The British Thoracic Society Interstitial Lung Disease Registry Programme ANNUAL REPORT 2015/16

Transcription

1 The British Thoracic Society Interstitial Lung Disease Registry Programme ANNUAL REPORT 2015/16 The third report from the BTS Idiopathic Pulmonary Fibrosis (IPF) and Sarcoidosis Registries

2 FOREWORD This third Annual report from the BTS ILD Registry provides the latest information from the rapidly expanding datasets for both IPF and sarcoidosis. As information on these two important conditions continues to build, the picture of the prevalence of IPF and sarcoidosis, their natural history and outcomes on different treatments will become clearer. The information provided via the Registry allows centres to monitor and improve standards of patient care, using robust data to target resources to where they are needed the most. To this end, work is ongoing to make Registry data more accessible through improved communications and the introduction of data dashboards (pages 6,7,14 & 15) which will highlight key messages for clinicians and managers. Data from the registry will add to the growing body of evidence to help all of us caring for patients with these diseases to advise and treat them better. The British Thoracic Society is committed to the ILD Registry and has taken steps over the past year to set in place arrangements for an expansion of the Lung Disease Registry Programme. This will include an enhanced Clinical Advice Service for MDR-TB and, in due course, data collection via a Registry for Silicosis. I am grateful to all those who have helped prepare this report, to all those who contribute data, to the Registry Steering Committee and, in particular, Professor Monica Spiteri who continues to chair this important venture. I would like to encourage colleagues in all centres who see patients with ILD to consider joining the Registry. Dr Lisa Davies Chair, BTS Board of Trustees The BTS ILD Registry is uniquely placed to assist with NHS evidence requirements and the challenges in delivering ILD patient care. Launched in 2013, the Registry has been steadily gaining momentum with 47 hospitals now entering data and containing over 1,200 patient records (978 IPF records and 278 sarcoidosis records). Data analysis continues to yield clinically important findings relevant to the day to day management of IPF and sarcoidosis. These findings, together with the recent organisational survey results, are showcased and discussed in this report. Areas of best practice are clearly demonstrated. Other areas of unmet patient needs and gaps in service require urgent action from healthcare commissioners and policymakers to ensure optimal and efficient quality care and early treatments for all ILD patients. This will significantly improve clinical outcomes. Professor Monica Spiteri, Chair, BTS Lung Disease Registry Steering Committee ISSN Volume 8 Issue 2 November

3 INTRODUCTION This is the third annual report from the BTS Lung Disease Registry. The report provides: (1) An analysis of the clinical metrics, diagnostic trends and service provision patterns for Idiopathic Pulmonary Fibrosis (IPF) and sarcoidosis since the Registry launched in 2013 to the end of September 2016; and (2) The operational challenges faced by NHS Hospital Trusts as reflected in a recent BTS ILD organisational survey in July Examples of best practice achieving the National Institute for Health and Care Excellence (NICE) quality standards for IPF are demonstrated; but other key areas are identified which require either resource investment and/or a change in national policy to expedite early therapeutic interventions and efficient patientfocused services. Such measures will beneficially impact on patients' experiences and outcomes. In line with the overall ambitions of the Society, the BTS IPF and Sarcoidosis Registry is designed as an easy-to-use and secure digital platform to assist clinical practice and improve standards of care for patients. Regular updates have ensured that the Registry remains intuitive to patient and clinician needs and the everchanging healthcare landscape. Individual datasets are regularly checked for quality assurance and updated to reflect published quality standards of care and international guidelines. For example, questions regarding oxygen assessment, palliative care and pulmonary rehabilitation have been revised to ensure more meaningful interpretation of the collected data (changes were implemented in November 2016 and will be fully reflected in the 2017/18 Annual Report). A major recent step forward has been the creation of agile data management dashboards for IPF (pages 6 & 7) and sarcoidosis (pages 14 & 15) which allow interactive snapshots of the clinical records from all participating Trusts and real time identification of the key strengths and gaps in care provision. Specifically the IPF dashboard facilitates benchmarking against nationally set quality standards of care. In addition, we are developing a customised dashboard for release to each participating NHS Trust; this will incorporate readily available metrics on their IPF and sarcoidosis patient cohorts. Captured data will be displayed in an easy-to-understand visual format. At a local level, these dashboards will enable clinicians, managers and commissioners to monitor patient outcomes and service performance, help identify resource limitations and build viable care models. The power of such data displayed visually and succinctly cannot be underestimated. Looking at the fast-evolving healthcare economy, it is inevitable that future service commissioning and support in ILD will be driven by interactive disease-specific dashboards using patient-related specific metrics so that crucial changes in care pathways are successfully implemented. The BTS Registry Programme is key to moving forward priorities in service delivery and setting a world example for standards of care for ILD patients. We wish to thank the British Lung Foundation (BLF) and SILA (the sarcoidosis charity) who have helped us enormously in raising awareness and ensuring that the Registry reflects patients' needs. Professor Monica Spiteri, Chair, BTS Lung Disease Registry Steering Committee BTS Lung Disease Registry Steering Committee Membership 2016: Professor Monica Spiteri, Chair Dr William Chang, Consultant Respiratory Physician Dr Nazia Chaudhuri, Consultant Respiratory Physician Dr Robina Coker, Consultant Respiratory Physician Dr Owen Dempsey, Consultant Respiratory Physician Dr Ian Forrest, Consultant Respiratory Physician Dr Michael Gibbons, Consultant Respiratory Physician Dr Ling-Pei Ho, Consultant Respiratory Physician Dr Toby Maher, Consultant Respiratory Physician Dr Helen Parfrey, Consultant Respiratory Physician Professor Luca Richeldi, Consultant Respiratory Physician Dr Lisa Spencer, Consultant Respiratory Physician Dr Sue Copley, Consultant Radiologist Mrs Sarah Lines, ILD Specialist Nurse Mr Steven Wibberley, corresponding member representing the British Lung Foundation Miss Sally Welham, BTS Deputy Chief Executive Miss Maria Loughenbury, BTS Lung Disease Registry Manager British Thoracic Society reports Vol 8, issue

