Lisa Spencer

Ambulatory oxygen in idiopathic pulmonary fibrosis: of what benefit?

To the Editors: We read with interest the Letter by Visca et al. [1], recently published in the European Respiratory Journal , which described improvement in 6-min walk distance with ambulatory oxygen in patients with interstitial lung disease (ILD). We have looked specifically at the effects of ambulatory oxygen on walk distance in patients with idiopathic pulmonary fibrosis (IPF), and here describe a practical way of ensuring patients are prescribed an optimum flow rate of ambulatory oxygen. Between 2004 and 2007, we conducted a retrospective review of anonymised data, studying the effect of ambulatory oxygen on the distance walked in patients with IPF …

Perceptions, experiences and needs of patients with idiopathic pulmonary fibrosis

Aims To understand the perceptions, needs and experiences of patients with Idiopathic Pulmonary Fibrosis. Background Idiopathic pulmonary fibrosis is a progressive interstitial lung disease, with a mean life expectancy similar to some forms of cancer of 2–4 years from diagnosis. Unlike the cancer literature, which is rich with studies exploring the needs of their disease group, few publications exist on patient needs with this severe fibrotic lung disease. Design A Qualitative study which took place between 2007–2012. Methods Seventeen patients with a multidisciplinary team confirmed diagnosis of Idiopathic Pulmonary Fibrosis, with moderate to advanced disease severity and six of their informal carers were interviewed. An interview topic guide was developed by the researchers and service user group. The interviews were audio-recorded, semi-structured and took place at a regional respiratory and lung transplant centre in North West England. Interviews were transcribed verbatim and data analysed using Framework Analysis. Findings Three main themes were identified: ‘Struggling to get a diagnosis’; ‘Loss of the life I previously had’; and ‘Living with Idiopathic Pulmonary Fibrosis’. Patients reported struggling to get a diagnosis and coping with a life-limiting, rapidly progressive illness with no good treatment and few support structures. Conclusions There is an urgent need for a better understanding of the difficulties faced by people with Idiopathic Pulmonary Fibrosis and their carers. This can be used to develop better supportive care in the United Kingdom and ultimately improve the quality of life of these patients.

Cross-Atlantic modification and validation of the A Tool to Assess Quality of Life in Idiopathic Pulmonary Fibrosis (ATAQ-IPF-cA).

Rationale The A Tool to Assess Quality of Life in Idiopathic Pulmonary Fibrosis (ATAQ-IPF) was developed in the USA to assess health-related quality of life in patients with IPF. It is likely that some of the original ATAQ-IPF items perform differently when applied in different countries. This paper reports results of a study conducted to identify the need to refine the content of the ATAQ-IPF to minimise cross-country bias between the USA and the UK. Methods The ATAQ-IPF and other study measures were completed by patients attending specialist IPF clinics in the USA and UK. Rasch analysis was used to determine which items performed differently across countries (USA vs UK) and refine the original ATAQ-IPF to an instrument without cross-country bias (ATAQ-IPF-cA). Preliminary validation of the modified instrument was examined by assessing correlations between ATAQ-IPF-cA scores and scores from dyspnoea-specific patient-reported outcome (PRO) measures. Results 139 patients with IPF (USA=74; UK=65) participated in the study. A total of 41 items and 4 domains were removed from the original, 86-item instrument to yield the 43 items and 10 domains of the ATAQ-IPF-cA. Each domain had good fit to the Rasch model, internal consistency was comparable to the corresponding domains for the original ATAQ-IPF, and validity was supported by significant correlations between its scores and scores from dyspnoea-specific PROs. Conclusions The reliability and validity of the substantially shortened ATAQ-IPF-cA are acceptable and comparable to the original instrument. We recommend use of the ATAQ-IPF-cA in IPF studies in which participants are enrolled from the USA and UK.

Phenotypes of organ involvement in sarcoidosis

Sarcoidosis is a highly variable, systemic granulomatous disease of hitherto unknown aetiology. The GenPhenReSa (Genotype–Phenotype Relationship in Sarcoidosis) project represents a European multicentre study to investigate the influence of genotype on disease phenotypes in sarcoidosis. The baseline phenotype module of GenPhenReSa comprised 2163 Caucasian patients with sarcoidosis who were phenotyped at 31 study centres according to a standardised protocol. From this module, we found that patients with acute onset were mainly female, young and of Scadding type I or II. Female patients showed a significantly higher frequency of eye and skin involvement, and complained more of fatigue. Based on multidimensional correspondence analysis and subsequent cluster analysis, patients could be clearly stratified into five distinct, yet undescribed, subgroups according to predominant organ involvement: 1) abdominal organ involvement, 2) ocular–cardiac–cutaneous–central nervous system disease involvement, 3) musculoskeletal–cutaneous involvement, 4) pulmonary and intrathoracic lymph node involvement, and 5) extrapulmonary involvement. These five new clinical phenotypes will be useful to recruit homogenous cohorts in future biomedical studies. Five new clinical phenotypes of sarcoidosis have been identified by analysing organ manifestations of 1932 patients http://ow.ly/UYLC30jpUkq

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

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 Kings 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 associated with significant improvements in total K-BILD scores (mean 55·5 [SD 13·8] on oxygen vs 51·8 [13·6] on no oxygen, mean difference adjusted for order of treatment 3·7 [95% CI 1·8 to 5·6]; p<0·0001), and scores in breathlessness and activity (mean difference 8·6 [95% CI 4·7 to 12·5]; p<0·0001) and chest symptoms (7·6 [1·9 to 13·2]; p=0·009) subdomains. However, the effect on the psychological subdomain was not significant (2·4 [-0·6 to 5·5]; p=0·12). The most common adverse events were upper respiratory tract infections (three in the oxygen group and one in the no-treatment group). Five serious adverse events, including two deaths (one in each group) occurred, but none were considered to be related to treatment. INTERPRETATION Ambulatory oxygen seemed to be associated with improved HRQOL in patients with interstitial lung disease with isolated exertional hypoxia and could be an effective intervention in this patient group, who have few therapeutic options. However, further studies are needed to confirm this finding. FUNDING UK National Institute for Health Research.

Safety and tolerability of nintedanib for the treatment of idiopathic pulmonary fibrosis in routine UK clinical practice

Nintedanib, a tyrosine kinase inhibitor approved for the treatment of idiopathic pulmonary fibrosis (IPF), reduces annual forced vital capacity (FVC) decline in these patients by ∼50% with combined analysis of data from clinical trials showing a trend towards reduction in mortality [1–3]. Nintedanib prescription criteria for the treatment of IPF vary between countries and in 2016, the National Institute for Health and Care Excellence approved nintedanib in England and Wales for patients with an FVC between 50% and 80% predicted [4]. Prior to this approval, nintedanib was available through a manufacturer-funded programme with varying prescribing criteria. We investigated the safety and tolerability of nintedanib in UK clinical practice during this period in which FVC prescription criteria differed from those now available in routine practice. In IPF, commencement of antifibrotic therapy in patients with preserved lung volume may increase the duration of therapy http://ow.ly/4HeM30lFS67