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Key Features

Essentials of Diagnosis

  • Age over 50 years

  • Giant cell arteritis: headache, jaw claudication, polymyalgia rheumatica, visual abnormalities, and a markedly elevated erythrocyte sedimentation rate (ESR)

  • Polymyalgia rheumatica: pain and stiffness in shoulders and hips

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 is that polymyalgia rheumatica alone does not cause blindness and responds to low-dose (10–20 mg/day) prednisone therapy, whereas giant cell arteritis can cause blindness and large artery complications and requires high-dose therapy (40–60 mg/day)

Demographics

  • Both affect patients over age 50

  • 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 heard 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 (TEMPORAL) ARTERITIS

  • Migraine

  • Glaucoma

  • Takayasu arteritis

  • Uveitis

  • Carotid plaque with embolic amaurosis fugax

  • Trigeminal neuralgia

Diagnosis

Laboratory Tests

  • An elevated ESR, with a median result of about 65 mm/h, occurs in more than 90% of patients with polymyalgia rheumatica or giant cell arteritis

  • Most patients also have a mild normochromic, normocytic anemia and thrombocytosis

Imaging Studies

  • Ultrasonography can detect abnormalities in inflamed temporal arteries but its results are highly operator dependent

  • Magnetic ...

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