4 Registry Ethics Approval, Information Governance and Data Security Routine clinical data including demographic and socio-economic data for all adults newly diagnosed with IPF and sarcoidosis from 1 st January, 2013 onwards can be collected; all eligible NHS Trusts (with secondary care and ILD specialist respiratory clinics) across the UK are encouraged to participate. The Registry provides vital information on the natural disease behaviour, mode of patient referral to specialist, diagnostic trends and clinical outcomes of IPF and sarcoidosis. Data also include treatments used and referral to other key services. For the IPF Registry, the data captured includes metrics in line with published NICE IPF quality standards (1). Ethical approval for the British Thoracic Society Interstitial Lung Disease Registry Project (12/EE/0381) was granted on 24 th October, 2012 by the NRES Committee East of England. Patient consent must be obtained before any patient information is entered on to the Registry. Information for patients and copies of consent forms are available on the Registry website (Internet Explorer version 9 and older may have difficulty displaying this site): Participating centres are required to provide confirmation of approval to participate from the Caldicott Guardian before access is granted to allow Registry data entry. Scottish national Caldicott approval was granted in June 2016, therefore Scottish centres are not required to provide Caldicott approval in order to participate in the Registry. All Patient Identifiable data (e.g. name, date of birth, postcode) is encrypted when saved to the Registry system, and will be visible only to the Registry users in the centre that has entered the patient details. No patient identifiable data are available to the BTS Registry Administrators. The British Thoracic Society Information Governance Policy and associated data security policy documents are available on the BTS website at: Who should be entered onto the ILD Registry? 1. For the IPF Registry: Participating centres are requested to enter data on patients who meet the following inclusion criteria: Patients with definite or strongly suspected idiopathic pulmonary fibrosis. Patients with a new diagnosis of IPF made at a clinic visit from 1 st January, 2013 onwards. Patients with a historical diagnosis of IPF seen for the first time in the clinic at the participating centre from 1 st January, Patients with non-idiopathic disease (for example, those with a history of significant asbestos exposure, strong possibility of sub-clinical or evolving connective tissue disease or clear history of exposure to drugs known to cause interstitial lung disease) are not eligible for inclusion in the IPF Registry. Data entry for individual patient records is organised into three sections: Patient information (age, gender, co-morbidities, etc.). Clinical features (clinical information including lung function data available at the time the patient is entered on to the Registry). Follow-up information (clinical information added at 6 months and then 12 month intervals following the entry onto the Registry). 2. For the Sarcoidosis Registry: Participating centres are requested to enter data on patients who meet the following inclusion criteria: Patients with a new diagnosis of sarcoidosis made at a clinic visit from 1st January, 2013 onwards. Patients with a historical diagnosis of sarcoidosis seen for the first time in the clinic at the participating centre from 1 st January, Data entry for individual patient records is divided into three sections: Patient information (age, gender, co-morbidities, etc.). Clinical features on initial diagnosis and at current clinic visit (data available if diagnosis made more than 12 months before clinic attendance) or clinical information available at current clinic visit if diagnosis made less than 12 months prior to clinic attendance. Follow-up information (clinical information added at 12 month intervals following the entry onto the Registry). Who can participate in the IPF Registry and how many are doing so now? The Registry is open to all secondary care institutions in England, Scotland, Wales and Northern Ireland. At the end of September 2016, 40 Trusts/Health Boards (47 sites) had obtained approval to participate and a further 28 centres have the approval process underway. 32 centres have uploaded data to the ILD Registry with over 1,200 patient records (978 IPF records and 278 sarcoidosis records). The current full list of participating NHS Trusts is given on page November 2016 ISSN

