Publications
565 results found
Vachiery J-L, Adir Y, Albert Barbera J, et al., 2013, Pulmonary Hypertension Due to Left Heart Diseases, JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY, Vol: 62, Pages: D100-D108, ISSN: 0735-1097
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- Citations: 449
Saketkoo LA, Mittoo S, Huscher D, et al., 2013, Connective tissue disease related interstitial lung diseases and idiopathic pulmonary fibrosis: provisional core sets of domains and instruments for use in clinical trials, Thorax, Vol: 69, Pages: 428-436, ISSN: 0040-6376
Rationale Clinical trial design in interstitial lung diseases (ILDs) has been hampered by lack of consensus on appropriate outcome measures for reliably assessing treatment response. In the setting of connective tissue diseases (CTDs), some measures of ILD disease activity and severity may be confounded by non-pulmonary comorbidities.Methods The Connective Tissue Disease associated Interstitial Lung Disease (CTD-ILD) working group of Outcome Measures in Rheumatology—a non-profit international organisation dedicated to consensus methodology in identification of outcome measures—conducted a series of investigations which included a Delphi process including >248 ILD medical experts as well as patient focus groups culminating in a nominal group panel of ILD experts and patients. The goal was to define and develop a consensus on the status of outcome measure candidates for use in randomised controlled trials in CTD-ILD and idiopathic pulmonary fibrosis (IPF).Results A core set comprising specific measures in the domains of lung physiology, lung imaging, survival, dyspnoea, cough and health-related quality of life is proposed as appropriate for consideration for use in a hypothetical 1-year multicentre clinical trial for either CTD-ILD or IPF. As many widely used instruments were found to lack full validation, an agenda for future research is proposed.Conclusion Identification of consensus preliminary domains and instruments to measure them was attained and is a major advance anticipated to facilitate multicentre RCTs in the field.
Seeger W, Adir Y, Albert Barbera J, et al., 2013, Pulmonary Hypertension in Chronic Lung Diseases, JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY, Vol: 62, Pages: D109-D116, ISSN: 0735-1097
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- Citations: 407
Boutou AK, Nair A, Sandhu R, et al., 2013, A NOVEL COMPOSITE INDEX FOR PROGNOSTIC STAGING OF COPD PATIENTS, Winter Meeting of the British-Thoracic-Society, Publisher: BMJ PUBLISHING GROUP, Pages: A137-A137, ISSN: 0040-6376
Garfield BE, Keir GJ, Price LC, et al., 2013, HYPERSENSITIVITY PNEUMONITIS COMPLICATED BY PULMONARY HYPERTENSION; PATIENT CHARACTERISTICS AND RESPONSE TO TARGETED THERAPY, Winter Meeting of the British-Thoracic-Society, Publisher: BMJ PUBLISHING GROUP, Pages: A27-A28, ISSN: 0040-6376
Abdullah RR, Ming D, Keir GJ, et al., 2013, RITUXIMAB AS RESCUE THERAPY IN INTERSTITIAL LUNG DISEASE REFRACTORY TO CONVENTIONAL IMMUNOSUPPRESSION, Winter Meeting of the British-Thoracic-Society, Publisher: BMJ PUBLISHING GROUP, Pages: A167-A167, ISSN: 0040-6376
Antoniou KM, Walsh SL, Hansell DM, et al., 2013, Smoking-related emphysema is associated with idiopathic pulmonary fibrosis and rheumatoid lung, RESPIROLOGY, Vol: 18, Pages: 1191-1196, ISSN: 1323-7799
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- Citations: 41
Wells AU, 2013, Chronic hypersensitivity pneumonitis in the setting of definite IPF: does the current study undermine IPF guideline recommendations?, LANCET RESPIRATORY MEDICINE, Vol: 1, Pages: 664-665, ISSN: 2213-2600
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- Citations: 3
Bajwah S, Higginson IJ, Ross JR, et al., 2013, The palliative care needs for fibrotic interstitial lung disease: A qualitative study of patients, informal caregivers and health professionals, PALLIATIVE MEDICINE, Vol: 27, Pages: 869-876, ISSN: 0269-2163
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- Citations: 110
Boland J, Martin J, Wells AU, et al., 2013, Palliative care for people with non-malignant lung disease: Summary of current evidence and future direction, PALLIATIVE MEDICINE, Vol: 27, Pages: 811-816, ISSN: 0269-2163
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- Citations: 60
Tomassetti S, Wells AU, Costabel U, et al., 2013, The diagnostic role of cryobiopsy in the multidisciplinary diagnosis of idiopathic pulmonary fibrosis, EUROPEAN RESPIRATORY JOURNAL, Vol: 42, ISSN: 0903-1936
