Publications
772 results found
Garner IM, Evans IC, Maher TM, et al., 2013, Genome-wide analysis identifies multiple genes with altered methylation and expression in IPF and SSc lung fibroblasts, Spring Meeting of the British-Society-for-Matrix-Biology, Publisher: WILEY-BLACKWELL, Pages: A15-A16, ISSN: 0959-9673
Garner IM, Evans IC, Barnes JL, et al., 2013, Hypomethylation of the TNXB gene contributes to increased expression and deposition of tenascin X in idiopathic pulmonary fibrosis, Spring Meeting of the British-Society-for-Matrix-Biology, Publisher: WILEY-BLACKWELL, Pages: A15-A15, ISSN: 0959-9673
Abdullah RR, Wells AU, Renzoni EA, et al., 2013, Infliximab: An effective rescue therapy in refractory extra-pulmonary sarcoidosis, EUROPEAN RESPIRATORY JOURNAL, Vol: 42, ISSN: 0903-1936
Lindahl G, Leoni P, Stock C, et al., 2013, Bromodomain inhibitor JQ1 attenuates TGF-β-suppressed SOD2 expression and reduces proliferation in human primary pulmonary fibroblasts, Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
Molyneaux PL, Maher TM, 2013, The role of infection in the pathogenesis of idiopathic pulmonary fibrosis., Eur Respir Rev, Vol: 22, Pages: 376-381
Idiopathic pulmonary fibrosis (IPF) is a progressive, and invariably fatal, condition that is believed to arise in genetically susceptible individuals as a consequence of an aberrant wound-healing response following repetitive alveolar injury. The exact triggers, which initiate the fibrotic process, remain unknown. Infectious agents, including both viruses and bacteria, have the capacity to cause alveolar-epithelial cell injury and apoptosis. Relatively few studies have examined the role of infection in IPF. Those that have, point to viruses playing a key role as cofactors in the initiation and progression of IPF. There is also some evidence to suggest that viral infection may be responsible for a proportion of acute exacerbations of IPF. The role played by bacteria in the pathogenesis of IPF is less clear cut. Studies from other respiratory diseases suggest that alterations in the lung microbiome are associated with disease and that these changes influence disease behaviour. Emerging molecular microbiological techniques are making the study of microbial communities in the lung easier. It is hoped that by combining such techniques with the careful longitudinal phenotyping of patients with IPF, it will be possible to elucidate the role played by bacteria and viruses in the pathogenesis of the disease. If infection plays a causal role in IPF then it is possible that therapeutic strategies, utilising currently available antiviral or antibiotic drugs, may be effective in modifying the course of this devastating condition.
Abdullah R, Ming D, Keir G, et al., 2013, Rituximab in severe, treatment-refractory interstitial lung disease, EUROPEAN RESPIRATORY JOURNAL, Vol: 42, ISSN: 0903-1936
Russell A-M, Fraser U, Molyneaux P, et al., 2013, Quality of life measures in patients with idiopathic pulmonary fibrosis, EUROPEAN RESPIRATORY JOURNAL, Vol: 42, ISSN: 0903-1936
- Author Web Link
- Cite
- Citations: 1
Keir G, Corte T, Parfitt L, et al., 2013, Bosentan in pulmonary hypertension associated with fibrotic idiopathic interstitial pneumonia: A randomized, double-blind, placebo-controlled study, EUROPEAN RESPIRATORY JOURNAL, Vol: 42, ISSN: 0903-1936
Antoniou K, Margaritopoulos G, Hansell D, et al., 2013, Scleroderma lung disease: Detection of pulmonary hypertension using pulmonary function tests is problematic in patients with emphysema, EUROPEAN RESPIRATORY JOURNAL, Vol: 42, ISSN: 0903-1936
Mandal S, Suh E, Davies M, et al., 2013, Provision of home mechanical ventilation and sleep services for England survey, THORAX, Vol: 68, Pages: 880-881, ISSN: 0040-6376
- Author Web Link
- Cite
- Citations: 20
Patel AS, Siegert RJ, Keir GJ, et al., 2013, The minimal important difference of the King's Brief Interstitial Lung Disease Questionnaire (K-BILD) and forced vital capacity in interstitial lung disease, RESPIRATORY MEDICINE, Vol: 107, Pages: 1438-1443, ISSN: 0954-6111
- Author Web Link
- Cite
- Citations: 38
Maher TM, 2013, Beyond the diagnosis of idiopathic pulmonary fibrosis; the growing role of systems biology and stratified medicine, CURRENT OPINION IN PULMONARY MEDICINE, Vol: 19, Pages: 460-465, ISSN: 1070-5287
- Author Web Link
- Cite
- Citations: 22
Lindahl GE, Stock CJW, Xu S-W, et al., 2013, Microarray profiling reveals suppressed interferon stimulated gene program in fibroblasts from scleroderma-associated interstitial lung disease, Respiratory Research, Vol: 14, ISSN: 1465-9921
