Symptoms and signs

• Aged ≥50 yr
• Sudden onset of unilateral headache
• Scalp tenderness
  
  • temporal artery tenderness
  • nodularity
  • reduced pulsations
• Visual symptoms e.g. 
  
  • amaurosis fugax
  • diplopia
  • blurred vision
  • changes to colour vision
• Constitutional symptoms e.g. 
  
  • fever
  • sweats
  • weight loss
• Limb/jaw claudication
• Polymyalgic symptoms 
  
  • pain and stiffness of hip and shoulder girdles

Investigations

• FBC
• U&Es
• CRP (≥10)
  • if inflammation levels are lower but high clinical suspicion present, discuss with on-call rheumatologist
• ESR (≥50)
• LFT
• Bone profile
• Serum electrophoresis
• Bence-Jones proteins 
  
  • especially if ESR raised out of proportion to CRP
• Screening tests for serious infections 
• Chest X-Ray
• Dipstick urine and MC&S 
  
  • if positive, blood cultures
• Temporal artery ultrasound scan (TA USS), also includes axillary artery scan
  
  • perform as soon as possible and ≤2 weeks of commencing prednisolone
  • performed at Bradwell Hospital (refer to rheumatology on-call for review and organising TA USS only)
• Temporal artery biopsy (TA Bx)
  
  • perform as soon as possible and ≤2 weeks of commencing prednisolone
  • rheumatology/ophthalmology to refer to vascular surgery 
• If patient has constitutional symptoms, PET CT scan/MR angiography of aorta/CT angiography of aorta and branches 
  
  • involvement of aorta and branches may be asymptomatic

Differential diagnosis

• In the younger age group Takayasu arteritis
• Other causes of headaches (migraine, sinusitis) 
• Occipital neuralgia
• Systemic vasculitis

End organ damage – visual loss, stroke constitutes severe case

• Methylprednisolone 500 mg–1 g daily IV for 3 days 
  
  • do not wait for blood test results before administering initial dose
  • if IV not possible commence prednisolone 60-100 mg oral daily for 3 days

No end organ damage

• Jaw claudication/visual disturbance
  
  • commence prednisolone 60 mg daily
• No jaw claudication/visual disturbance
  
  • commence prednisolone 40 mg daily

Who to contact

If visual symptoms present

• Contact ophthalmology on-call

If no visual symptoms present

• Contact rheumatology on-call (0900–1900 hr, via switchboard or as an inpatient Careflow referral)

Monitoring during prednisolone taper

• Relapse of headache symptoms or jaw/tongue claudication
  
  • increase steroids back up to 40–60 mg daily and discuss with rheumatology on-call
• Constitutional symptoms persist or recur (weight loss, fever, night sweats, anaemia, persistent acute phase response, new/recurrent PMR symptoms, limb claudication, abdominal pain or back pain) discuss with rheumatology on-call:
  
  • PET CT scan
  • increasing steroids (after PET CT if possible) or adding in steroid sparing agents 

• Refer to rheumatology outpatients
• Continue prednisolone 40–60 mg daily for 3 weeks (until normalisation of inflammatory markers), then
  
  • reduction at 10 mg every 2 weeks until daily dose 20 mg, then 
  • reduction at 2.5 mg every 2 weeks until daily dose 10 mg, then 
  • reduction at 1 mg every month
• If unable to reduce steroids, steroid sparing agents (methotrexate, leflunomide) and possibly a biologic agent (tocilizumab is available for use for a limited period) to be considered at rheumatology outpatient review
• Commence gastroprotection with omeprazole 20 mg daily
• If aged <70 yr, commence bone protection with calcium and vitamin D supplements (Calcichew® D3 Forte or Adcal® D3)
• If patient aged ≥70 yr, no contraindication and tolerated, commence oral bisphosphonates (alendronate 70 mg once weekly)
• Check FRAX (https://frax.shef.ac.uk/) score to plan need for a DEXA scan on Careflow
• No indication for anti-platelet treatment (follow National guidance for secondary prevention of cardiovascular and other atherosclerotic diseases, where applicable)
• No indication for cholesterol lowering agent (follow National guidance for secondary prevention of cardiovascular and other atherosclerotic diseases, where applicable)

• If no visual symptoms - rheumatology follow-up
• If visual loss present - joint ophthalmology and rheumatology follow-up 
• Joint follow-up with other involved specialities (stroke/neurology/vascular surgery)