Publications
159 results found
Bax S, Jacob J, Ahmed R, et al., 2020, Right ventricle to left ventricle ratio at CTPA predicts mortality in interstitial lung disease, Chest, Vol: 157, Pages: 89-98, ISSN: 0012-3692
INTRODUCTION: Patients with interstitial lung disease (ILD) may develop pulmonary hypertension (PH), often disproportionate to ILD severity. Right ventricle to left ventricle diameter ratio (RV:LV) measured at CT pulmonary angiography (CTPA), has been shown to provide valuable information in pulmonary arterial hypertension patients and to predict death or deterioration in acute pulmonary embolism. METHODS: Demographics, ILD subtype, echocardiography and detailed CTPA measurements were collected in consecutive patients undergoing both CTPA and right heart catheterisation (RHC) at the Royal Brompton Hospital between 2005 and 2015. Fibrosis severity was formally scored using CT criteria. RV:LV ratio at CTPA was evaluated by three different methods. Cox-proportional hazard analysis was used to assess the relation of CTPA-derived parameters to predict death or lung transplantation. RESULTS: 92 patients were included: 64% male, mean age 65±11 years, with FVC 57±20% (predicted), TLCOc 22±8% (predicted) and KCOc 51±17% (predicted). PH was confirmed at RHC in 78%. Of all CTPA-derived measures, an RV:LV ratio ≥1.0 strongly predicted mortality or transplantation at univariate analysis (HR 3.26, 95%CI:1.49-7.13, p=0.003), whereas invasive haemodynamic data did not. The RV:LV ratio remained an independent predictor at multivariate analysis (HR: 3.19, CI:1.44-7.10, p=0.004), adjusting for an ILD diagnosis of IPF and CT derived ILD severity. CONCLUSION: An increased RV:LV ratio measured at CTPA provides a simple, non-invasive method of risk stratification in patients with suspected ILD-PH. This should prompt closer follow up, more aggressive treatment and consideration of lung transplantation.
Mackintosh J, Kumar R, Mann J, et al., 2020, Vitamin D Deficiency Is Associated with Adverse Survival in Patients with Idiopathic Pulmonary Fibrosis, International Conference of the American-Thoracic-Society (ATS), Publisher: AMER THORACIC SOC, ISSN: 1073-449X
Thillai M, Herlekar R, Denneny EK, et al., 2020, The QRISK-2 Score Identifies an Increased Cardiovascular Risk in Patients with Idiopathic Pulmonary Fibrosis at Time of Diagnosis, International Conference of the American-Thoracic-Society (ATS), Publisher: AMER THORACIC SOC, ISSN: 1073-449X
Stock CJW, Visca D, DeLauretis A, et al., 2019, SERUM BIOMARKERS IN SSC-ILD: ASSOCIATION WITH PRESENCE, SEVERITY AND PROGNOSIS, Winter Meeting of the British-Thoracic-Society, Publisher: BMJ PUBLISHING GROUP, Pages: A56-A56, ISSN: 0040-6376
Stock CJW, DeLauretis A, Visca D, et al., 2019, VERIFICATION OF GENETIC ASSOCIATIONS WITH SCLERODERMA ASSOCIATED INTERSTITIAL LUNG DISEASE, Winter Meeting of the British-Thoracic-Society, Publisher: BMJ PUBLISHING GROUP, Pages: A46-A46, ISSN: 0040-6376
Alfieri V, Crisafulli E, Visca D, et al., 2019, Physiological predictors of exertional oxygen desaturation in patients with fibrotic interstitial lung disease, European Respiratory Journal, Vol: 55, Pages: 1-4, ISSN: 0903-1936
George PM, Mitchell JA, 2019, Defining a pathological role for the vasculature in the development of fibrosis and pulmonary hypertension in interstitial lung disease, AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY, Vol: 317, Pages: L431-L433, ISSN: 1040-0605
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- Citations: 6
Stock C, De Lauretis A, Visca D, et al., 2019, Verification of genetic associations with Seleroderma associated Interstitial Lung Disease, International Congress of the European-Respiratory-Society (ERS), Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
Patel S, Orme M, Nolan CM, et al., 2019, Clinical Correlates of Physical Activity in Idiopathic Pulmonary Fibrosis (IPF), International Congress of the European-Respiratory-Society (ERS), Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
Nolan CM, Maddocks M, Maher TM, et al., 2019, Decline in 4-metre gait speed (4MGS) over 6 months is associated with mortality in IPF, International Congress of the European-Respiratory-Society (ERS), Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
Stock C, De Lauretis A, Visca D, et al., 2019, Serum biomarkers in SSc-ILD: association with presence, severity and prognosis, International Congress of the European-Respiratory-Society (ERS), Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
Margaritopoulos G, Proklou A, Badenes Bonet D, et al., 2019, Nocturnal desaturation predicts mortality in immune-mediated ILDs, International Congress of the European-Respiratory-Society (ERS), Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
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- Citations: 1
Nolan CM, Birring SS, Maddocks M, et al., 2019, King's Brief Interstitial Lung Disease questionnaire: responsiveness and minimum clinically important difference, European Respiratory Journal, Vol: 54, ISSN: 0903-1936
