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interstitial pulmonary 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/28339739/unabated-occupational-risk-in-a-patient-with-rheumatoid-pulmonary-fibrosis
#4
V K Holden, S J Kligerman, T G Hastings, S E Hines
Background: This case highlights the importance of considering hypersensitivity pneumonitis (HP) in the differential diagnosis of interstitial lung disease (ILD) and of obtaining an occupational history so that remediable risk factors may be identified and managed. Aims: To report a case of a chicken sexer with severe rheumatoid arthritis (RA) who developed progressively worsening dyspnoea and restrictive lung disease associated with pulmonary fibrosis. Methods: Clinical investigation included physical examination, occupational history, pulmonary function tests (PFTs), chest imaging and bronchoalveolar lavage (BAL), as well as serological tests including standard IgE bird feather mixture and local IgG precipitin preparation to chicken excrement...
February 23, 2017: Occupational 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
#5
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/28335088/further-characterization-of-computed-tomographic-and-clinical-features-for-staging-and-prognosis-of-idiopathic-pulmonary-fibrosis-in-west-highland-white-terriers
#6
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/28326312/a-comparison-of-published-multidimensional-indices-to-predict-outcome-in-idiopathic-pulmonary-fibrosis
#7
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
#8
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/28325283/silencing-of-carbohydrate-sulfotransferase-15-hinders-murine-pulmonary-fibrosis-development
#9
Yoshiro Kai, Koichi Tomoda, Hiroyuki Yoneyama, Masahiro Kitabatake, Atsuhiro Nakamura, Toshihiro Ito, Masanori Yoshikawa, Hiroshi Kimura
Pulmonary fibrosis is a progressive lung disorder characterized by interstitial fibrosis, for which no effective treatments are available. Chondroitin sulfate proteoglycan (CSPG) has been shown to be a mediator, but the specific component of glycosaminoglycan chains of CSPG has not been explored. We show that chondroitin sulfate E-type (CS-E) is involved in fibrogenesis. Small interfering RNA (siRNA) targeting carbohydrate sulfotransferase 15 (CHST15) was designed to inhibit CHST15 mRNA and its product, CS-E...
March 17, 2017: Molecular Therapy. Nucleic Acids
https://www.readbyqxmd.com/read/28323056/fty720-promotes-pulmonary-fibrosis-when-administered-during-the-remodelling-phase-following-a-bleomycin-induced-lung-injury
#10
David R Gendron, Anne-Marie Lemay, Pascale Blais Lecours, Valérie Perreault-Vallières, Carole-Ann Huppé, Ynuk Bossé, Marie-Renée Blanchet, Geneviève Dion, David Marsolais
Fibrosis complicates numerous pathologies including interstitial lung diseases. Sphingosine analogs such as FTY720 can alleviate lung injury-induced fibrosis in murine models. Contradictorily, FTY720 also promotes in vitro processes normally leading to fibrosis and high doses in vivo foster lung fibrosis by enhancing vascular leakage into the lung. The goal of this study was to determine the effect of low doses of FTY720 on lung fibrosis triggered by an acute injury in mice. We first defined the time-boundaries delimiting the inflammatory and remodelling phases of an injury elicited by bleomycin based on neutrophil counts, total lung capacity and lung stiffness...
March 16, 2017: Pulmonary Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/28322466/the-role-of-mir-497-eif3a-axis-in-tgf%C3%AE-1-induced-epithelial-mesenchymal-transition-and-extracellular-matrix-in-rat-alveolar-epithelial-cells-and-pulmonary-fibroblasts
#11
Ren Guo, Yu Lv, Yang Ouyang, Siyu Liu, Dai Li
Multi-cause-induced interstitial lung disease, particularly pulmonary fibrosis, is a serious clinical concern. fibroblasts have been suggested to have a major role, with it recently being revealed that some of these fibroblasts are derived from alveolar epithelial cells through epithelial-mesenchymal transition (EMT). Eukaryotic translation initiation factor 3 subunit A (EIF3A) is a protein that in humans is encoded by the EIF3A gene, and has been suggested to play roles in regulating translation of a subset of mRNAs and in regulating cell cycle progression and cell proliferation...
