Fast track diagnosis and management of GCA Bhaskar Dasgupta Consultant Rheumatologist Honorary Professor, Essex University Visiting Professor, Anglia Ruskin University Southend University Hospital Bhaskar.dasgupta@southend.nhs.uk NHS England Webinar 24 June 2016

Case Study Southend University Hospital NHS Trust Preventing blindness by fast-tracking suspected Giant Cell Arteritis patients to immediate treatment Outstanding Best Practice Award 2016 What was the process for the Award? Shortlisting Visit by the BSR – rheumatologist, CEO, Health Economist,patient Presentation and report Award adjudicated by experts within BSR, RCP President,NICE representatives Distinguishing features – reduce sight loss, collaborative with primary care and other specialists, patient centred, evidence based,financial savings

Reduction of sight loss in GCA BSR Best Practice Workshop The challenge The solution Evidence of improved outcomes (clinical, patient experience, financial) Next steps What was learned from the experience What we would do differently/tips 30 minute presentation, 30 minute demo of ultrasound Royal Botanical Gardens Birmingham June 29 2016

Blood supply of the Optic Nerve Why is the optic nerve so vulnerable in GCA? Intimal hyperplasia blocking arterial lumen Lack of collaterals Very vulnerable to ischemic injury Elderly patients with possibly pre-existing atherosclerosis factors

Fast track pathway to reduce sight loss in GCA Permanent vision impairment is seen in 15-25% of GCA some of whom have bilateral involvement. Irreversible ischemic complications such as sight loss occur early, prior to steroid therapy This is preventable with steroid therapy Need fast-track treatment analogous to stroke for GCA with ischemia Evidence – Reggio Emilia study Note results from VCRC survey of sight loss done in 2015 Similar results from Yellow card survey of sight loss in the UK

A case history 75 year old lady Figure: 20x16cm Parietal-occipital scalp lesion representing scalp necrosis in 75 year old female patient newly diagnosed Giant Cell Arteritis taking high dose steroids for over 4 weeks. Her temporal artery biopsy taken fourteen days after commencing Prednisolone 60mg demonstrated chronic inflammatory infiltrates and giant cells were noted around the elastic lamina. Images courtesy of Dr Shyanthi Pattapola (Rheumatology Spr Southend Hospital)

Flourescein angiogram showing Choroidal ischemia in GCA Fluorescein angiogram in a patient with visual loss from GCA. In these early phase pictures note the marked darkness on the left side of the pictures denoting delayed choroidal filling of fluorescein due to severe choroidal ischaemia on the nasal half of the fundus

Case 2: the lucky one !! 76y male (independent, very active) 8 weeks - night sweats 3 weeks - constitutional symptoms (malaise, weak, polymyalgia) headache and neck pain blurred vision Right eye Treated for presumed sinusitis CRP 20, ESR 24

The lucky one !! Admitted as possible GCA Reviewed by Ophthalmology (R optic neuritis) Commenced immediately IV methylprednisolone Next day vision returned US: “halo” sign positive (L common temporal, L parietal, L frontal, R common temporal) Discharged on oral prednisolone after 3/7 IV methylprednisolone Biopsy positive

Outcomes of acute sight loss Loss of independence Loss of confidence Depression Loss of mobility Residential care Medical complications such as hip fractures, infections Impact on family

Cost of Blindness Low vision clinic assessment, provision of low vision aids, training in their use Low vision rehabilitation in activities for daily living Acute admission to geriatric ward for broken hip, total hip replacement, rehabilitation Registration as blind or partially sighted Community care—provision of a home care worker Social security benefits, in particular attendance allowance Blind person’s tax allowance Treatment and support of depression in the elderly C Meads and C Hyde Br J Ophthalmol 2003 87: 1201-1204

What is to be done? AION in GCA : ‘Stroke in the eye’ Need a campaign analogous to ACT-FAST for stroke? ‘Time is Sight’ instead of ‘Time is Brain’ ‘’Symptom to steroid time’ as a performance marker

Obstacles to early recognition Delayed presentation Delayed referral - failure to recognize symptoms/urgency Delayed therapy - Multiplicity of referral routes

Temporal Artery Ultrasound How long does the examination take?