Background: Interstitial lung disease is a major cause of morbidity and mortality in systemic sclerosis (SSc), with insufficiently effective treatment options. Progression of pulmonary fibrosis involves expanding populations of fibroblasts, and the accumulation of extracellular matrix proteins. Characterisation of SSc lung fibroblast gene expression profiles underlying the fibrotic cell phenotype could enable a better understanding of the processes leading to the progressive build-up of scar tissue in the lungs. In this study we evaluate the transcriptomes of fibroblasts isolated from SSc lung biopsies at the time of diagnosis, compared with those from control lungs.Methods: We used Affymetrix oligonucleotide microarrays to compare the gene expression profile of pulmonary fibroblasts cultured from 8 patients with pulmonary fibrosis associated with SSc (SSc-ILD), with those from control lung tissue peripheral to resected cancer (n=10). Fibroblast cultures from 3 patients with idiopathic pulmonary fibrosis (IPF) were included as a further comparison. Genes differentially expressed were identified using two separate analysis programs following a set of pre-determined criteria: only genes significant in both analyses were considered. Microarray expression data was verified by qRT-PCR and/or western blot analysis.Results: A total of 843 genes were identified as differentially expressed in pulmonary fibroblasts from SSc-ILD and/or IPF compared to control lung, with a large overlap in the expression profiles of both diseases. We observed increased expression of a TGF-β response signature including fibrosis associated genes and myofibroblast markers, with marked heterogeneity across samples. Strongly suppressed expression of interferon stimulated genes, including antiviral, chemokine, and MHC class 1 genes, was uniformly observed in fibrotic fibroblasts. This expression profile includes key regulators and mediators of the interferon response, such as STAT1, and CXCL10
Lota HK, Keir GJ, Hansell DM, et al., 2013, Novel use of rituximab in hypersensitivity pneumonitis refractory to conventional treatment, THORAX, Vol: 68, ISSN: 0040-6376
- Author Web Link
- Cite
- Citations: 39
Richeldi L, Collard HR, du Bois RM, et al., 2013, Mapping the future for pulmonary fibrosis: report from the 17th International. Colloquium on Lung and Airway Fibrosis, EUROPEAN RESPIRATORY JOURNAL, Vol: 42, Pages: 230-238, ISSN: 0903-1936
- Author Web Link
- Cite
- Citations: 20
Woodcock HV, Molyneaux PL, Maher TM, 2013, Reducing lung function decline in patients with idiopathic pulmonary fibrosis: potential of nintedanib, Drug Design, Development and Therapy, Vol: 7, Pages: 503-510, ISSN: 1177-8881
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, fibrotic lung disease with no clear etiology and a paucity of therapeutic options. Nintedanib (previously known as BIBF 1120) is a tyrosine kinase receptor antagonist which inhibits a number of key receptors, including those for platelet derived growth factor (PDGF), vascular endothelial growth factor (VEGF), and fibroblast growth factor (FGF). These growth factors are profibrotic and each has been investigated as a potential standalone therapeutic target in IPF. Simultaneous inhibition of these receptors, with an analog of nintedanib, has proved to be effective in experimental animal models of pulmonary fibrosis. This observation, together with extensive safety and pharmacokinetic data from studies of nintedanib in malignancy, paved the way for the clinical development of this drug in IPF. The Phase IIb TOMORROW trial demonstrated that treatment with nintedanib may potentially slow decline in lung function, decrease the frequency of acute exacerbations, and improve quality of life in patients with IPF. While these observations are drawn from a single clinical trial, taken together with the preclinical data they suggest that nintedanib may yet become an important therapeutic option for individuals with IPF. The results of ongoing parallel, international, multicenter Phase III clinical trials are therefore eagerly awaited.
Spagnolo P, Luppi F, Maher TM, et al., 2013, Primary Endpoints in Phase 3 Clinical Trials in Idiopathic Pulmonary Fibrosis: One Step at a Time, AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE, Vol: 187, Pages: 1271-1272, ISSN: 1073-449X
- Author Web Link
- Cite
- Citations: 2
Maher TM, 2013, PROFILEing idiopathic pulmonary fibrosis: rethinking biomarker discovery., Eur Respir Rev, Vol: 22, Pages: 148-152
Despite major advances in the understanding of the pathogenesis of idiopathic pulmonary fibrosis (IPF), diagnosis and management of the condition continue to pose significant challenges. Clinical management of IPF remains unsatisfactory due to limited availability of effective drug therapies, a lack of accurate indicators of disease progression, and an absence of simple short-term measures of therapeutic response. The identification of more accurate predictors of prognosis and survival in IPF would facilitate counseling of patients and their families, aid communication among clinicians, and would guide optimal timing of referral for transplantation. Improvements in molecular techniques have led to the identification of new disease pathways and a more targeted approach to the development of novel anti-fibrotic agents. However, despite an increased interest in biomarkers of IPF disease progression there are a lack of measures that can be used in early phase clinical trials. Careful longitudinal phenotyping of individuals with IPF together with the application of novel omics-based technology should provide important insights into disease pathogenesis and should address some of the major issues holding back drug development in IPF. The PROFILE (Prospective Observation of Fibrosis in the Lung Clinical Endpoints) study is a currently enrolling, prospective cohort study designed to tackle these issues.
