Giant cell arteritis and Polymyalgia rheumatica

1. Giant cell arteritis and Polymyalgiarheumatica Dr. S. Bhalara Rheumatology unit West Herts Trust

2. Polymyalgiarheumatica • Cause? • Not a muscle disease (despite the name) Capsulitis • synovitis/bursitis - Imaging/histology • Synovitis, tenosynovitis and oedema in hands and feet Vasculitis • Subclinical vasculitis of temporal arteries seen • Vascular production of inflammatory mediators – IL-1, TGF-B, IL-2 (even without cellular infiltrate) • Circulating activated macropages/monocytes

3. Polymyalgiarheumatica • Often very acute onset • Bilateral (symmetrical) • Pains may be widespread but proximal limb girdle predilection • Chest wall symptoms • Morning stiffness /systemic symptoms

4. Link with arthritis • Synovitis – seronegative arthritis often seen • Late onset rheumatoid arthritis – PMR very common presenting feature Gonzalez J Rheumatol 2000;27:2179 • Inflammatory oedema – RS3PE

5. Investigations: acute phase • ESR, CRP, IL-6 • Normochromic, normocytic anaemia • Reactive thrombocytosis • Liver enzymes (esp alk phos)

6. Investigations: acute phase • ESR < 40mm/hr in 7-22% Helfgott Arthritis Rheum 1996;39(2):304 Gonzalez Arch Int Med 1997; 157(3):317 Gabriel J Rheumatol 1999;26(6):1333 • ? CRP more reliable • Steroid trial (10-20mg for 1-2 weeks)

7. Differential diagnosis • Malignancy • Paraneoplastic musculoskeletal syndrome • Metastatic disease • Myeloma • Fibromyalgia • Vitamin D deficiency • Hypothyroidism • Cervical and lumbar spondylosis/spinal stenosis • Bursitis/tendonitis

8. PMR therapy Steroids • 15-20mg prednisolone • maintain 2-4 weeks after resolution of symptoms • Taper by 10% every 2-4 weeks • Once below 10mg/day by approx 1mg/month • Benign diagnosis – adjust according to symptoms – ESR/CRP guides but does not dictate therapy Steroid sparing drugs • 20% steroid resistant (must exclude paraneoplastic syndrome or CTD/RA) • MethotrexateCaporali Ann Int Med 2004;141:568 • azathioprine/mycophenolate/leflunomide • NSAID therapy alone is acceptable

9. PMR prognosis No increase in mortality Survival in 315 PMR patients longer than controls Myklebust et al Scand J Rheumatol 2003;32::38 • Use steroids/immunosuppresants with caution • Recurrence rate approx 20% • PMR causes increased bone turnover in it’s own right – Osteoporosis prophylaxis

11. Madonna and Cannon van derPaele – Jan Van Eyck (1436)

12. Discussion: How should we manage GCA in West Herts? Suspected GCA Primary carestart high dose steroids visual symptoms Secondary care AAU urgent OPD Review Opthalmology acute medicine Rheumatology Neurology COE TA Biopsy Opthalmology Gen surgeons Vascular surgeons Follow up and Opthalmology Rheumatology COE General Med Neurology steroid taper GP

13. Temporal Arteritis Diagnostic and Management Pathway • Dr Hannah Cowling GP Watford • Dr S Bhalara Consultant Rheumatologist West Herts NHS Trust

14. Temporal Arteritis Diagnostic Pathway Later onset signs and less frequent presentations Ishaemic Optic Neuropathy Thickened Temporal Artery Central Retinal Artery Occlusion 3rd, 4th, 6th Nerve Palsies Arthralgia Intracerebral Artery Involvement Angina or Myocardial Infarction Temporal Headache (localised ) Scalp Tenderness (over temporal artery) Jaw Claudication Transient Visual Disturbance PolymyalgiaRheumatica Malaise Anorexia Fever ESR >50 Age >50 If four or more of the above symptoms/signs are present (must include 3 of those marked in bold) indicates high suspicion of Temporal Arteritis Visual Symptoms Prednisolone 1mg/kg 60-80mg daily Same Day referral to opthalmologist Aspirin +PPI Start Bone Protection (egAlendronate) Consider Amphotericin Lozenges No Visual Symptoms Prednisolone 40-60mg daily PPI Start Bone Protection (egAlendronate) Consider Amphotericin Lozenges Remember Bone Protection should continue for 6 months after stopping steroids Refer Urgently for Temporal Artery Biopsy Please Fax a referral letter, marked ‘For Temporal Artery Biopsy’ to: Mr R Awad , Vascular Surgeon, Watford General Hospital, Fax – 01923 Please state date of starting steroids as biopsy should be done within 2 weeks.

15. Calcium and cardiovascular disease Bolland et al BMJ Meta-analysis of of 11 RCTS • 12000 – healthy postmenopausal women • Ca supps > 500mg/day • hazard ratio for non fatal MI = 1.31 (95% CI 1.02-1.67) (ie 30% increase) Caveats MI not a primary or secondary end point in any study CA+ Vit D not analysed No increase in MI mortality No increase in other cardiovascular events eg strokes

16. Recommendations • Those on calcium and vitamin D – no change • Calcium alone + coronary risk factors/past history of IHD • Review need for calcium and either stop or replace with calcium and vitamin D. Assess dietary intake of Calcium and give dietary advice •  New osteopenic/osteoporotic patients • no change in practice but if dietary intake likely to be good check daily intake formally with diet chart as it may be possible to withhold supplementation. • Search for and treat Vitamin D deficiency aggressively (this will improve dietary calcium absorption).

17. Bisphosphonate drug holiday after 5 yrs Rx • aseptic necrosis of jaw, atypical femoral neck fractures, Oesophageal Ca, only occur with prolonged therapy Schwartz et al J Bone Min Res 2007;22:S1057 ALN 10 years continuously Vs 5 yrs on then 5 yrs off Dexa at year 5 :- Rel risk of fracture in continued group vs discontinued group (at year 10) Fem neck T score ≤ -2.5 0.5 (0.26-0.96) Fem neck T score ≥ -2.0 1.41 (0.75-2.66)

18. Thank you