5 The BTS Idiopathic Pulmonary Fibrosis Registry Background Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease characterised by variable degrees of inflammation and scarring. The aetiology of IPF is not known, although possible risk factors may include infectious agents, gastro-oesophageal reflux, smoking, exposure to wood dusts and genetic factors. It is more common in men and in people over the age of 70 years. The true incidence and prevalence of IPF are unknown. Data from a recent British Lung Foundation (BLF) report (2) now suggest that in the UK there are over 30,000 people living with IPF and annually there are more than 5,000 new cases; an incidence rate of 12 per 100,000, but rising with age to as high as 72 per 100,000 in those over the age of 70. The prevalence rate is estimated to be 50 per 100,000 with the highest rates in Northern Ireland, North West England, Scotland and Wales. Mortality statistics for IPF are disheartening but could change in the future with the recent advent of anti-fibrotic therapies. In 2012 over 5,000 people in the UK died of IPF. This is virtually 1% of all UK deaths over a year and represents 4.6% of all UK deaths from lung disease (2). The reported median survival in IPF is 3 years from diagnosis (3); as such IPF has a poorer prognosis than many cancers including colon, breast or ovary. Only 20% of IPF patients are reported to survive to 5 years post-diagnosis. Therefore, this relatively uncommon disease punches far above its small incidence in terms of impact on UK mortality. Whilst IPF prognosis sits more comfortably with that of cancer, as a disease its service funding and infrastructural support are significantly below those associated with cancer services. Key developments in IPF care Over the past 3 years there have been a number of major developments in IPF that have affected care in the UK: Clear NICE guidelines for the diagnosis and management of adults with IPF(4) and Quality standards of care for IPF patients published by NICE (1). New IPF treatments have become available (5, 6) Both the NICE documents on IPF management guidelines (4) and quality standards of care (1) have clearly set the bar for the presence of a core multidisciplinary team (MDT) around the patient which vitally specifies interactive specialist clinician and nursing input. It encourages cross-boundary working between hospital and community-based teams (ideally within a network model of care) to provide seamless access to key services such as pulmonary rehabilitation, oxygen provision and palliative support at appropriate disease stages for patients with IPF. In July 2013 the first NICE approved drug to treat IPF became available pirfenidone (Esbriet ) (5) with a second drug, nintedanib (Ofev ), gaining NICE approval in 2016 (6). Both these drugs are first-in-class products and have been shown, in large multinational clinical trials, to reduce annual decline in FVC by 50% (7,8,9). However, in NHS England and Wales, use of both pirfenidone and nintedanib is currently limited to IPF patients with a predicted FVC range between 50 and 80%. This appears to have been a cost effectiveness (rather than clinical effectiveness) decision by NICE, which has subsequently been challenged but still stands. The Scottish Medicines Consortium (SMC) also produced approvals for use of these drugs in Scotland (refs: SMC No. (835/13) and (1076/15)) under slightly different criteria. In Scotland, prescription can be considered for IPF patients with an FVC 80%; effectively there is no lower FVC limit. Evidence is available, however, that demonstrates that these anti-fibrotic drugs are equally as effective at slowing disease progression in patients who have an FVC 80% predicted. The NICE guidance (but not the SMC guidance) also invokes a stopping rule for anti-fibrotic treatment. Any IPF patient on treatment who sustains a 10% fall in their FVC over a 12 month period has to be taken off the drug due to presumed ineffectiveness. This is a further challenging recommendation. Post-hoc analysis of clinical trial data for these therapeutic agents suggests that those patients who experience a significant drop in their FVC over 12 months are less likely to do so again over the next time period, if they remain on the drug (10). The UK is not alone in having certain restrictions placed on the use of these drugs. Various European countries also have their own often different guidance. Interestingly, in the USA - where healthcare is funded very differently to the UK - pirfenidone and nintedanib are available to any IPF patient regardless of their FVC. Notwithstanding the above limitations, the availability of pirfenidone and nintedanib has been vital in improving IPF management. Patients have gone from being untreatable at first presentation to being a group of patients who need to be assessed and referred for treatment. In the UK anti-fibrotic treatments are only available to be prescribed at nominated ILD Specialist Centres. It is yet to be established whether this approach will drive up standards in IPF care delivery and what impact anti-fibrotic treatment will have on future disease morbidity and mortality. The BTS ILD Registry is currently only capturing a small proportion of the incident IPF cases; notwithstanding the IPF Registry dashboard (pages 6 & 7) is already revealing patterns in care delivery, specifically areas that are doing well and others that urgently need more resource and funding. Within the current NHS constraints, investment in IPF services will only be realised if Registry data can demonstrate the full extent of the problem; for this we need to capture data on all our IPF patients. Information on participating centres A brief survey of participating centres was undertaken in July Of the 24 centres that contributed to the survey: 62.5% (15/24) were university /teaching hospitals 37.5% (9/24) were district general hospitals. ILD referrals to centres The average (mean) number of new ILD referrals per annum (based on estimates for the last available 12 month period) were: university/teaching hospital: 380 per annum (range from 120 to 850) district general hospital: 37 per annum (range from 3 to 100) Clinic Type 100% (15/15) of the university/teaching hospitals run a dedicated ILD clinic for ILD patients only. Of the district general hospitals, 33% run a dedicated ILD clinic and in the remaining 67% ILD patients are seen as part of general respiratory clinics. British Thoracic Society reports Vol 8, issue

