Takayasu s arteritis. Justin Mason. Professor of Vascular Rheumatology Imperial College London Hammersmith Hospital

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1 Takayasu s arteritis Justin Mason Professor of Vascular Rheumatology Imperial College London Hammersmith Hospital

2 I have nothing to disclose.

3 Takayasu s clinical features Early phase non-specific features visual disturbance Later occlusive phase bruits carotid, abdo, subclavian hypertension abnormal retinal vasculature Late complications

4 Takayasu s Arteritis Vascular Injury ** *

5 Carotid A wall volume Takayasu s Arteritis Outcomes Current medical management sub-optimal 74% compromised daily activity, 23% unable to work Mortality 5-15% at 15 years (33% in severe subset) Age at scan Keenan et al Arthritis Rheum 2009

6 Red flags In patients under 40 years of age Unexplained acute phase response (CRP, ESR) Carotidynia Hypertension Arterial bruit Discrepant blood pressure in arms (>10mmHg) Absent/weak peripheral pulse(s) Limb claudication Angina Mason JC, Nat Rev Rheumatol Jul;6(7):

7 Takayasu arteritis - Diagnosis Identification of pre-stenotic disease FDG-PET MR angiography CT angiography High resolution US Andrews and Mason Rheumatology 2007; 46: 6-15

8 18 F FDG-Positron Emission Tomography 18 F -deoxyglucose glucose analogue High uptake in metabolically active cells Proportional to rate of glucose uptake - tumour cells - inflammatory cells - brain cells - infected cells PET 8mSv msv CT 7 msv

9 CT FDG-PET in LVV

10 US imaging in Takayasu s Early pre-stenotic disease Normal Add normal carotid doppler Concentric thickening Common carotid/vertebral Response to therapy TA Giordana P et al. Circulation 2011;124:

11 ITAS Score Multidisciplinary management of TA Prednisolone mg/kg/day +/- methylprednisolone Methotrexate/Azathioprine/MMF Pulsed cyclophosphamide 6 months BP/Pulm BP CV risk factors Surgery Anti-TNF x 7 pts 6 responders - ITAS, TADs 6 5 One relapse on infliximab Pre Post One non-responder to infliximab Anti-IL-6 x 3 - ITAS 3 responders

12 Takayasu s Arteritis Follow-up Prevention of disease progression FDG-PET MR angiography CT angiography High resolution US 1. CRP and ESR useful but insensitive 2. No specific antibodies Andrews and Mason Rheumatology 2007; 46: 6-15

13 Follow-up with MRA and CTA + * * * * CTA JC Mason Nature Reviews Rheum 2010

14 MRA imaging for follow-up

15 Takayasu s Arteritis Intervention

16 Takayasu s Arteritis Intervention Malik et al Heart 2003

17 Aged 21 Stents Occluded FDG-PET MTX + pred FDG-PET Surgery Intervention role of imaging

18 Endovascular approaches Pre- Subclavian Renal Iliac Aorta Coeliac SMA Long irregular stenoses Short focal stenoses in non-inflamed arteries Post-

19 Future Prospects

20 11 C-PK11195 imaging LVV A B Baseline TBR 1.63 TBR 1.63 C D 6 mths Rx TBR 0.87 TBR 0.87 Pugliese, Mason, Camici JACC 2010

21 Challenges Cardiology: Pulses, BP, AR, angina, carotidynia, heart failure, CRP 1. Optimal diagnostic imaging 2. Optimal follow-up imaging and serology 3. Controlled clinical trials including imaging 4. Accurate activity and damage indices 5. Evidence for Rx benefit on long-term outcome

22 Conclusions Combination of imaging modalities: Improve diagnostic certainty PET/CT localise inflammation Identify pre-stenotic disease Improve disease monitoring & therapy Demonstrate benefits of therapy

23 Acknowledgements Prof P Camici Dr O Rimoldi Dr F Pugliese Dr O Gaemperli Prof A Al-Nahhas Dr N Keenan Prof R Mohiaddin Prof D Pennell MRC Cyclotron Unit, HH San Raffaele, Milan Dept of Nuclear Medicine, HH Cardiovascular MR Unit, Royal Brompton Hospital

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25 Patient no. Takayasu - outcomes MRA outcome data 9.8% 8.7% Morbidity Upper limb claudication 34% Hypertension 18% CVA/TIA 12% Angina 10% Dizziness 10% Lower limb claudication 8% Pulmonary artery hypertension 6% Blindness 2%

26 FDG-PET limitations Mild Moderate Severe FDG-PET size of vessel >4mm ANCA-associated vasculitis, PAN Intense signal in the brain Superficial location signal at body/air Disease relapse Limitations in sensitivity Webb et al EJNM Molecular Imaging 2004; 31:

27 Carotid A wall volume Integrated MRA a quantitative tool Normal TA FMD Aortic stiffness CMR Age at scan Keenan et al Arthritis Rheum Nov 2009