British Thoracic Society (BTS) Interstitial Lung Disease (ILD) Registry Programme December 2015 Update Professor Monica Spiteri University Hospitals of North Midlands .

BTS ILD Registry ambitions To provide an easily accessible secure system for the national collection of longitudinal demographic and clinical information on two ILD conditions in the first instance: Idiopathic Pulmonary Fibrosis (IPF) Sarcoidosis Launched in February 2013. At 23 November 2015: 37 active participating sites; 29 other sites had approval in process. Each participating hospital can generate graphical reports displaying data for their hospital for each registry (IPF and sarcoidosis). All captured data can be exported to Excel for local analysis; enabling audit of local practice and benchmarking against national standards of care. Coming soon – facility to upload to Registry website from Excel spreadsheets.

ILD Registry Steering Committee Membership: Professor Monica Spiteri (Chair) Dr Lisa Spencer, Dr Toby Maher, Dr Robina Coker, Dr Michael Gibbons, Dr Ling-Pei Ho, Dr Helen Parfrey, Dr Fiona Black, Dr William Chang, Dr Nazia Chaudhuri, Dr Sue Copley, Dr Owen Dempsey, Dr Ian Forrest, Professor Luca Richeldi

Steering Committee role Actively manage the Registry to enable all centres to enter patient-level data relevant to disease diagnosis and on-going care (with patient consent). Ensure database information is in line with patient needs and ever-changing clinical landscape. Oversee Registry governance and address requests for data. Seek funding opportunities and collaborations to invest in continued system maintenance and upgrades. Develop specific proposals with researchers to further knowledge in IPF and sarcoidosis; wherever possible, seek project adoption onto NIHR portfolio. NICE, National Institute for Health and Care Excellence; NIHR, National Institute for Health Research

BTS ILD Registry Report 2014/15 First report to include highlights from data collected since launch in 2013. At 1 October 2015: 660 IPF patient records submitted (consented patients attending first clinic appointment from 1 Jan 2013). At 1 October 2015: 181 Sarcoidosis patient records submitted (consented patients with new or historical diagnosis of sarcoidosis seen at clinic from 1 Jan 2013).

Idiopathic pulmonary fibrosis: NICE Quality Standards (2015) Statement 1. People are diagnosed with IPF only with the consensus of MDT team with expertise in ILD Statement 2. People with IPF have an ILD specialist nurse available to them Statement 3. People with IPF 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 Statement 4. Pulmonary rehabilitation programmes provide services that are designed specifically for IPF Statement 5. People with IPF and their families and carers have access to services that meet their palliative care needs

What current key information does the Registry provide? % of patients by age at diagnosis, gender, smoking habit % of patients with co-morbidities suffered Symptom duration prior to seeing consultant and diagnosis (lag time to referral) % of patients by therapy (% receiving what drugs/oxygen) [NICE Q.S.3] % of patients whose diagnosis is confirmed at MDT [NICE Q.S.1] % of IPF patients referred for pulmonary rehabilitation [NICE Q.S.4] % of patients referred to palliative supportive care [NICE Q.S.5] % of patients referred for lung transplantation % of patients in clinical trials Mortality data

IPF – Clinical presentation Annual Report 2014/15

Who is referring IPF patients to the clinic? Annual report data 2014/15

IPF patients: Lung Function at presentation

IPF diagnosis – Has this case been discussed at MDT?

IPF patient referral for Pulmonary Rehabilitation

IPF patient – Referral to palliative care

IPF patient– oxygen assessment

BTS SURVEY on ILD SERVICES A brief survey of Registry participating centres was undertaken in July 2015 to complement information obtained via the Registry. Of the 20 centres that contributed to the survey: 70% (14/20) were university /teaching hospitals 30% (6/20) were district general hospitals.

BTS Survey results: ILD Referrals The average (mean) number of new ILD referrals per annum (based on estimates for the last available 12 month period) were: university/teaching hospital: 354 p.a. (range 100 to 564) district general hospital: 83 p.a. (range from 20 to 200)

BTS Survey results: ILD Clinics 100% (14/14) of the University/teaching hospitals run a dedicated ILD clinic for ILD patients only. Of the district general hospitals, 50% run a dedicated ILD clinic and the remaining 50% ILD patients are seen as part of general respiratory clinics.

Conclusions on current IPF Care At least 45% of IPF patients at presentation have an FVC that falls outside current NICE- defined criteria for treatment with Pirfenidone (across UK) and Nintedanib (in Scotland). Registry data shows 6.3% of patients with an IPF diagnosis were not discussed at ILD MDT. 23% of IPF patients at presentation are on oxygen (ambulatory, LTOT or short burst), with oxygen assessment taking place in 72% follow-up records. 46% of IPF patients were referred for Pulmonary Rehabilitation (PR). 95% of the IPF patients were not referred to palliative care services. A specialist ILD nurse was a member of the ILD team in 85% of Registry-enrolled centres taking part in the 2015 BTS-led organisational survey.

BTS Sarcoidosis Registry At 1 October 2015: 181 Sarcoidosis patient records submitted (consented patients with new or historical diagnosis of sarcoidosis seen at clinic from 1 Jan 2013). 38% female, 62% male 71% British/Caucasian 10% smokers at presentation 58% one or more co-morbidities at presentation

Sarcoidosis patients – age at clinic presentation

Sarcoidosis patients– symptoms at presentation

Histological Diagnosis of Sarcoidosis Initial diagnosis – patients with historical diagnosis more than 12 months before presentation at clinic; Current diagnosis – at presentation in clinic

Sarcoidosis Registry Data Conclusions Over 70% of sarcoidosis patients are Caucasian, which may not reflect ethnic mix in inner city populations, but could reflect current distribution of Registry centres. Over 80% of patients were referred from respiratory physicians in secondary care, possibly reflecting delayed recognition in primary care. Over 60% of patients had a biopsy to confirm diagnosis, using EBUS-TNA as preferred procedure. Over half of sarcoidosis patients had significant co-morbidity, including diabetes and hypertension, which needs to be taken into account when deciding treatment . Most commonly used treatment was oral corticosteroids, with a wide range of alternative immunosuppressants used as second-line agents. There is still no clear consensus on which is the best second line drug.

ILD REGISTRY crucial to Patient Care “We are working in an NHS in which demand grows faster than funding, and if we are going to argue the corner for our patients with respiratory disease it is vital for us to have good data. We need information about the size of the problem, and we need to be able to show where current care is sub-optimal but could be improved with new resource, targeting this for maximal benefit to patients. The ILD Registry will serve this purpose and more”. Dr Bernard Higgins, Chair BTS Executive Committee

Become involved! Contact registry@brit-thoracic.org.uk for more participation details Once Caldicott approval from your hospital is obtained, access to Registry website is provided Visit the BTS dedicated website: https://www.brit-thoracic.org.uk/audit-and-quality-improvement/bts-lung-disease-registry-programme/ for more information and useful simple ‘get-started’ tips (A4 sheet for clinic use)