6 Figure 1a IPF Patient Demographics Dashboard 6 November 2016 ISSN

7 Figure 1b IPF Patient Clinical Information Dashboard British Thoracic Society reports Vol 8, issue

8 IPF Registry Data At 1 st October, 2016, 31 centres had contributed data to the IPF Registry: Patient records - demographic information 978 patient records, an increase of 318 patients compared to 2014/2015. Diagnosis 712 complete clinical records - diagnosis information Follow-up 374 follow-up records in a total of 225 patients The Registry currently holds follow up data on 23% of recorded patients. This is, in part, a result of patients only recently added to the Registry not yet having attended a follow-up appointment. However, the primary reason for this low percentage of recorded follow-up records is likely a result of resource constraints at site level. The full benefits of a longitudinal database such as the Registry may only be realised with the analysis of follow-up data collected consistently, over time, for a large proportion of cases. IPF Registry Analysis of Demographic Data Comments Gender Male Female Age Mean age ± SD (yrs) Aged Aged 70 and over Smoking history Current Ex Never Not known Duration of chest symptoms prior to presentation at the clinic Co -morbidities First degree relatives with IPF 76% (721/953) 24% (232/953) 72.4 ± % (273/946) 64% (607/946) 4% (39/941) 66% (624/941) 28% (259/941) 2% (19/941) 46% (422/921) of patients recorded on the registry had chest symptoms for more than 24 months before their first clinic visit. A significant number of comorbidities were reported in this patient group who are mainly over the age of 60 years old. Of the 778 records where family history was available, 7% (51/778) had at least one first degree relative with IPF. As expected for this disease group. As expected for this disease group. Disease prevalence is much higher in older patients. As the population of the UK ages these numbers will rise. As expected for this disease group. These data demonstrate that patients present late with significant symptom burden and disease severity. Earlier diagnosis of IPF remains a key challenge for healthcare professionals. Approximately 1486 co-morbidities were reported across 926 patients (of whom, 152 reported no co-morbidities) on average giving 1.6 co-morbidities per patient. The incidence of symptomatic GORD was 20%. 8 November 2016 ISSN

9 IPF Registry Analysis of Clinical and Diagnostic Data Referral to first clinic visit (weeks) Lung function at first presentation to clinic FVC predicted >80% FVC predicted 50-80% FVC predicted < 50% HRCT pattern Definite UIP Possible UIP Inconsistent with UIP Surgical lung biopsy Rate UIP Probable UIP Possible UIP Unclassifiable fibrosis 9.9 weeks (10.3 weeks for specialist centres and 9.1 weeks for non-specialist centres). 39% (277/711) 57% (405/711) 4% (29/711) 42% (301/710) 53% (374/710) 5% (32/710) 13% (95/711) of cases had a surgical lung biopsy 70% (66/94) 20% (19/94) 7% (6/94) 3% (3/94) (In one case the outcome was not reported.) Comments IPF patients are being seen well within the NHS 18 week referral to treatment (RTT). However it could be argued that much earlier assessment is required for IPF patients as they have cancer-like outcomes. 43% of IPF patients at presentation/data input point fell outside the current NICE defined criteria for treatment with anti- fibrotic drugs so could not access treatment at that time. Those patients with FVC above 80% predicted are usually monitored until their FVC falls into the treatment range. This cut-off approach based on a single metric to crucial treatment in UK goes against all the efforts being made nationally (from GP to specialist) to assess, diagnose and refer patients early in their disease process. Importantly Registry data show that patients presenting with FVC greater than 80% predicted already exhibit substantial decrease in their diffusion capacity (mean TLCO= 48% predicted) and only a small percentage of these patients (7%) have significant radiological emphysema. Using ATS/ERS criteria (11), these data are similar to other IPF reported cohorts in the literature. Note possible UIP pattern is largest single pattern seen. In older IPF guidelines a lung biopsy showing UIP would have been required to label these patients as IPF. A working diagnosis of IPF based on expert multidisciplinary integration of clinical information (with the premise of excluding known causes of fibrosis, specifically connective tissue disease and hypersensitivity pneumonitis), the clinical assessment of disease behaviour and progression over time and responses to prior therapies is acceptable and considered a more practical approach. These data are similar to the 2015 report. The UK has demonstrated a stable lung biopsy rate in these types of patients for many years now (range 12-15%). Many patients are unsuitable for lung biopsy due to age, frailty and co-morbidities. British Thoracic Society reports Vol 8, issue

10 IPF Registry Treatment Data Current drug treatment Received pirfenidone Received nintedanib Lung transplantation 37% (258/697) 9% (65/697) At entry into the Registry, 5% (38/711) of patients were referred for lung transplantation assessment. From the available followup data, the number of patients referred for assessment increased to 17% (15/90). Comments The data were collected over a period of significant evolution in IPF treatment and therefore represent only a snapshot of the current situation. The results reflect differing access to the two available anti-fibrotic drugs since 2013 and are expected to alter going forward. Nintedanib was only available in the UK at some sites via named Patient Access and Patient in Need programmes since October 2014 and only via the NHS since April In addition, a number of other treatments were recorded that patients were also on that may now not be the current standard of care. Again, this simply reflects the time periods over which data were collected. The majority of IPF patients are potentially not suitable for lung transplant due to age and/or significant co-morbidities. International lung transplant data suggest that, of all the adult lung transplant indicators from January 1995 to June 2014, 24.1% of all transplants were for patients with IPF (12). As the Registry collects more data, it will be interesting to establish what percentage of those referred actually receive a lung transplant. IPF Registry Other key Data Comments Follow-up data reveals: Lung cancer reported rate Entry into clinical trials Hospital admissions Death since entry on to Registry: 1% (4/225) of IPF patients at follow-up were diagnosed with lung cancer since last review (range of follow-up period 6 months to 3.5 yrs). At follow-up, 10% (24/225) of patients had been enrolled on a clinical trial. 8% (17/220) of cases had an acute hospital admission with respiratory disease following initial presentation. Of these: 18% (3/17) for acute exacerbation of IPF; 59% (10/17) for pneumonia/ respiratory tract infection. 6% (59/978) Of these, acute or chronic progression of IPF was the cause of death in 42 cases (71%). A hospitalisation rate of 7% for this disease is low compared to the literature. We therefore expect the true burden of the disease to be shown as the number of recorded follow-ups in the Registry increases. This dataset covers a period of 3.5 years maximum and is incomplete at the moment. This recorded mortality rate is much lower than the annual mortality expected in a generic IPF population. 10 November 2016 ISSN