Health status is increasingly used in clinical practice to quantify symptom burden and as a clinical trial endpoint in patients with interstitial lung disease (ILD). The Kings Brief Interstitial Lung Disease (KBILD) questionnaire is a brief validated 15-item, disease-specific, health-related quality of life questionnaire that is increasingly used in clinical trials, but little data exist regarding the minimum clinically important difference (MCID). Using pulmonary rehabilitation as a model, we aimed to determine responsiveness of the KBILD and provide estimates of the MCID.KBILD, Chronic Respiratory Questionnaire (CRQ), Medical Research Council dyspnoea scale (MRC) and incremental shuttle walk test (ISW) were measured in 209 patients with ILD (105 with idiopathic pulmonary fibrosis (IPF)) before and after an outpatient pulmonary rehabilitation programme. Changes with intervention and Cohen's effect size were calculated. Anchor- (linear regression and Receiver Operating Characteristic plots) or distribution-based approaches (0.5 * standard deviation, standard error of measurement) were used to estimate the MCID of KBILD domain and total scores.KBILD, CRQ, MRC and ISW improved with intervention and the effect sizes of KBILD domain and total scores ranged from 0.28 to 0.38. Using anchor-based estimates, the MCID estimate for KBILD-Psychological, KBILD-Breathlessness and activities and KBILD-Total score were 5.4, 4.4 and 3.9 respectively. Using distribution-based methods, the MCID estimate for KBILD-Chest symptoms was 9.8. The MCID estimates for KBILD in IPF patients were similar.In patients with ILD and IPF, KBILD is responsive to intervention with an estimated MCID of 3.9 for the total score.
Mackintosh JA, Desai SR, Adamali H, et al., 2019, In patients with idiopathic pulmonary fibrosis the presence of hiatus hernia is associated with disease progression and mortality, European Respiratory Journal, Vol: 53, ISSN: 0903-1936
George PM, Patterson CM, Reed AK, et al., 2019, Lung transplantation for idiopathic pulmonary fibrosis, LANCET RESPIRATORY MEDICINE, Vol: 7, Pages: 271-282, ISSN: 2213-2600
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- Citations: 131
Mackintosh J, Desai S, Adamali HI, et al., 2019, In Treated Patients with Idiopathic Pulmonary Fibrosis Hiatus Hernia but Not Acid Reflux Predicts Disease Progression and Survival, International Conference of the American-Thoracic-Society, Publisher: AMER THORACIC SOC, ISSN: 1073-449X
Nolan CM, Maddocks M, Maher T, et al., 2019, Single-Versus Multi-Component Physical Performance Measures and Mortality in Idiopathic Pulmonary Fibrosis, International Conference of the American-Thoracic-Society, Publisher: AMER THORACIC SOC, ISSN: 1073-449X
Desai SR, Mackintosh JA, Chua F, et al., 2018, HIATUS HERNIA IS HIGHLY PREVALENT IN IDIOPATHIC PULMONARY FIBROSIS AND IMPACTS DISEASE PROGRESSION, Winter Meeting of the British-Thoracic-Society, Publisher: BMJ PUBLISHING GROUP, Pages: A187-A188, ISSN: 0040-6376
Stock CJW, Hoyles R, D'accord C, et al., 2018, SERUM CYFRA 21-1 AS A PROGNOSTIC MARKER IN SCLERODERMA-ASSOCIATED INTERSTITIAL LUNG DISEASE, Winter Meeting of the British-Thoracic-Society, Publisher: BMJ PUBLISHING GROUP, Pages: A89-A90, ISSN: 0040-6376
Hastings RA, Saunders P, Hogben C, et al., 2018, CYCLOPHOSPHAMIDE FOR THE TREATMENT OF REFRACTORY CHRONIC HYPERSENSITIVITY PNEUMONITIS, Winter Meeting of the British-Thoracic-Society, Publisher: BMJ PUBLISHING GROUP, Pages: A191-A191, ISSN: 0040-6376
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- Citations: 2
Mackintosh JA, Adamali H, Patel K, et al., 2018, MEASURES OF OESOPHAGEAL IMPEDANCE ARE NOT PREDICTIVE OF LUNG FUNCTION DECLINE IN INTERSTITIAL LUNG DISEASE, Winter Meeting of the British-Thoracic-Society, Publisher: BMJ PUBLISHING GROUP, Pages: A90-A90, ISSN: 0040-6376
Nolan CM, Maddocks M, Maher TM, et al., 2018, Gait speed and prognosis in patients with idiopathic pulmonary fibrosis: a prospective cohort study, European Respiratory Journal, Vol: 53, Pages: 1-10, ISSN: 0903-1936
The four metre gait speed (4 MGS), a simple physical performance measure and surrogate marker of frailty, consistently predicts adverse prognosis in older adults. We hypothesised that 4 MGS could predict all-cause mortality and non-elective hospitalisation in patients with idiopathic pulmonary fibrosis (IPF).4 MGS and lung function were measured at baseline in 130 outpatients newly diagnosed with IPF. Survival status and non-elective hospital admissions were recorded over one year. We assessed the predictive value of 4 MGS (as a continuous variable and as a binary variable: slow versus preserved 4 MGS) by calculating hazard ratios (HR) using Cox proportional regression, adjusting for potential confounding variables. Receiver Operating Characteristic curves assessed discrimination between the multivariable regression models and established prognostic indices.Continuous 4 MGS and slow 4 MGS were independent predictors of all-cause mortality (4 MGS: HR 0.03 (0.01-0.31), p=0.004; slow 4 MGS: 2.63 (1.01-6.87), p=0.049) and hospitalisation (4 MGS: HR 0.02 (0.01-0.14), p<0.001; slow 4 MGS: 2.76 (1.16-6.58), p=0.02). Multivariable models incorporating 4 MGS or slow 4 MGS had better discrimination for predicting mortality than either the Gender Age Physiology index or Composite Physiologic Index.In patients with IPF, 4 MGS is an independent predictor of all-cause mortality and non-elective hospitalisation.
