiopathic-pulmonary-fibrosis-clinical-behavior-and-aging-associated-comorbidities
#3
REVIEW
Ivette Buendía-Roldán, Mayra Mejía, Carmen Navarro, Moisés Selman
Idiopathic pulmonary fibrosis (IPF) is a progressive, irreversible and usually lethal lung disease of unknown etiology. Once considered as a relatively homogeneous, slowly progressive disease, is now recognized that the clinical behavior shows substantial heterogeneity, including an accelerated variant, and the presence of acute exacerbations. In addition, since IPF largely affects individuals over 60 years of age, the patients are at increased risk of several comorbidities that in turn have a remarkable clinical impact on the disease and increases mortality rate...
August 2017: Respiratory Medicine
https://www.readbyqxmd.com/read/28732832/current-approaches-to-the-management-of-idiopathic-pulmonary-fibrosis
#4
REVIEW
Ganesh Raghu, Luca Richeldi
Idiopathic pulmonary fibrosis (IPF) is a progressive and ultimately fatal lung disease associated with dyspnoea, cough and impaired quality of life. Currently, the aims of patient care are to improve outcomes for patients by slowing the progression of the disease, extending life, and improving quality of life. A prompt, accurate diagnosis is important to enable patients to receive treatment early in the course of the disease and to be considered for lung transplantation. Two anti-fibrotic drugs, nintedanib and pirfenidone, have been shown to reduce decline in lung function in patients with IPF...
August 2017: Respiratory Medicine
https://www.readbyqxmd.com/read/28732823/correlation-of-pulmonary-function-and-usual-interstitial-pneumonia-computed-tomography-patterns-in-idiopathic-pulmonary-fibrosis
#5
Antonella Arcadu, Suzanne C Byrne, Pietro Pirina, Thomas E Hartman, Brian J Bartholmai, Teng Moua
BACKGROUND: Little is known about presenting 'inconsistent' or 'possible' usual interstitial pneumonia (UIP) computed tomography (CT) patterns advancing to 'consistent' UIP as disease progresses in idiopathic pulmonary fibrosis (IPF). We hypothesized that if 'consistent' UIP represented more advanced disease, such a pattern on presentation should also correlate with more severe pulmonary function test (PFT) abnormalities. MATERIAL AND METHODS: Consecutive IPF patients (2005-2013) diagnosed by international criteria with baseline PFT and CT were included...
August 2017: Respiratory Medicine
https://www.readbyqxmd.com/read/28731277/tnf-%C3%AE-induced-nf-%C3%AE%C2%BAb-activation-promotes-myofibroblast-differentiation-of-lr-mscs-and-exacerbates-bleomycin-induced-pulmonary-fibrosis
#6
Jiwei Hou, Tan Ma, Honghui Cao, Yabing Chen, Cong Wang, Xiang Chen, Zou Xiang, Xiaodong Han
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, and irreversible lung disease of unknown cause. It has been reported that both lung resident mesenchymal stem cells (LR-MSCs) and tumor necrosis factor-α (TNF-α) play important roles in the development of pulmonary fibrosis. However, the underlying connections between LR-MSCs and TNF-α in the pathogenesis of pulmonary fibrosis are still elusive. In this study, we found that the pro-inflammatory cytokine TNF-α and the transcription factor nuclear factor kappa B (NF-κB) p65 subunit were both upregulated in bleomycin-induced fibrotic lung tissue...
July 21, 2017: Journal of Cellular Physiology
https://www.readbyqxmd.com/read/28728146/chronic-kidney-disease-predicts-survival-in-patients-with-idiopathic-pulmonary-fibrosis
#7
Kohei Ikezoe, Tomohiro Handa, Kiminobu Tanizawa, Hideki Yokoi, Takeshi Kubo, Kensaku Aihara, Akihiko Sokai, Yoshinari Nakatsuka, Seishu Hashimoto, Kiyoshi Uemasu, Susumu Sato, Shigeo Muro, Sonoko Nagai, Motoko Yanagita, Kazuo Chin, Toyohiro Hirai, Yoshio Taguchi, Michiaki Mishima
BACKGROUND: The prevalence of chronic kidney disease (CKD) increases with age as with idiopathic pulmonary fibrosis (IPF). OBJECTIVES: We assessed the prevalence of CKD (stages 3-5) and investigated the relationship of CKD to clinical features and outcomes in patients with IPF. METHODS: This study comprised 123 patients with IPF; 61 subjects with chronic obstructive pulmonary disease (COPD), which was reportedly associated with CKD, were also enrolled as a disease control...
