100 Cases in Clinical Medicine

(Rick Simeone) #1

ANSWER 82


This woman has the typical clinical symptoms of polymyalgia rheumatica/giant cell arteritis.
Most patients are over 65 years. The onset of symptoms is often sudden. Patients may pres-
ent primarily with polymyalgia-type symptoms (proximal muscle pain and stiffness most
marked in the mornings) or temporal arteritis symptoms (severe headaches with tenderness
over the arteries involved). Patients may have systemic symptoms such as general malaise,
weight loss and night sweats. Characteristically, the ESR is very elevated (at least 40 mm/h)
and there is a mild anaemia and leucocytosis. The liver enzymes are often slightly raised. In
polymyalgia, the main symptoms are muscle stiffness and pain which may simulate muscle
weakness. The creatine kinase is normal, unlike in polymyositis.


The diagnosis of polymyalgia rheumatica is essentially a clinical diagnosis. A very elevated
ESR is useful. Around 25 per cent of patients with giant cell arteritis have polymyalgia.
When there are headaches and giant cell arteritis is suspected, a temporal artery biopsy
should be performed. However, the histology may be normal because the vessel involve-
ment with inflammation is patchy. Nevertheless, a positive result provides reassurance
about the diagnosis and the need for long-term steroids.


This patient has clear evidence of giant cell arteritis (also known as temporal arteritis
although other vessels are involved), and is at risk of irreversible visual loss either due to
ischaemic damage to the ciliary arteries causing optic neuritis, or central retinal artery
occlusion. The patient should immediately be started on high-dose prednisolone (before the
biopsy result is available). The steroid dose should be slowly tapered according to clinical
features and ESR, but is likely to need to be continued for around 2 years. Bone protection
measures should be part of the management.


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  • Polymyositis

  • Systemic vasculitis

  • Systemic lupus erythematosus

  • Parkinsonism

  • Hypothyroidism/hyperthyroidism

  • Osteomalacia


Differential diagnoses of proximal muscle weakness and stiffness


  • Polymyalgia rheumatica and giant cell arteritis often co-exist.

  • Patients with these conditions have markedly elevated ESR levels.

  • There is a risk of blindness in giant cell arteritis, and steroids should be started immediately.


KEY POINTS

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