11 How do the data collected by the IPF Registry relate to NICE Quality Standards for IPF? (1). In this section, data from the IPF Registry and the organisational survey of participating centres conducted in July 2016 is presented in relation to each published NICE quality standard. IPF Quality statement Registry & Survey data analysis Comments QUALITY STATEMENT 1: People are diagnosed with idiopathic pulmonary fibrosis only with the consensus of a multidisciplinary team (MDT) with expertise in interstitial lung disease. QUALITY STATEMENT 2: People with idiopathic pulmonary fibrosis have an interstitial lung disease specialist nurse available to them. QUALITY STATEMENT 3: People with idiopathic pulmonary fibrosis have an assessment for home and ambulatory oxygen therapy at each follow up appointment and before they leave hospital following an exacerbation of the disease. 92% (654/711) of cases were discussed at an ILD MDT 5% (32/711) were not discussed Registry data indicate the mean time from patient referral to MDT was 10 weeks (8.8 weeks for specialist centres and 12.8 weeks for nonspecialist centres). Each of these figures had a high standard deviation (of ±15 weeks) indicating some MDTs are being held before the first clinic appointment and some afterwards. Multidisciplinary Team Meetings (MDT): Data from the organisational survey show that MDT meetings held specifically for ILD take place in 100% (15/15) of the university/teaching hospital centres, and in 33% (3/9) district general hospitals. The organisational survey shows that the composition of the MDT varies between university/teaching hospitals and district general hospitals, which is to be expected. 83% of sites (15/18) included a thoracic radiologist. 94% (17/18) of MDTs included a respiratory nurse (15 ILD Specialist Nurses and 3 Respiratory Nurses, with one site involving both an ILD Specialist Nurse and a Respiratory Nurse with an interest in ILD). Pathologists were included in 89% (16/18) of MDTs, with thoracic pathologists included in 15 cases. The ILD team: The survey found that in 79% (19/ 24) centres, a specialist ILD nurse, or a respiratory nurse with an interest in ILD, was a member of the ILD team. This figure was 100% for university/teaching hospitals and only 44% for district general hospitals. There was a mean of 772 ILD patients per specialist ILD nurse. For university/teaching hospitals the mean was 740 patients per specialist nurse. However, 78% (7/9) of district general hospitals did not have a specialist ILD nurse in their ILD team. At presentation, Registry data show that 23% (162/702) of patients are on oxygen (at least one of ambulatory, LTOT or short burst) and that oxygen assessment takes place in 56% (94/167) of follow-up records. At presentation, 79% (19/24) of centres assess the ambulatory needs of their patients and 92% (22/24) assess their LTOT needs. All centres carried out either ambulatory oxygen or LTOT assessment at presentation. Additionally, oxygen saturation levels of patients are measured at all follow up clinic appointments at 96% of centres. Note: in 19/645 cases records showed that MDT was pending or not known at data entry. 22/32 of those cases not discussed were from district general hospitals (DGHs). Many DGHs are still in process of setting up their local ILD services and establishing consultant leads. The frequency of MDT meetings varies according to the number of referrals (typically from weekly to monthly). NICE IPF Clinical Guidelines mandate that all MDTs should include a thoracic radiologist and an ILD nurse (4). Thoracic-specific radiologists only tend to be available in larger organisations, as expected. These survey data strongly indicate a need for investment in ILD specialist nurses across the UK to manage IPF patient cohorts to optimal clinical standards. More work has to be done to ensure the oxygen needs of all IPF patients across the UK are being met. The apparent underperformance in attaining the NICE quality standard for oxygen assessment is most likely due to time constraints in clinics and reflects the current limited resources to deliver optimal ILD services. British Thoracic Society reports Vol 8, issue

12 QUALITY STATEMENT 4: Pulmonary rehabilitation programmes provide services that are designed specifically for idiopathic pulmonary fibrosis. QUALITY STATEMENT 5: People with idiopathic pulmonary fibrosis and their families and carers have access to services that meet their palliative care needs. Data from the organisational survey of participating centres show that 100% (24/24) of centres routinely assess the pulmonary rehabilitation (PR) needs of their patients. The Registry has not collected data on whether PR programmes provided are designed specifically for IPF patients. In the Registry, patient referral rates for PR show: 53% (373/709) were referred for PR 34% (239/709) were not referred 7% (47/709) patients declined 7% (50/709) information not known All centres have access to palliative care services. However, with the currently available data on the Registry we are unable to provide meaningful comment on how often such services were actually utilised and/or if palliative drugs were part of the 360 degree assessment of IPF patients. Some centres will have much closer working with palliative care services than others with access to for example, breathlessness management clinics and community palliative networks. Of the 24 centres surveyed on the frequency of assessing palliative care needs of their patients in clinic at every visit: 54% always did ; 21% often did; 25% sometimes did. When asked about what palliative care resources centres had good access to and used for their ILD patients, the breakdown was (centres could select as many options as appropriate): Hospital services 71% (17/24) Community services 67% (16/24) Hospice services 58% (14/24) In-house services 50% (12/24) GP services 21% (5/24) Not having good access to a full range of services 8% (2/24) The current Registry data for palliative care referral at first visit show: 3% (22/708) patients were referred; 91% (645/708) were not referred; 5% (32/708) declined referral 1% (9/708) not known Registry questions will be amended in November 2016 to try and tease out why PR referrals may not be happening. Participating sites were invited to comment on any challenges they faced in the delivery of palliative care services. Many sites reported no challenges. Where challenges were reported, common themes included limitations in capacity of available services, resource/ time constraints preventing co-operative working, delays between referral and assessment/delivery of care, and a postcode lottery of availability of services across the UK. 12 November 2016 ISSN