July 21, 2017: Respiration; International Review of Thoracic Diseases
https://www.readbyqxmd.com/read/28722352/serpine-1-induces-alveolar-type-ii-cell-senescence-through-activating-p53-p21-rb-pathway-in-fibrotic-lung-disease
#8
Chunsun Jiang, Gang Liu, Tracy Luckhardt, Veena Antony, Yong Zhou, A Brent Carter, Victor J Thannickal, Rui-Ming Liu
Senescence of alveolar type 2 (ATII) cells, progenitors of the alveolar epithelium, is implicated in the pathogeneses of idiopathic pulmonary fibrosis (IPF), an aging-related progressive fatal lung disorder with unknown etiology. The mechanism underlying ATII cell senescence in fibrotic lung diseases, however, remains poorly understood. In this study, we report that ATII cells in IPF lungs express higher levels of serpine 1, also known as plasminogen activator inhibitor 1 (PAI-1), and cell senescence markers p21 and p16, compared to ATII cells in control lungs...
July 19, 2017: Aging Cell
https://www.readbyqxmd.com/read/28717640/lung-microbiome-and-host-immune-tone-in-subjects-with-idiopathic-pulmonary-fibrosis-treated-with-inhaled-interferon-%C3%AE
#9
Jing Wang, Melissa Lesko, Michelle H Badri, Bianca C Kapoor, Benjamin G Wu, Yonghua Li, Gerald C Smaldone, Richard Bonneau, Zachary D Kurtz, Rany Condos, Leopoldo N Segal
Therapies targeting inflammation reveal inconsistent results in idiopathic pulmonary fibrosis (IPF). Aerosolised interferon (IFN)-γ has been proposed as a novel therapy. Changes in the host airway microbiome are associated with the inflammatory milieu and may be associated with disease progression. Here, we evaluate whether treatment with aerosolised IFN-γ in IPF impacts either the lower airway microbiome or the host immune phenotype. Patients with IPF who enrolled in an aerosolised IFN-γ trial underwent bronchoscopy at baseline and after 6 months...
July 2017: ERJ Open Research
https://www.readbyqxmd.com/read/28709421/health-related-quality-of-life-in-patients-with-idiopathic-pulmonary-fibrosis-in-clinical-practice-insights-ipf-registry
#10
Michael Kreuter, Jeff Swigris, David Pittrow, Silke Geier, Jens Klotsche, Antje Prasse, Hubert Wirtz, Dirk Koschel, Stefan Andreas, Martin Claussen, Christian Grohé, Henrike Wilkens, Lars Hagmeyer, Dirk Skowasch, Joachim F Meyer, Joachim Kirschner, Sven Gläser, Felix J F Herth, Tobias Welte, Claus Neurohr, Martin Schwaiblmair, Matthias Held, Thomas Bahmer, Marion Frankenberger, Jürgen Behr
BACKGROUND: The INSIGHTS-IPF registry provides one of the largest data sets of clinical data and self-reported patient related outcomes including health related quality of life (QoL) on patients with idiopathic pulmonary fibrosis (IPF). We aimed to describe associations of various QoL instruments between each other and with patient characteristics at baseline. METHODS: Six hundred twenty-three IPF patients with available QoL data (St George's Respiratory Questionnaire SGRQ, UCSD Shortness-of-Breath Questionnaire SoB, EuroQol visual analogue scale and index EQ-5D, Well-being Index WHO-5) were analysed...
July 14, 2017: Respiratory Research
https://www.readbyqxmd.com/read/28706918/the-respiratory-microbiome-in-idiopathic-pulmonary-fibrosis
#11
REVIEW
Richard J Hewitt, Philip L Molyneaux
Idiopathic pulmonary fibrosis (IPF) is a progressive and fatal disease of unknown cause. Current evidence suggests that it arises in genetically susceptible individuals as a consequence of an aberrant wound-healing response following repetitive alveolar injury. Overt respiratory infection and immunosuppression carry a high mortality, while polymorphisms in genes related to epithelial integrity and host defence predispose to IPF. Recent advances in sequencing technologies have allowed the use of molecular microbial technologies to characterise the respiratory microbiota in patients with IPF...
