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idiopathic lung fibrosis

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https://www.readbyqxmd.com/read/28345383/pulmonary-fibrosis-part-i-epidemiology-pathogenesis-and-diagnosis
#1
Keith C Meyer
Many forms of interstitial lung disease (ILD) can progress to extensive fibrosis and respiratory failure. Idiopathic pulmonary fibrosis (IPF), which generally has a poor prognosis, has been thoroughly studied over the past two decades, and many important discoveries have been made that pertain to genetic predisposition, epidemiology, disease pathogenesis, diagnosis, and management. Additionally, non-IPF forms of ILD can have radiologic and histopathologic manifestations that mimic IPF, and making an accurate diagnosis is key to providing personalized medicine to patients with pulmonary fibrosis...
March 26, 2017: Expert Review of Respiratory Medicine
https://www.readbyqxmd.com/read/28345369/pulmonary-fibrosis-part-ii-state-of-the-art-patient-management
#2
Keith C Meyer
While many pharmacologic therapies for the treatment of idiopathic pulmonary fibrosis (IPF) have been evaluated via randomized, placebo-controlled clinical trials (RCTs) conducted over the past two decades, most therapies have been shown to be ineffective or even potentially harmful. However, a number of recently completed RCTs have shown significant efficacy for pirfenidone and nintedanib for the treatment of IPF. Areas Covered: This manuscript reviews recent advances in the management of IPF and other forms of fibrosing interstitial lung disease (ILD) with an emphasis on IPF...
March 26, 2017: Expert Review of Respiratory Medicine
https://www.readbyqxmd.com/read/28343230/idiopathic-pulmonary-fibrosis-in-switzerland-diagnosis-and-treatment
#3
Manuela Funke-Chambour, Andrea Azzola, Dan Adler, Constance Barazzone-Argiroffo, Christian Benden, Annette Boehler, Pierre-Olivier Bridevaux, Martin Brutsche, Christian F Clarenbach, Katrin Hostettler, Rebekka Kleiner-Finger, Laurent P Nicod, Paola M Soccal, Michael Tamm, Thomas Geiser, Romain Lazor
Idiopathic pulmonary fibrosis (IPF) is a severe progressive and irreversible lung disease. Novel antifibrotic drugs that slow disease progression are now available. However, many issues regarding patient management remain unanswered, such as the choice between available drugs, their use in particular subgroups and clinical situations, time of treatment onset, termination, combination or switch, or nonpharmacologic management. To guide Swiss respiratory physicians in this evolving field still characterized by numerous areas of uncertainty, the Swiss Working Group for interstitial and rare lung diseases of the Swiss Respiratory Society provides a position paper on the diagnosis and treatment of IPF...
March 25, 2017: Respiration; International Review of Thoracic Diseases
https://www.readbyqxmd.com/read/28341270/age-dependent-gender-disparities-in-post-lung-transplant-survival-among-patients-with-idiopathic-pulmonary-fibrosis
#4
Shahid I Sheikh, Don Hayes, Stephen E Kirkby, Joseph D Tobias, Dmitry Tumin
BACKGROUND: The influence of age and gender on survival after lung transplant in patients with idiopathic pulmonary fibrosis (IPF) is not well defined. METHODS: The United Network for Organ Sharing database was queried to identify IPF patients receiving lung transplant between 2005 and 2015. RESULTS: There were 6,677 patients receiving lung transplant between May 2015 and June 2015 who met the inclusion criteria, predominantly males (n = 4,769, 71%)...
February 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28340864/multi-dimensional-scores-to-predict-mortality-in-patients-with-idiopathic-pulmonary-fibrosis-undergoing-lung-transplantation-assessment
#5
Jolene H Fisher, Faris Al-Hejaili, Sonja Kandel, Alim Hirji, Shane Shapera, Marco Mura
BACKGROUND: The heterogeneous progression of idiopathic pulmonary fibrosis (IPF) makes prognostication difficult and contributes to high mortality on the waitlist for lung transplantation (LTx). Multi-dimensional scores (Composite Physiologic index [CPI], [Gender-Age-Physiology [GAP]; RIsk Stratification scorE [RISE]) demonstrated enhanced predictive power towards outcome in IPF. The lung allocation score (LAS) is a multi-dimensional tool commonly used to stratify patients assessed for LTx...