13 The BTS Sarcoidosis Registry Background Sarcoidosis is a disease of unknown aetiology, characterised by small nodules (granulomas) of inflammatory cells which may form in any organ of the body. The presentation, site and severity of sarcoidosis may be related to the age, sex and ethnicity of the patient (13). Pulmonary sarcoidosis is the most common manifestation of this disease, affecting over 90% of patients and accounting for up to one third of ILD in specialist respiratory clinics. The British Lung Foundation s Battle for Breath report (2) indicates that sarcoidosis resulted in nearly 9,000 hospital bed days in the UK in According to the Department of Health, the average bed day is estimated to cost 400. Therefore, hospital admissions for sarcoidosis can be estimated to cost in the region of 3.5 million per annum. Generally reported to be more common in females, the incidence peaks between the ages of 20 and 50 years, with a smaller peak after 60. The disease is more prevalent in Black and Afro-Caribbean subjects, who also suffer more severe disease and a higher mortality. Despite the increased prevalence of sarcoidosis in Black and Afro-Caribbean populations, it should be noted that the majority (two thirds) of patients included in this Registry are Caucasian. This is likely to reflect the populations at participating centres. A significant proportion of patients with active disease are limited in their daytime activities by dyspnoea, fatigue or joint pains. Fatigue affects almost a third of Registry patients at the time of presentation and can be highly debilitating. In addition to its impact on quality of life, fatigue also has associated economic outcomes through reduced productivity and loss of working days. There are no current British, European or North American guidelines for the treatment of fatigue in sarcoidosis, although some studies indicate that treatment with TNF-α inhibitors or neurostimulants may be of value (14, 15, 16). The course of sarcoidosis varies considerably; there is a high rate of spontaneous remission, but chronic disease may occur in up to 30%. To date, it remains difficult to predict which patients will develop chronic, progressive severe disease and how best to manage them. Increased availability of cardiac MRI has prompted growing recognition of myocardial involvement, sometimes presenting with life-threatening ventricular tachycardias. Treatment when indicated, usually for vital organ involvement, generally includes systemic corticosteroids with or without other immunosuppressive agents. Biologicals such as infliximab may be of value, perhaps especially in severe and chronic cases (14, 15) (although further studies would be beneficial). Long term oxygen may be required. Lung transplantation is reserved for those with respiratory failure who fail to respond to maximal therapy, and is limited by organ availability. Other serious complications include pulmonary hypertension, mycetomas, and opportunistic infections resulting from immunosuppression. We anticipate that the BTS Sarcoidosis Registry will enable a greater understanding of the characteristics of the sarcoidosis population across the country, and ultimately lead to both earlier recognition and improvements in diagnosis and management. British Thoracic Society reports Vol 8, issue

14 Figure 2a Sarcoidosis Patient Demographics Dashboard 14 November 2016 ISSN

15 Figure 2b Sarcoidosis Patient Clinical Information Dashboard British Thoracic Society reports Vol 8, issue

16 Registry data At 1 st October, 2016, 22 centres had contributed data to the Sarcoidosis Registry. Patient records - demographic information 278 patient records Diagnosis 203 completed clinical records - diagnosis information Of these there are 78 records for Part B (data from where the initial diagnosis was made more than 12 months before their initial presentation). Follow-up 43 completed follow-up records, for a total of 35 patients. Sarcoidosis Registry Analysis of Demographic Data Comments Gender 41% (109/268) female; 59% (159/268) male. Of note, 59% were male. Although a female preponderance is well-reported, the North American ACCESS study of sarcoidosis reported equal gender distribution in patients presenting under the age of 35, more males than females presenting aged 35-39, and a gradually increasing female preponderance in older subjects (13). British Lung Foundation data from show no gender differences in either incidence or prevalence (17). Age The age distribution is shown in Figure 2a. Mean age at presentation was 49 years, with a standard deviation of ± 13 years. The cohort included in the ACCESS study (13) first presented at a younger age (especially males, who tended to present under the age of 40). A significant number of British patients thus present to respiratory physicians over the age of 50, with some presenting over the age of 80. The diagnosis must therefore be kept in mind when assessing older patients. Ethnicity Referral Smoking history First degree relatives with sarcoidosis 65% (165/254) were British and Caucasian. Just over half (53%) the patients were referred from respiratory physicians in secondary care, which may reflect lack of familiarity in primary care. This could potentially contribute to delays in diagnosis. Just 8% (16/189) of patients were smokers at presentation and 26% (50/189) were ex-smokers. Only 3% (7/222) reported having a first degree relative with sarcoidosis. This proportion is likely to represent the populations from which the Registry data were taken. This proportion is lower than the previous year, but it is too early to determine whether this reflects increasing awareness in primary care and a greater willingness to refer early. Co-morbidities Over one third of patients (42%, 80/189) had no co-morbidity at the time of their current presentation. The most common co-morbidities were asthma, hypertension, diabetes and obesity. These conditions are also highly prevalent in the general population. They need to be taken into consideration when choosing therapies and predicting their impact in individual patients. 16 November 2016 ISSN

