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Key Features

Essentials of Diagnosis

  • Age over 50 years

  • Elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CPR)

  • Polymyalgia rheumatica: pain and stiffness in shoulders and hips lasting for several weeks without other explanation

  • Giant cell arteritis: headache, jaw claudication, polymyalgia rheumatica, visual abnormalities; without treatment, permanent blindness may occur

General Considerations

  • Polymyalgia rheumatica and giant cell arteritis probably represent a spectrum of one disease and frequently coexist

  • The important difference between the two conditions

    • Polymyalgia rheumatica alone

      • Is not a systemic vasculitis

      • Does not cause blindness

      • Responds to low-dose (10–20 mg/day) prednisone therapy

    • Giant cell arteritis can cause

      • Blindness

      • Aortitis

      • Large artery complications

      • Requires high-dose prednisone therapy (40–60 mg/day)

Demographics

  • Giant cell arteritis is more common in northern Europeans and their descendants

  • Incidence of the disease increases with each decade of life

Clinical Findings

Symptoms and Signs

POLYMYALGIA RHEUMATICA

  • Pain and stiffness of the shoulder and pelvic girdle areas

  • Fever, malaise, and weight loss

  • Anemia and a markedly elevated sedimentation rate are almost always present

  • Muscle pain much greater than muscle weakness

GIANT CELL ARTERITIS

  • Headache, scalp tenderness, visual symptoms, jaw claudication, or throat pain

  • The temporal artery is usually normal on physical examination but may be nodular, enlarged, tender, or pulseless

  • Blindness

    • Results from occlusive arteritis of the posterior ciliary branch of the ophthalmic artery

    • Ischemic optic neuropathy may produce no funduscopic findings for the first 24–48 hours after the onset of blindness

  • Asymmetry of pulses in the arms, a murmur of aortic regurgitation, or bruits near the clavicle resulting from subclavian artery stenoses identify an affected aorta or its major branches

  • Forty percent of patients with giant cell arteritis have nonclassic symptoms at presentation, primarily respiratory tract problems (most frequently dry cough), mononeuritis multiplex (most frequently with painful paralysis of a shoulder), or fever of unknown origin

  • The fever can be as high as 40°C and is frequently associated with rigors and sweats

  • Unexplained head or neck pain in an older patient may signal the presence of giant cell arteritis

Differential Diagnosis

POLYMYALGIA RHEUMATICA

  • Rheumatoid arthritis

  • Polymyositis

  • Chronic infection, eg, endocarditis

  • Plasma cell myeloma

  • Malignancy

  • Fibromyalgia

  • Polyarteritis nodosa

GIANT CELL ARTERITIS

  • Migraine

  • Glaucoma

  • Takayasu arteritis

  • Uveitis

  • Carotid plaque with embolic amaurosis fugax

  • Trigeminal neuralgia

Diagnosis

Laboratory Tests

POLYMYALGIA RHEUMATICA

  • Elevated acute-phase reactants (generally ESR > 30 mm/h and CRP > 0.5 mg/dL) are universally present

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