April 2017: Respiratory Medicine
https://www.readbyqxmd.com/read/28339528/the-utility-of-comprehensive-autoantibody-testing-to-differentiate-connective-tissue-disease-associated-and-idiopathic-interstitial-lung-disease-subgroup-cases
#6
Caroline V Cotton, Lisa G Spencer, Robert P New, Robert G Cooper
Interstitial lung disease (ILD) comprises many heterogeneous disease groups, the largest being CTD-associated and those labelled as idiopathic out of necessity. The mechanisms causing ILD are poorly understood, but most CTD- and idiopathic-ILD cases can respond to immunosuppression, clearly suggesting a pathological role for inflammation. By contrast, corticosteroid immunosuppression causes harm without benefit in the feared idiopathic pulmonary fibrosis, suggesting that inflammation plays little pathological role, and where ILD progresses rapidly to lethal outcome even with anti-fibrotic drug use...
October 20, 2016: Rheumatology
https://www.readbyqxmd.com/read/28337871/cytokine-modulation-in-patients-with-idiopathic-pulmonary-fibrosis-undergoing-treatment-with-steroids-immunosuppressants-and-ifn-%C3%AE-1b
#7
S Marinari, V De Iuliis, V Dadorante, S Colella, A Marino, A Nunziata, V Flati, M Caruso, A Pennelli, F De Benedetto, S Matera, S Capodifoglio, S Martinotti, S Caputi, E Toniato
Idiopathic pulmonary fibrosis (IPF) is a chronic lung disease of unknown etiology and pathogenic mechanisms. From an etiopathogenic point of view, alveolar macrophages play a key role in accumulation of fibroblasts and deposition of collagen and extracellular matrix by releasing specific cytokines and inflammatory mediators. IPF seems to be also associated with circulating fibrocytes, which might be involved with an abnormal pulmonary vascular repair and remodeling. Based on its hypothesized pathologic mechanisms, anti-inflammatory, anti-fibrotic and immunosuppressive therapies are often used...
January 2017: Journal of Biological Regulators and Homeostatic Agents
https://www.readbyqxmd.com/read/28337301/endoplasmic-reticulum-stress-a-new-wrestler-in-the-pathogenesis-of-idiopathic-pulmonary-fibrosis
#8
Lei Zhang, Yi Wang, Nuruliarizki Shinta Pandupuspitasari, Guorao Wu, Xudong Xiang, Quan Gong, Weining Xiong, Cong-Yi Wang, Ping Yang, Boxu Ren
Idiopathic pulmonary fibrosis (IPF) has attracted extensive attention for its unexplained progressive lung scarring, short median survival and its unresponsiveness to traditional therapies. Despite extensive studies, the mechanisms underlying IPF pathoetiologies, however, remain poorly understood. Recent advances delineated a potential function of endoplasmic reticulum (ER) stress in meeting the need of fibrotic response, which pinpointed a critical role for the unfolded protein response (UPR) pathways in IPF pathogenesis...
2017: American Journal of Translational Research
https://www.readbyqxmd.com/read/28335088/further-characterization-of-computed-tomographic-and-clinical-features-for-staging-and-prognosis-of-idiopathic-pulmonary-fibrosis-in-west-highland-white-terriers
#9
Florence Thierry, Ian Handel, Gawain Hammond, Lesley G King, Brendan M Corcoran, Tobias Schwarz
Idiopathic pulmonary fibrosis is an interstitial lung disease of unknown etiology resulting in progressive interstitial fibrosis, with a known predilection in West Highland white terriers. In humans, computed tomography (CT) is a standard method for providing diagnostic and prognostic information, and plays a major role in the idiopathic pulmonary fibrosis staging process. Objectives of this retrospective, analytical, cross-sectional study were to establish descriptive criteria for reporting CT findings and test correlations among CT, clinical findings and survival time in West Highland white terriers with idiopathic pulmonary fibrosis...
March 23, 2017: Veterinary Radiology & Ultrasound
https://www.readbyqxmd.com/read/28332341/risk-factors-for-acute-exacerbation-of-idiopathic-pulmonary-fibrosis-a-systematic-review-and-meta-analysis
#10
Meihua Qiu, Yuqing Chen, Qiao Ye
BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a chronic andprogressive fibrotic disease limited to the lungs.The course of disease varies widely, with some patients experiencing acute respiratory deterioration, a conditioncalled acute exacerbations of IPF (AE-IPF). The risk factors contributing to AE-IPF are unclear. This systematic review and meta-analysis investigatedthe risk factors for AE-IPF. METHODS: Studies ofrisk factors for AE-IPF were identified in Medline, EMBASE and Cochrane databases...