17 Sarcoidosis Registry Analysis of Clinical and Diagnostic Data Comments Symptoms at current presentation Diagnosis made Biopsy Sarcoidosis incidental Blood test Current treatment HRCT pattern Inclusion in clinical trial The most common symptoms at current presentation were cough and breathlessness, with fatigue, joint pain and eye symptoms also frequently reported. Improved treatment of fatigue in sarcoidosis, and early recognition of uveitis, will be essential to improving management. In 64% (121/189) of patients, the initial diagnosis was made through biopsy, and in 26% (49/189) the diagnosis was made on the basis of HRCT. In the remainder, the diagnosis was made on the basis of clinical features alone. The most common feature on HRCT was the presence of widespread pulmonary nodules. Two thirds (67%) of diagnoses were confirmed through biopsy, in keeping with recommended guidelines. EBUS was used in 27% of biopsies in 2013, rising to 48% in 2015 (overtaking mediastinoscopy and transbronchial biopsy as the most common biopsy techniques for confirming pulmonary disease). In one third of patients (31%, 59/188), the diagnosis of sarcoidosis was made as a result of an incidental finding during the course of investigations for another condition. The most common finding from blood tests was peripheral lymphopaenia, identified in over half of patients (57%) at presentation. Patients presented with mean serum IgG of 14.87g/l (ranging from 5.7g/l to 67g/l). 27% of patients had IgG levels above the normal range of 5-16g/l. The mean ACE level at presentation was 67 IU/l with 50.6% of patients having an ACE level in excess of 55 IU/l. At follow up the mean ACE level had dropped to 52. In the majority of cases, patients were either not started on treatment, or managed with systemic corticosteroids. Alternative immunosuppressant agents included a wide range of options with no particular preponderance of any one agent. Nodules were by far the most common feature identified in HRCT, found in almost three quarters of patients (74%) at presentation. Ground glass density was a relatively infrequent feature, found in only 5% of patients at presentation. While no patients were currently recruited into a clinical trial, 88% (167/189) would be considered for recruitment subject to inclusion criteria. Fatigue may be associated with small fibre neuropathy. Patients may benefit from treatment with TNF inhibitors or neurostimulants. Further studies are required to evaluate efficacy in this setting, as well as the role of pulmonary rehabilitation and other exercise programmes (16). The diagnostic yield of EBUS was not assessed and may be a helpful question in future questionnaires. It supports a requirement for EBUS-TBNA trained respiratory physicians to be available in all centres (18, 19, 20). This highlights the importance of educating healthcare professionals more widely as to how to recognise the condition. Although not specific to sarcoidosis, peripheral lymphopaenia should raise the question of whether the patient might have sarcoidosis, within the correct clinical context. It may also be of value in estmating disease activity, as activated T cells home to areas of inflammation, e.g. lungs. High levels of serum IgG have been observed in many sarcoidosis patients in some centres. This measurement may be used as a marker of disease activity and the database provides a means to examine if this is a phenomenon observed across different centres and a larger cohort of patients. Normal serum ACE reference ranges will likely be affected by ACE polymorphism (21). This broadly reflects current British Thoracic Society guidance on the management of sarcoidosis (22). The data collected from the BTS Sarcoidosis Registry should inform the design of future clinical trials. Questions which need addressing include when to start treatment, what dose to use, what tapering regime to use, when to add alternative immunosuppression and which agent to use first, how to long to continue treatment, and how to treat associated, often debilitating symptoms such as fatigue. British Thoracic Society reports Vol 8, issue

