ILD Registry in the UK: IPF & Sarcoidosis databases Professor Monica Spiteri Chair, BTS Lung Registry

The BTS ILD Registry Provides an easily accessible, secure system for the national collection of longitudinal demographic and clinical information on two ILD conditions: Idiopathic Pulmonary Fibrosis (IPF) and Sarcoidosis Launched in February 2013; all UK NHS hospitals invited to participate and enter data following approval and patient consent. Each hospital can generate reports and data displays for their own site. Captured data enables audit of both national and local practices, and benchmarking against national quality standards of care. Key areas in care pathways identified as exemplars of best practice or requiring attention and further investment.

BTS Registry sites across the UK Launched in February 2013 At 30th November 2016 47 NHS Hospitals actively participating; 29 others in approval process 1,052 IPF patient records and 286 sarcoidosis records.

IPF diagnosis: HRCT scan and surgical biopsies Data correct to 30/11/16

IPF: NICE Quality Standards (2015) Statement 1. People are diagnosed with IPF only with the consensus of an MDT 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

IPF Registry & organisational data 2016 shows: Mean+SD time from referral to first clinic appointment is 10.0±7.6 weeks. Could it be argued that earlier assessment is mandated for IPF patients as they have cancer-like outcomes? 91% of patients have been discussed at MDT prior to data entry, and 74% of centres have a specific ILD MDT. Mean time from patient referral to MDT Is 10 weeks (8.8 weeks for specialist centres and 12.8 weeks for non-specialist centres); some MDTs are held before first clinic visit and some after. 61% of centres have a dedicated ILD nurse specialist. Urgent need for investment in ILD specialist nurses across UK to manage IPF patients to optimal clinical standards. 92% of centres routinely assess LTOT needs of their patients; whilst 79% also routinely assess ambulatory oxygen needs. More work and investment required to ensure ambulatory oxygen needs of all IPF patients across the UK are being met. Palliative care assessment is: always carried out in 57% of centres; often in 22% of centres; only sometimes in 21% of centres. A ‘postcode lottery’ of service availability. Challenges include limitations in capacity of available services; resource/time constraints with delays between referral and assessment

Pulmonary Rehabilitation Referral in IPF Data correct to 30/11/16 Registry questions will be amended in Dec 2016 to tease out why PR referrals are not happening to the required standard; could it be that IPF patients are not prioritised for PR and lose out to COPD patients?

Patient stratification according to NICE cut-offs for treatment NICE Recommended Cut-offs for therapy - FVC% predicted <50% 50 to 80% >80% % Patients in each FVC group at first clinic presentation 4 58 38 Mean + SD % predicted TLCO 28.3 + 16.3 37.7 + 14.1 47.8 + 15.5 % Patients with emphysema on HRCT – over 25% lung volume 7 Data correct to 30/11/16

Recorded Mortality in IPF patients At present, all cause mortality rate is 6% Data correct to 30/11/16

SARCOIDOSIS

Diagnosis of sarcoidosis Data correct to 30/11/16

Radiological presentation Data correct to 30/11/16

Was diagnosis confirmed on biopsy? Data correct to 30/11/16

Treatment at presentation Data correct to 30/11/16

Huge thanks to: BTS: Miss Maria Loughenbury – BTS Lung Disease Registry Manager Miss Sally Welham – BTS Deputy Chief Executive ILD Registry Steering Committee: Dr Robina Coker, Dr Lisa Spencer, Dr Nazia Chaudhuri, Dr Sue Copley, Dr Helen Parfrey, Dr Michael Gibbons, Mrs Sarah Lines, Dr Ling-Pei Ho, Dr William Chang, Dr Owen Dempsey, Dr Ian Forrest, Dr Toby Maher, Professor Luca Richeldi All participating sites: A full list of all participating sites is available on the BTS website.

And thank you to: The British Thoracic Society for central funding and support for the ILD Registry. British Lung Foundation and SILA (sarcoidosis charity) for helping us to keep patients’ interests at the heart of the Registry. HQIP for some unrestricted seed funding towards development of the platform (2011-13) and Boehringer Ingelheim and InterMune for limited funding towards additional developments (2014).

Information for patients and copies of consent forms are available on the Registry website: https://www.brit-thoracic.org.uk/standards-of-care/lung-disease-registries/bts-ild-registry/