TA ultrasound halo Definition of halo Homogenous, hypoechoic wall thickening, well delineated towards the luminal side, visible both, in longitudinal and transverse planes, most commonly concentric in transverse scans. The thickened arterial wall remains visible upon compression, i.e. the echogenicity contrasts hypoechoic due to vasculitic vessel wall thickening in comparison to the mid- to hyperechoic surrounding tissue.

Axillary vasculitis (Large-Vessel GCA) Similar definition for axillary artery halo

FTP Awareness Campaign Pathway was publicised to the sources of patient referral Reminders every 3 months Regular time-to-learn sessions arranged for GPs PMRGCAUK and the groups: newsletters, meetings and to newly diagnosed patients contacting the helpline.

Results: Sight loss Year Total number of GCA patient Number of GCA patients who lost vision (%) 2003-2008 61 19 (29) 2009 17 4 (23.5) 2010 26 7 (26.9) 2011 30 7 (23.3) 2012 33 3 (9) Note major reduction in the absolute numbers So it is not better results due to treatment of milder disease Similar findings have been replicated from Norway

Comparisons of Pathways   GCA – conventional (n=46) GCA –fast track (n=33) p-value GP Referral to review [days]* 3 (0-71) 1 (0-8) 0.068 Symptom to diagnosis [days]* 21 (1-196) 14 (0-168) 0.85 We also observed a reduction of the median ‘symptom to diagnosis time’ by 1 week (statistically not significant due to low patient numbers and wide range data); and the median time from referral by GP to review in rheumatology clinic, was also reduced. *median (range)

Giant Cell Arteritis – A Cost-Benefit Analysis Study

Incremental cost effectiveness ratio Costs and Savings EQ-5D QALYs ICER is a tool helps us to compare different pathways to determine which is the best use of resources. Involves calculating: Costs and savings, EQ-5D health questionnaire (looking at health outcomes) , Quality adjusted life years (QALY’s= life years x QOL) In order to determine to Incremental cost effectiveness ratio (ICER). This is to be calculated for the FTGCA pathway and the conventional referral route. Difference in the overall costs between the conventional and FTP per patient ICER = Difference in the number of QALY’s gained between conventional and FTP per patient

Costs and Savings GP Appointments ↓ Diagnosis and Treatment Inpatient stay Readmissions Drugs ↑ Training and awareness to doctors QALY’s gained Cost Benefit

Average cost of diagnosing and treating a patient with suspected GCA A difference of £400 per patient treated for suspected GCA. The ICER of implementing the FTP = -£840 per QALY. ‘FTP saves money’ Conventional pathway £2,600 Fast-Track pathway £2,200

Fast track clinics & non-GCA diagnoses

Service Performance and Outcomes On introduction of the FTP, the proportion of patients suffering from sight loss dropped significantly from 37% to 9% when compared with the conventional pathway.1 A reduction in the time from referral to rheumatology review was likely a major driving force behind the improved clinical outcomes observed, with 79% of patients ultimately diagnosed with GCA seen within one working day.1 Patients referred using the FTP were diagnosed 2–3 days sooner than those in the conventional pathway, limiting exposure to precautionary high-dose steroids associated with debilitating side-effects.1 Patil, P, M. Williams, W. Maw, K. Achilleos, S. Elsideeg, C. Dejaco, F. Borg, S. Gupta, B. Dasgupta (2015) Fast-track pathway reduces sight loss in giant cell arteritis: results of a longitudinal observational cohort study. Clin Exp Rheumatol. 2015 Mar-Apr;33(2 Suppl 89):S-103-6. Epub.

Financial Performance and Outcomes Implementation of the FTP was associated with cost-savings to the Trust, with a reduction in the average overall cost of diagnosing and treating a patient with suspected GCA from £2.6k to £2.2k per patient. In a cost-effectiveness analysis to compare the FTP with the conventional pathway, patients gained on average 2.6 quality-adjusted life years (QALYs) by avoiding the complication of sight loss. The economic evaluation determined that the FTP dominated the conventional pathway (−£840 per QALY).

Patient Focus and Satisfaction The FTP aims to ensure improved public and professional awareness of GCA, conduct rapid specialist reviews and initiation of treatments, with the aim to improve patient care by preventing visual loss and unnecessary exposure to potential harmful treatment. Clearly defined referral pathways and well-coordinated teams ensure that care is patient-centred. Demonstrable close links with patient groups and uniform backing from for the FTP. Improved recruitment to GCA-related trials including GIACTA and SIRRESTA. Public education initiatives to improve awareness including through PMRGCAuk, Fight for Sight and ARMA.