18 References 1. National Institute for Health and Care Excellence, Quality Standard 79: Idiopathic pulmonary fibrosis in adults (January 2015); 2. British Lung Foundation, The Battle for Breath (2016) 3. Bjoraker, J. A., et al. (1998) Prognostic significance of histopathologic subsets in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 157(1): National Institute for Health and Care Excellence, Clinical Guidance 163: Idiopathic pulmonary fibrosis: The diagnosis and management of suspected idiopathic pulmonary fibrosis (June 2013) 5. National Institute for Health and Care Excellence, TA282: Pirfenidone for treating idiopathic pulmonary fibrosis (April 2013) 6. National Institute for Health and Care Excellence, TA379 Nintedanib for treating idiopathic pulmonary fibrosis (January 2016) 7. King, T.E., et al. (2014) The ASCEND Study: A randomized, double-blind, placebo controlled trial of pirfenidone in patients with Idiopathic Pulmonary Fibrosis (IPF). The New England Journal of Medicine 370(22): Noble, P.W., et al. (2011) Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. Lancet 377(9779): Richeldi, L., et al. (2014) Efficacy and safety of nintedanib in Idiopathic Pulmonary Fibrosis. The New England Journal of Medicine 370(22): Nathan, S.D., et al. (2016) Effect of continued treatment with pirfenidone following clinically meaningful declines in forced vital capacity: analysis of data from three phase 3 trials in patients with idiopathic pulmonary fibrosis. Thorax 71: Raghu, G., et al. (2011) An official ATS/ERS/JRS/ALAT statement: Idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med 183(6): International Society for Heart and Lung Transplantation (2015) The Registry of the International Society for Heart and Lung Transplantation: Thirty second official adult lung and heart-lung transplantation report 2015; Focus theme: early graft failure. JHLT 34(10): Baughman, R.P., et al. (2001) Clinical Characteristics of Patients in a Case Control Study of Sarcoidosis. Am J Respir Crit Care Med 164: Baughman, R.P., et al. (2006) Infliximab therapy in patients with chronic sarcoidosis and pulmonary involvement. Am J Respir Crit Care Med 174: Hostettler K.E., Studler U., Tamm M. and Brutsche M.H. (2012) Long-term treatment with infliximab in patients with sarcoidosis. Respiration 83(3): Drent M., Lower E.E., de Vries J. (2012) Sarcoidosisassociated fatigue. ERJ 40: British Lung Foundation, Sarcoidosis Statistics (accessed 1 st November, 2016) National Institute for Health and Care Excellence, Clinical Guidance 121: Lung Cancer: diagnosis and management (April 2011) Gupta, D., et al. (2014) Endobronchial ultrasoundguided transbronchial needle aspiration vs conventional transbronchial needle aspiration in the diagnosis of sarcoidosis. Chest 146(3): Medford, A.R.L. (2014) Endobronchial ultrasound-guided versus conventional transbronchial needle aspiration: time to re-evaluate the relationship? Journal of Thoracic Disease 6(5): Tomita, H., et al. (1997) Polymorphism in the Angiotensin- Converting Enzyme (ACE) Gene in Sarcoidosis. Am J Respir Crit Care Med 156: Bradley, B., et al. (2008) Interstitial lung disease guideline: the British Thoracic Society in collaboration with the Thoracic Society of Australia and New Zealand and the Irish Thoracic Society. Thorax 63: Supplement November 2016 ISSN

19 The following organisations are currently participating in the BTS Lung Disease Registry our thanks to all involved: England Aintree University Hospital NHS Foundation Trust Countess of Chester Hospital NHS Foundation Trust County Durham and Darlington NHS Foundation Trust Croydon Health Services NHS Trust Gateshead Health NHS Foundation Trust George Eliot Hospital NHS Trust Gloucestershire Hospitals NHS Foundation Trust Harrogate and District NHS Foundation Trust Heart of England NHS Foundation Trust Hinchingbrooke Health Care NHS Trust Hull and East Yorkshire Hospitals NHS Trust Imperial College Healthcare NHS Trust King s Health Partners (Kings, Guys & St Thomas ILD service) Leeds Teaching Hospital NHS Trust Liverpool Heart and Chest Hospital NHS Foundation Trust London North West Healthcare NHS Trust Newcastle upon Tyne Hospitals NHS Foundation Trust Norfolk and Norwich University Hospitals NHS Foundation Trust North Bristol NHS Trust North Middlesex University Hospital NHS Trust Northern Devon Healthcare NHS Trust Northumbria Healthcare NHS Foundation Trust Nottingham University Hospitals NHS Trust Oxford University Hospitals NHS Foundation Trust Papworth Hospital NHS Foundation Trust Peterborough & Stamford Hospitals NHS Foundation Trust Portsmouth Hospitals NHS Trust Royal Devon & Exeter NHS Foundation Trust Royal Free London NHS Foundation Trust The Royal Wolverhampton NHS Trust Sheffield Teaching Hospitals NHS Foundation Trust Taunton & Somerset NHS Foundation Trust University Hospital of South Manchester NHS Foundation Trust University Hospital Southampton NHS Foundation Trust University Hospitals Coventry & Warwickshire NHS Trust University Hospitals of Morecambe Bay NHS Foundation Trust University Hospitals of North Midlands NHS Trust Scotland Aberdeen Royal Infirmary, NHS Grampian Glasgow Royal Infirmary, NHS Greater Glasgow & Clyde Lorn & Islands District General Hospital, NHS Greater Glasgow & Clyde Royal Alexandra Hospital, NHS Greater Glasgow & Clyde Vale of Leven District General Hospital, Greater Glasgow & Clyde Wales Betsi Cadwaladr University Local Health Board If you would like to know more about the BTS Interstitial Lung Disease Registry see the details on our website at: Acknowledgements: The BTS ILD Registry Programme is funded by the British Thoracic Society. A grant ( ) from the Healthcare Quality Improvement Partnership (HQIP) contributed to the initial development of the ILD Lung Disease Programme and this support is gratefully acknowledged. The Society is grateful for financial assistance provided from Boehringer Ingelheim and InterMune for the enhancement of the data collection software (2014). The BTS Interstitial Lung Disease Registry Programme is supported by: The British Lung Foundation SILA the UK Sarcoidosis Charity British Thoracic Society reports Vol 8, issue