March 23, 2017: Clinical Respiratory Journal
https://www.readbyqxmd.com/read/28326312/a-comparison-of-published-multidimensional-indices-to-predict-outcome-in-idiopathic-pulmonary-fibrosis
#11
Charles Sharp, Huzaifa I Adamali, Ann B Millar
Idiopathic pulmonary fibrosis (IPF) has an unpredictable course and prognostic factors are incompletely understood. We aimed to identify prognostic factors, including multidimensional indices from a significant IPF cohort at the Bristol Interstitial Lung Disease Centre in the UK. Patients diagnosed with IPF between 2007 and 2014 were identified. Longitudinal pulmonary physiology and exercise testing results were collated, with all-cause mortality used as the primary outcome. Factors influencing overall, 12- and 24-month survival were identified using Cox proportional hazards modelling and receiver operating characteristic curve analysis...
January 2017: ERJ Open Research
https://www.readbyqxmd.com/read/28326180/are-physicians-in-primary-health-care-able-to-recognize-pulmonary-fibrosis
#12
Minna Purokivi, Ulla Hodgson, Marjukka Myllärniemi, Eija-Riitta Salomaa, Riitta Kaarteenaho
Background: The early diagnosis of idiopathic pulmonary fibrosis (IPF) has become increasingly important due to evolving treatment options. IPF patients experience a significant delay in receiving an accurate diagnosis, thus delayed access to tertiary care is associated with higher mortality independently from disease severity. Objective: The aims were to evaluate whether there had been a delay in the referral process, and to determine whether the referring doctors had suspected IPF or other interstitial lung disease (ILD) already during the time of referral...
2017: European Clinical Respiratory Journal
https://www.readbyqxmd.com/read/28321059/arterial-carboxyhemoglobin-measurement-is-useful-for-evaluating-pulmonary-inflammation-in-subjects-with-interstitial-lung-disease
#13
Yu Hara, Masaharu Shinkai, Soichiro Kanoh, Yuji Fujikura, Bruce K Rubin, Akihiko Kawana, Takeshi Kaneko
Objective The arterial concentration of carboxyhemoglobin (CO-Hb) in subjects with inflammatory pulmonary disease is higher than that in healthy individuals. We retrospectively analyzed the relationship between the CO-Hb concentration and established markers of disease severity in subjects with interstitial lung disease (ILD). Methods The CO-Hb concentration was measured in subjects with newly diagnosed or untreated ILD and the relationships between the CO-Hb concentration and the serum biomarker levels, lung function, high-resolution CT (HRCT) findings, and the uptake in gallium-67 ((67)Ga) scintigraphy were evaluated...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28320097/asiatic-acid-ameliorates-pulmonary-fibrosis-induced-by-bleomycin-blm-via-suppressing-pro-fibrotic-and-inflammatory-signaling-pathways
#14
Shu-Hong Dong, Yan-Wei Liu, Feng Wei, Hui-Zhen Tan, Zhi-Dong Han
Idiopathic pulmonary fibrosis is known as a life-threatening disease with high mortality and limited therapeutic strategies. In addition, the molecular mechanism by which pulmonary fibrosis developed is not fully understood. Asiatic acid (AA) is a triterpenoid, isolated from Centella asiatica, exhibiting efficient anti-inflammatory and anti-oxidative activities. In our study, we attempted to explore the effect of Asiatic acid on bleomycin (BLM)-induced pulmonary fibrosis in mice. The findings indicated that pre-treatment with Asiatic acid inhibited BLM-induced lung injury and fibrosis progression in mice...
March 17, 2017: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/28315487/xpln-is-modulated-by-hdac-inhibitors-and-negatively-regulates-sparc-expression-by-targeting-mtorc2-in-human-lung-fibroblasts
#15
Koichiro Kamio, Arata Azuma, Jiro Usuki, Kuniko Matsuda, Minoru Inomata, Nobuhiko Nishijima, Shioto Itakura, Hiroki Hayashi, Takeru Kashiwada, Nariaki Kokuho, Kenichiro Atsumi, Tomoyoshi Yamaguchi, Kazue Fujita, Yoshinobu Saito, Shinji Abe, Kaoru Kubota, Akihiko Gemma
Pathogenesis of idiopathic pulmonary fibrosis (IPF) remains unclear. Secreted protein acidic and rich in cysteine (SPARC) is a matricellular protein that participates in the assembly and turnover of the extracellular matrix, whose expression is regulated by transforming growth factor (TGF)-β1 through activation of mammalian target of rapamycin complex 2 (mTORC2). Exchange factor found in platelets, leukemic, and neuronal tissues (XPLN) is an endogenous inhibitor of mTORC2. However, whether XPLN modulates SPARC expression remains unknown...