Macaluso C, Furcada JM, Alzaher O, et al., 2018, The potential impact of azithromycin in idiopathic pulmonary fibrosis, European Respiratory Journal, Vol: 53, Pages: 1-3, ISSN: 0903-1936
Visca D, Mori L, Tsipouri V, et al., 2018, Effect of ambulatory oxygen on quality of life for patients with fibrotic lung disease (AmbOx): a prospective, open-label, mixed-method, crossover randomised controlled trial, Lancet Respiratory Medicine, Vol: 6, Pages: 759-770, ISSN: 2213-2600
BACKGROUND: In fibrotic interstitial lung diseases, exertional breathlessness is strongly linked to health-related quality of life (HRQOL). Breathlessness is often associated with oxygen desaturation, but few data about the use of ambulatory oxygen in patients with fibrotic interstitial lung disease are available. We aimed to assess the effects of ambulatory oxygen on HRQOL in patients with interstitial lung disease with isolated exertional hypoxia. METHODS: AmbOx was a prospective, open-label, mixed-method, crossover randomised controlled clinical trial done at three centres for interstitial lung disease in the UK. Eligible patients were aged 18 years or older, had fibrotic interstitial lung disease, were not hypoxic at rest but had a fall in transcutaneous arterial oxygen saturation to 88% or less on a screening visit 6-min walk test (6MWT), and had self-reported stable respiratory symptoms in the previous 2 weeks. Participants were randomly assigned (1:1) to either oxygen treatment or no oxygen treatment for 2 weeks, followed by crossover for another 2 weeks. Randomisation was by a computer-generated sequence of treatments randomly permuted in blocks of constant size (fixed size of ten). The primary outcome, which was assessed by intention to treat, was the change in total score on the King's Brief Interstitial Lung Disease questionnaire (K-BILD) after 2 weeks on oxygen compared with 2 weeks of no treatment. General linear models with treatment sequence as a fixed effect were used for analysis. Patient views were explored through semi-structured topic-guided interviews in a subgroup of participants. This study was registered with ClinicalTrials.gov, number NCT02286063, and is closed to new participants with all follow-up completed. FINDINGS: Between Sept 10, 2014, and Oct 5, 2016, 84 patients were randomly assigned, 41 randomised to ambulatory oxygen first and 43 to no oxygen. 76 participants completed the trial. Compared with no oxygen, ambulatory oxygen was ass
Shanks A-M, Desai SR, Rice A, et al., 2018, Restrictive lung defects: parenchymal, chest wall and neuromuscular, THORAX, Vol: 73, Pages: 989-991, ISSN: 0040-6376
Patel S, Hastings RA, Lota HK, et al., 2018, Application of a predictive formula for hypoxic challenge testing in patients with interstitial lung disease, 28th International Congress of the European-Respiratory-Society (ERS), Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
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- Citations: 1
Bax S, Jacobs J, Kouranos V, et al., 2018, 10 years' experience of referral of ILD patients with Suspected PH to a National PH Service: Demographics and Outcomes, 28th International Congress of the European-Respiratory-Society (ERS), Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
Kokosi M, Saunders P, Molyneaux PL, et al., 2018, Lung cysts in pulmonary alveolar proteinosis., 28th International Congress of the European-Respiratory-Society (ERS), Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
Nolan CM, Maddocks M, Maher TM, et al., 2018, Four metre gait speed (4MGS) predicts mortality and hospitalisation in IPF, 28th International Congress of the European-Respiratory-Society (ERS), Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
Patel S, Nolan C, Maddocks M, et al., 2018, Upper and lower limb muscle strength in idiopathic interstitial pneumonias (IIP), 28th International Congress of the European-Respiratory-Society (ERS), Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
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