Gialafos E, Rapti A, Kouranos V, et al., 2013, BNP can be an all-cause mortality predictor in 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
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
Lota H, Stock C, Sato H, et al., 2013, LSC 2013 abstract - Hypoxia-inducible factor 1 alpha polymorphisms in relation to pulmonary involvement in systemic sclerosis, 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
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
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
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- Citations: 1
Antoniou K, Hansell D, Goh N, et al., 2013, Scleroderma lung disease: A scleroderma physiologic index (SPI) derived from lung physiology and oxygen saturation, EUROPEAN RESPIRATORY JOURNAL, Vol: 42, ISSN: 0903-1936
Lota H, Stock C, Sato H, et al., 2013, Hypoxia-inducible factor 1 alpha polymorphisms in relation to pulmonary involvement in systemic sclerosis, EUROPEAN RESPIRATORY JOURNAL, Vol: 42, ISSN: 0903-1936
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
Bajwah S, Ross JR, Peacock JL, et al., 2013, Interventions to improve symptoms and quality of life of patients with fibrotic interstitial lung disease: a systematic review of the literature, THORAX, Vol: 68, Pages: 867-879, ISSN: 0040-6376
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- Citations: 70
Cottin V, Wells A, 2013, Unclassified or unclassifiable interstitial lung disease: confusing or helpful disease category?, EUROPEAN RESPIRATORY JOURNAL, Vol: 42, Pages: 576-579, ISSN: 0903-1936
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- Citations: 24
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
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- Citations: 38
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
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- Citations: 39
Aihara K, Handa T, Oga T, et al., 2013, Clinical Relevance of Plasma Prostaglandin F<sub>2α</sub> Metabolite Concentrations in Patients with Idiopathic Pulmonary Fibrosis, PLOS ONE, Vol: 8, ISSN: 1932-6203
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- Citations: 20
Wells AU, 2013, Managing diagnostic procedures in idiopathic pulmonary fibrosis., Eur Respir Rev, Vol: 22, Pages: 158-162
Idiopathic pulmonary fibrosis (IPF), the most prevalent of the idiopathic interstitial pneumonias, is associated with a poor prognosis. An accurate diagnosis of IPF is essential for its optimal management. The 2011 American Thoracic Society (ATS)/European Respiratory Society (ERS)/Japanese Respiratory Society (JRS)/Latin American Thoracic Association (ALAT) recommendations on the diagnosis and management of IPF were developed from a systematic review of the published literature. High-resolution computed tomography (HRCT) scanning has a central role in the IPF diagnostic pathway, with formal designation of criteria for an HRCT pattern of usual interstitial pneumonia. In the correct clinical context, a usual interstitial pneumonia pattern on HRCT is indicative of a definite diagnosis of IPF and negates the need for a surgical lung biopsy. However, although the 2011 ATS/ERS/JRS/ALAT statement is a major advance, the application of the guideline recommendations by clinicians has identified limitations that should be addressed in future statements. Key problems include: 1) HRCT misdiagnosis, particularly by less experienced radiologists; 2) lack of management recommendations for the highly prevalent clinical scenarios of "probable" or "possible" IPF; 3) ongoing confusion concerning the diagnostic role of bronchoalveolar lavage; and 4) the lack of integration of clinical data in the designation of the diagnostic likelihood of IPF, including the treated course of disease. These issues become evident as the recommendations are applied and highlight the need for continued guideline adjustments.
Saketkoo LA, Khanna D, Huscher D, et al., 2013, DEVELOPING A DISEASE ACTIVITY AND THERAPEUTIC RESPONSE INDEX IN CONNECTIVE TISSUE DISEASE - INTERSTITIAL LUNG DISEASE (CTD-ILD): RESULTS FROM A DELPHI EXERCISE: CONSENSUS ON DOMAINS, ANNALS OF THE RHEUMATIC DISEASES, Vol: 71, Pages: 52-52, ISSN: 0003-4967
Wells A, 2013, HISTOPATHOLOGY OF ILD IN RHEUMATIC DISEASE - WHAT CLUES DOES IT PROVIDE RE PATHOGENESIS AND TREATMENT OPTIONS, ANNALS OF THE RHEUMATIC DISEASES, Vol: 71, Pages: 3-3, ISSN: 0003-4967
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