Polymyalgia Rheumatica & Giant Cell Arteritis

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

• Giant cell arteritis: headache, jaw claudication, polymyalgia rheumatica; 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 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

POLYMYALGIA RHEUMATICA

• Anemia and elevated acute- phase reactants (generally ESR > 30 mm/h and CRP > 0.5 mg/dL) are present in most cases