March 14, 2017: Pulmonary Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/28303154/mesenchymal-stem-cell-administration-in-patients-with-chronic-obstructive-pulmonary-disease-state-of-the-science
#16
REVIEW
Shih-Lung Cheng, Ching-Hsiung Lin, Chao-Ling Yao
Patients with chronic obstructive pulmonary disease (COPD) have chronic, irreversible airway inflammation; currently, there is no effective or curative treatment and the main goals of COPD management are to mitigate symptoms and improve patients' quality of life. Stem cell based therapy offers a promising therapeutic approach that has shown potential in diverse degenerative lung diseases. Preclinical studies have demonstrated encouraging outcomes of mesenchymal stem/stromal cells (MSCs) therapy for lung disorders including emphysema, bronchopulmonary dysplasia, fibrosis, and acute respiratory distress syndrome...
2017: Stem Cells International
https://www.readbyqxmd.com/read/28300574/sex-steroid-receptor-expression-in-idiopathic-pulmonary-fibrosis
#17
Mitra Mehrad, Humberto E Trejo Bittar, Samuel A Yousem
Usual interstitial pneumonia (UIP) is characterized by progressive scarring of the lungs and is associated with high morbidity and mortality despite therapeutic interventions. Sex steroid receptors have been demonstrated to play an important role in chronic lung conditions; however, their significance is unknown in patients with UIP. We retrospectively reviewed 40 idiopathic UIP cases for the expression of hormonal receptors. Forty cases including 10 normal lung, 10 cryptogenic organizing pneumonia (COP), 10 idiopathic organizing diffuse alveolar damage (DAD), 7 hypersensitivity pneumonitis (HP) and 3 nonspecific interstitial pneumonitis (NSIP) served as controls...
March 11, 2017: Human Pathology
https://www.readbyqxmd.com/read/28300570/idiopathic-interstitial-pneumonia-associated-with-autoantibodies-a-large-case-series-followed-over-one-year
#18
Bridget F Collins, Charles F Spiekerman, Megan A Shaw, Lawrence A Ho, Jennifer Hayes, Carolyn A Spada, Caroline M Stamato, Ganesh Raghu
BACKGROUND: Some patients with autoimmune characteristics and idiopathic interstitial pneumonia (IIP), particularly usual interstitial pneumonia (UIP), do not fit neatly into the category of connective tissue disease associated interstitial lung disease (CTD-ILD), idiopathic pulmonary fibrosis (IPF) or recently proposed yet to be validated criteria for interstitial pneumonia with autoimmune features (IPAF). Outcomes of these patients are unknown. METHODS: Retrospective single center study; ANOVA analyses compared differences in mean change in forced vital capacity (FVC) and diffusion capacity (DLCO) over 1-year among 124 well defined patients (20 AI-ILD [positive autoantibodies with or without symptoms of CTD], 15 IPAF, 36 CTD-ILD, 53 "Lone-IPF" [patients with IPF without any autoantibodies]) RESULTS: 75% of patients with AI-ILD, 33% IPAF, and 33% CTD-ILD had UIP...
March 11, 2017: Chest
https://www.readbyqxmd.com/read/28298912/heterologous-matrix-metalloproteinase-gene-promoter-activity-allows-in-vivo-real-time-imaging-of-bleomycin-induced-lung-fibrosis-in-transiently-transgenized-mice
#19
Fabio Franco Stellari, Francesca Ruscitti, Daniela Pompilio, Francesca Ravanetti, Giulia Tebaldi, Francesca Macchi, Andrea Elizabeth Verna, Gino Villetti, Gaetano Donofrio
Idiopathic pulmonary fibrosis is a very common interstitial lung disease derived from chronic inflammatory insults, characterized by massive scar tissue deposition that causes the progressive loss of lung function and subsequent death for respiratory failure. Bleomycin is used as the standard agent to induce experimental pulmonary fibrosis in animal models for the study of its pathogenesis. However, to visualize the establishment of lung fibrosis after treatment, the animal sacrifice is necessary. Thus, the aim of this study was to avoid this limitation by using an innovative approach based on a double bleomycin treatment protocol, along with the in vivo images analysis of bleomycin-treated mice...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28297158/unclassifiable-interstitial-lung-diseases-clinical-characteristics-and-survival
#20
Charlotte Hyldgaard, Elisabeth Bendstrup, Athol U Wells, Ole Hilberg
BACKGROUND AND OBJECTIVE: Unclassifiable disease in chronic interstitial lung disease (ILD) is a common and challenging problem but has been insufficiently studied. The 2013 update of the international multidisciplinary classification of the idiopathic interstitial pneumonias presented a classification based on observed disease behaviour with the purpose of providing guidance to clinicians in the management of these patients. The aim of this study was to apply the new disease behaviour classification (DBC) and the previously validated ILD-gender age physiology (GAP) score to a cohort of unclassifiable ILD patients and to assess the prognostic value of these two composite approaches...
April 2017: Respirology: Official Journal of the Asian Pacific Society of Respirology
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