Hull JH, Menzies-Gow A, Nicholson AG, et al., 2013, Exercise-induced haemoptysis: a thoroughbred cause?, THORAX, Vol: 68, Pages: 599-600, ISSN: 0040-6376
- Author Web Link
- Cite
- Citations: 3
Fingerlin TE, Murphy E, Zhang W, et al., 2013, Genome-wide association study identifies multiple susceptibility loci for pulmonary fibrosis, Nature Genetics, Vol: 45, Pages: 613-624, ISSN: 1061-4036
We performed a genome-wide association study of non-Hispanic, white individuals with fibrotic idiopathic interstitial pneumonias (IIPs; n = 1,616) and controls (n = 4,683), with follow-up replication analyses in 876 cases and 1,890 controls. We confirmed association with TERT at 5p15, MUC5B at 11p15 and the 3q26 region near TERC, and we identified seven newly associated loci (Pmeta = 2.4 × 10−8 to 1.1 × 10−19), including FAM13A (4q22), DSP (6p24), OBFC1 (10q24), ATP11A (13q34), DPP9 (19p13) and chromosomal regions 7q22 and 15q14-15. Our results suggest that genes involved in host defense, cell-cell adhesion and DNA repair contribute to risk of fibrotic IIPs.
Raghu G, Behr J, Brown KK, et al., 2013, Treatment of Idiopathic Pulmonary Fibrosis With Ambrisentan A Parallel, Randomized Trial, ANNALS OF INTERNAL MEDICINE, Vol: 158, Pages: 641-+, ISSN: 0003-4819
- Author Web Link
- Cite
- Citations: 347
Stock CJ, Sato H, Fonseca C, et al., 2013, Mucin 5B promoter polymorphism is associated with idiopathic pulmonary fibrosis but not with development of lung fibrosis in systemic sclerosis or sarcoidosis, THORAX, Vol: 68, Pages: 436-441, ISSN: 0040-6376
- Author Web Link
- Cite
- Citations: 159
Cullinan P, McGavin CR, Kreiss K, et al., 2013, Obliterative bronchiolitis in fibreglass workers: a new occupational disease?, OCCUPATIONAL AND ENVIRONMENTAL MEDICINE, Vol: 70, Pages: 357-359, ISSN: 1351-0711
- Author Web Link
- Cite
- Citations: 22
Eves ND, Song Y, Piper A, et al., 2013, Year in review 2012: Acute lung injury, interstitial lung diseases, sleep and physiology, RESPIROLOGY, Vol: 18, Pages: 555-564, ISSN: 1323-7799
- Author Web Link
- Cite
- Citations: 7
De Lauretis A, Sestini P, Pantelidis P, et al., 2013, Serum Interleukin 6 Is Predictive of Early Functional Decline and Mortality in Interstitial Lung Disease Associated with Systemic Sclerosis, JOURNAL OF RHEUMATOLOGY, Vol: 40, Pages: 435-446, ISSN: 0315-162X
- Author Web Link
- Cite
- Citations: 180
Lindahl G, Leoni P, Stock C, et al., 2013, Bromodomain Inhibitor Jq1 Attenuates Tgf-B-Induced Nox4 And Asma Expression As Well As Collagen Gel Contraction By Human Pulmonary Fibroblasts, Publisher: AMER THORACIC SOC, ISSN: 1073-449X
Fingerlin TE, Murphy E, Zhang W, et al., 2013, Novel Genetic Risk Loci For Pulmonary Fibrosis, Publisher: AMER THORACIC SOC, ISSN: 1073-449X
Russell A-M, Molyneaux PL, Wells AU, et al., 2013, Symptom Measures In Patients With Idiopathic Pulmonary Fibrosis, AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE, Vol: 187, ISSN: 1073-449X
Maher TM, 2013, Idiopathic pulmonary fibrosis, OUTCOMES IN CLINICAL TRIALS, Editors: Kolb, Vogelmeier, Welte, Publisher: EUROPEAN RESPIRATORY SOCIETY, Pages: 37-53, ISBN: 978-1-84984-044-6
- Author Web Link
- Cite
- Citations: 2
Davies ER, Calderwood C, Jones MG, et al., 2013, The Depsipeptide Hdac Inhibitor Fk228 Has Potent Anti-Fibrotic Properties In Ipf Fibroblasts And Decreases The Expression Of Lysyl Oxidase A Potential Ipf Biomarker, Publisher: AMER THORACIC SOC, ISSN: 1073-449X
- Author Web Link
- Cite
- Citations: 1
This data is extracted from the Web of Science and reproduced under a licence from Thomson Reuters. You may not copy or re-distribute this data in whole or in part without the written consent of the Science business of Thomson Reuters.