June 2017: Annals of Translational Medicine
https://www.readbyqxmd.com/read/28705908/importance-of-kynurenine-in-pulmonary-hypertension
#12
Bence M Nagy, Chandran Nagaraj, Andreas Meinitzer, Neha Sharma, Rita Papp, Vasile Foris, Bahil Ghanim, Grazyna Kwapiszewska, Gabor Kovacs, Walter Klepetko, Thomas R Pieber, Harald Mangge, Horst Olschewski, Andrea Oslchewski
The tryptophan metabolite kynurenine is significantly increased in pulmonary arterial hyper-tension (PAH) patients, and it is a potent vasodilator of systemic arteries. Our aim was to in-vestigate the role of kynurenine in the pulmonary circulation. Serum tryptophan, kynurenine and kynurenic-acid levels were measured in 20 idiopathic PAH (IPAH) patients, 20 healthy controls and 20 patients with chronic lung disease or meta-bolic syndrome without-PH. Laser-dissected pulmonary arteries from IPAH and control lungs were tested for the expression of IDO, the rate-limiting enzyme for the conversion from tryptophan to kynurenine...
July 13, 2017: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/28705305/clinical-characteristics-of-japanese-candidates-for-lung-transplant-for-interstitial-lung-disease-and-risk-factors-for-early-death-while-on-the-waiting-list
#13
Hisao Higo, Takeshi Kurosaki, Eiki Ichihara, Toshio Kubo, Kentaroh Miyoshi, Shinji Otani, Seiichiro Sugimoto, Masaomi Yamane, Nobuaki Miyahara, Katsuyuki Kiura, Shinichiro Miyoshi, Takahiro Oto
BACKGROUND: Lung transplants have produced very favorable outcomes for patients with interstitial lung disease (ILD) in Japan. However, because of the severe donor lung shortage, patients must wait approximately 2.5 years before they can undergo transplantation and many candidates die before allocation. We reveal the clinical characteristics of Japanese patients with ILD who are candidates for lung transplants and the risk factors for early death while on the waiting list. METHODS: We retrospectively reviewed the clinical data of patients registered in the Japan Organ Transplant Network from Okayama University Hospital who are candidates for cadaveric lung transplants for ILD between 1999 and 2015...
July 2017: Respiratory Investigation
https://www.readbyqxmd.com/read/28704913/treatment-of-connective-tissue-disease-associated-interstitial-lung-disease-the-pulmonologist-s-point-of-view
#14
REVIEW
So-My Koo, Soo-Taek Uh
Interstitial lung disease (ILD) occurs in 15% of patients with collagen vascular disease (CVD), referred to as connective tissue disease (CTD). Despite advances in management strategies, ILD continues to be a significant cause of mortality in patients with CVD-associated ILD (CTD-ILD). There is a lack of randomized, clinical trials assessing pharmacological agents for CTD-ILD, except in cases of ILD-associated systemic sclerosis (SSc). This may be due to the lack of CTD cases available, the difficulty of histological confirmation of ILD, and the various types of CTD and ILD...
July 2017: Korean Journal of Internal Medicine
https://www.readbyqxmd.com/read/28702966/possible-role-of-helicobacter-pylori-in-diseases-of-dermatological-interest
#15
C Guarneri, J Lotti, M Fioranelli, M G Roccia, T Lotti, F Guarneri
Helicobacter pylori is a gram-negative, flagellate, microaerophilic bacterium identified for the first time about 30 years ago, as a pathogenic factor of gastritis and peptic ulcer. Soon after, it was linked to several gastrointestinal and extra-gastrointestinal diseases (hematological, cardiovascular, neurological, pulmonary and ocular diseases, obesity, diabetes mellitus, growth retardation and extragastric MALT lymphoma). Association and possible cause-effect correlation with H. pylori infection were suggested in diseases of dermatological interest such as chronic urticaria, rosacea, Henoch-Schoenleins purpura, idiopathic thrombocytopenic purpura, cutaneous and oral lichen planus, atopic dermatitis, recurrent aphthous stomatitis, systemic sclerosis, psoriasis, Sjögrens syndrome, Behçet's disease, pruritus, alopecia areata, primary cutaneous marginal zone B-cell lymphomas, vitiligo, chronic prurigo, multiformis, prurigo nodularis, leukocytoclastic vasculitis, prurigo pigmentosa, eczema nummulare, primary cutaneous MALT-type lymphoma, sublamina densa-type linear IgA bullous dermatosis, Sweet's syndrome, cutaneous T-cell pseudolymphoma and pemphigus vulgaris...