March 21, 2017: Journal of Cellular Biochemistry
https://www.readbyqxmd.com/read/28321059/arterial-carboxyhemoglobin-measurement-is-useful-for-evaluating-pulmonary-inflammation-in-subjects-with-interstitial-lung-disease
#12
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/28300665/grape-seed-extract-ameliorates-bleomycin-induced-mouse-pulmonary-fibrosis
#13
Qi Liu, Jun-Xia Jiang, Ya-Nan Liu, Yan Guan, Wei Zhao, Yong-Liang Jia, Xin-Wei Dong, Yun Sun, Qiang-Min Xie
Pulmonary fibrosis is common in a variety of inflammatory lung diseases, such as interstitial pneumonia, chronic obstructive pulmonary disease, and silicosis. There is currently no effective clinical drug treatment. It has been reported that grape seed extracts (GSE) has extensive pharmacological effects with minimal toxicity. Although it has been found that GSE can improve the lung collagen deposition and fibrosis pathology induced by bleomycin in rat, its effects on pulmonary function, inflammation, growth factors, matrix metalloproteinases and epithelial-mesenchymal transition remain to be researched...
March 11, 2017: Toxicology Letters
https://www.readbyqxmd.com/read/28300574/sex-steroid-receptor-expression-in-idiopathic-pulmonary-fibrosis
#14
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
#15
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
#16
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
#17
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
https://www.readbyqxmd.com/read/28292522/guidelines-for-the-medical-treatment-of-idiopathic-pulmonary-fibrosis
#18
Antoni Xaubet, María Molina-Molina, Orlando Acosta, Elena Bollo, Diego Castillo, Estrella Fernández-Fabrellas, José Antonio Rodríguez-Portal, Claudia Valenzuela, Julio Ancochea
Idiopathic pulmonary fibrosis is defined as chronic fibrosing interstitial pneumonia limited to the lung, with poor prognosis. The incidence has been rising in recent years probably due to improved diagnostic methods and increased life expectancy. In 2013, the SEPAR guidelines for the diagnosis and treatment for idiopathic pulmonary fibrosis were published. Since then, clinical trials and meta-analyses have shown strong scientific evidence for the use of pirfenidone and nintedanib in the treatment of idiopathic pulmonary fibrosis...
March 11, 2017: Archivos de Bronconeumología
https://www.readbyqxmd.com/read/28285268/cardiac-manifestations-of-parasitic-diseases
#19
REVIEW
Maria Carmo P Nunes, Milton Henriques Guimarães Júnior, Adriana Costa Diamantino, Claudio Leo Gelape, Teresa Cristina Abreu Ferrari
The heart may be affected directly or indirectly by a variety of protozoa and helminths. This involvement may manifest in different ways, but the syndromes resulting from impairment of the myocardium and pericardium are the most frequent. The myocardium may be invaded by parasites that trigger local inflammatory response with subsequent myocarditis or cardiomyopathy, as occurs in Chagas disease, African trypanosomiasis, toxoplasmosis, trichinellosis and infection with free-living amoebae. In amoebiasis and echinococcosis, the pericardium is the structure most frequently involved with consequent pericardial effusion, acute pericarditis, cardiac tamponade or constrictive pericarditis...
March 11, 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/28275536/rheumatoid-arthritis-associated-pulmonary-hypertension-clinical-challenges-reflecting-the-diversity-of-pathophysiology
#20
Evangelia Panagiotidou, Evdokia Sourla, Serafim Xrisovalantis Kotoulas, Sofia Akritidou, Vasileios Bikos, Vasileios Bagalas, Ioannis Stanopoulos, Georgia Pitsiou
The present article reports three clinical cases in order to elucidate the diversity of the pathophysiological mechanisms that underlie rheumatoid arthritis associated pulmonary hypertension. The condition's three major causes are: interstitial lung disease, vasculitis, and chronic thromboembolic disease, but it should be noted that the multiple pulmonary manifestations of rheumatoid arthritis, can all contribute to chronic lung disease or hypoxia. The first patient in this report suffered from moderate restriction due to fibrosis and was diagnosed with pulmonary hypertension during an episode of life threatening hypoxia...
2017: Respiratory Medicine Case Reports
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