Commissioning Implications Secondary prevention (King’s Fund 2013 Commissioning Priority1) – the FTP demonstrates a significant improvement in the number of patients who suffer sight-loss as a result of an avoidable complication of GCA. Care co-ordination through integrated health and social care teams (King’s Fund 2013 Commissioning Priority1) – improved communications between primary and secondary care ensure patients are referred quickly and appropriately. Effective medicines management (King’s Fund 2013 Commissioning Priority1) – through timely referral and diagnosis, patients avoid unnecessary side-effects of high-dose steroids. Managing urgent and emergency activity (King’s Fund 2013 Commissioning Priority1) – through working closely with GPs and committing to advancing the education around GCA, referrals into secondary care are more streamlined and appropriate. Furthermore, the FTP allows early diagnosis of serious non GCA pathology that may mimic GCA Naylor, C., Imison, C., Addicott, R., Buck, D. and Goodwin, N., Transforming our health care system: ten priorities for commissioners. London: The King’s Fund; 2013.

Target Outcomes Target Improvement Measure(s) Faster diagnosis Symptom to diagnosis time –time from symptom onset to GP diagnosis Faster initial treatment Symptom to steroid time – time from symptom onset to start of steroid therapy Faster referral Referral to review time – time to GP referral to rheumatology review Faster assessment Review to biopsy time – rheumatology review to temporal artery biopsy Review to ultrasound time – rheumatology review to temporal artery ultrasound Reduced incidence of GCA related disease GCA disease control, cardiovascular and other co-morbidities, ischemic complications such as sight loss, strokes, resolution of inflammatory markers, Reductions in GCA related sight loss Percentage of sight loss Improved cost effectiveness in GCA treatment Reductions achieved in total healthcare cost of GCA care versus baseline cost Improved patient safety Adverse events related to disease – ischemic complications, large vessel involvement Adverse events related to steroids e.g fractures, diabetes, glaucoma, cataracts, Improved patient satisfaction Better social care with reduced use of benefits and social services Improved patient quality of life HRQOL and EQ5D scores (EQ5D measures ability in 5 domains – pain/discomfort, anxiety/depression, mobility, self-care, usual activities) , sleep disturbance and vision deficit questionnaires

Next steps : For patients A patient-friendly booklet which will be available as a DVD, handout or web link which educates patients to the nature of GCA discusses signs and symptoms with need for urgent action to prevent ischaemic complications explains the assessment process explains the nature of various tests Treatment and results to expect Online Webinar

Next Steps : for clinicians an online package consisting of documents, powerpoint presentations and webinar videos, addressing: Education and training and questions for subjective evaluation Signs and symptoms of GCA Tests and investigations education e.g. temporal and axillary artery ultrasound/ temporal artery biopsy Advice and education on pathway and referral routes Treatments and monitoring protocols Shared-care advice and protocols Support for co-morbidities that may affect or change management Latest research and news

Thank you Case Study Southend University Hospital NHS Trust Preventing blindness by fast-tracking suspected Giant Cell Arteritis patients to immediate treatment Outstanding Best Practice Award 2016 Thank you Thank you

Supra –aortic vascular ultrasound

Temporal artery ultrasound ’Halo sign’ - Meta-analysis evaluating 23 studies – sensitivity 87%, specificity 96% Requirements- trained sonographer, linear probes covering 9-15 MHz, standaridised color adjustment, consider wall swelling, stenosis, occlusion Axillary artery can be included Karassa et al Ann. Intern. Med. 2005; 142, 359–369

Superficial temporal artery

Temporal Artery Ultrasound

TAUS – Settings summary Vascular preset Use colour not power Doppler Colour frequency 8-12 MHz Steer colour box PRF 2-3 KHz Adjust colour gain to be in vessel lumen Test settings in 30 normals Finalise settings with US equipment reps

Highest B mode frequency Large image not too dark Vascular preset Steer color box PRF 2-3 kHZ Color freq ½ to 2/3 grey scale scale

Axillary scan in LVV

„Halo“ sign vs. clinical diagnosis Results: pSens 81% pSpec 94%

„Halo“ sign vs. TAB Results: pSens 73% pSpec 84%