July 13, 2017: Journal of Biological Regulators and Homeostatic Agents
https://www.readbyqxmd.com/read/28702718/single-center-experience-using-selexipag-in-a-pediatric-population
#16
Roberto Gallotti, Diana E Drogalis-Kim, Gary Satou, Juan Alejos
Pulmonary arterial hypertension (PAH) is a rare and progressive disorder. Current treatment in the pediatric population includes phosphodiesterase 5 inhibitors (PDE-5i), endothelin receptor antagonists (ERA), and both inhaled and intravenous prostacyclin pathway agonists. As of December 22, 2015 the first oral prostacyclin pathway agonist, selexipag (Uptravi(®)), was FDA approved in the US. In this case series, we discuss our single-center experience using selexipag in a pediatric population, composed of both patients with idiopathic PAH, and patients with congenital heart disease and PAH...
July 13, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28701304/hif1a-up-regulates-the-adora2b-receptor-on-alternatively-activated-macrophages-and-contributes-to-pulmonary-fibrosis
#17
Kemly Philip, Tingting Weng Mills, Jonathan Davies, Ning-Yuan Chen, Harry Karmouty-Quintana, Fayong Luo, Jose G Molina, Javier Amione-Guerra, Neeraj Sinha, Ashrith Guha, Holger K Eltzschig, Michael R Blackburn
Idiopathic pulmonary fibrosis (IPF) is a deadly chronic lung disease. Extracellular accumulation of adenosine and subsequent activation of the ADORA2B receptor play important roles in regulating inflammation and fibrosis in IPF. Additionally, alternatively activated macrophages (AAMs) expressing ADORA2B have been implicated in mediating adenosine's effects in IPF. Although hypoxic conditions are present in IPF, hypoxia's role as a direct modulator of macrophage phenotype and identification of factors that regulate ADORA2B expression on AAMs in IPF is not well understood...
July 12, 2017: FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology
https://www.readbyqxmd.com/read/28701252/idiopathic-pulmonary-fibrosis-a-case-discussion
#18
Meghan K Felton, Brian Bautista, Lee E Morrow, Mark Malesker
OBJECTIVE: The purpose of this report is to describe the case of a 68-year-old man who was treated for idiopathic pulmonary fibrosis (IPF), a chronic and fatal lung disease that is characterized by progressive deterioration of pulmonary function. He was initially prescribed pirfenidone and developed significant gastric distress. The treatment was transitioned to nintedanib. This article will provide the pharmacist with a therapeutic overview of IPF, as well as review the unique process involved with drug acquisition, dosing, patient education, and monitoring of pirfenidone and nintedanib...
July 1, 2017: Consultant Pharmacist: the Journal of the American Society of Consultant Pharmacists
https://www.readbyqxmd.com/read/28700255/disease-progression-in-idiopathic-pulmonary-fibrosis-fvc-is-not-enough
#19
Jürgen Behr
No abstract text is available yet for this article.
July 12, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28690220/-investigation-and-analysis-for-current-situation-and-pathogenesis-relevant-to-pulmonary-hypertension
#20
Si Lei, Doudou Tang, Nianru Xu, Shangjie Wu
To investigate the demographic characteristics and the causes for pulmonary hypertension (PH) in adult patients.
 Methods: A total of 2 508 adult patients diagnosed as PH, who came from the Second Xiangya Hospital of Central South University from January 2010 to December 2014, were retrospectively investigated. All subjects underwent the clinical diagnosis, or the echocardiographic diagnosis, or thetraditional hemodynamic criteria by right heart catheterization (RHC). The patient's data including hospital numbers, gender, ages, primary diseases, etc, are collected and analyzed...
June 28, 2017: Zhong Nan da Xue Xue Bao. Yi Xue Ban, Journal of Central South University. Medical Sciences
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