Dr. Alper is medical director of clinical reference products for EBSCO Publishing, Inc., in Ipswich, Mass., and editor-in-chief of DynaMed (www.dynamicmedical.com), a database of comprehensive updated summaries covering nearly 2,000 clinical topics. Ms. Meskimen is a clinical instructor, University of Missouri-Columbia, and a reviewer for DynaMed.

Description
• Nonarticular rheumatic disorder resulting in muscle pain and stiffness

ICD-9 code
• 725 polymyalgia rheumatica

Areas of involvement
• Shoulders, pelvic-girdle musculature, torso
• Occasionally peripheral joints in upper extremities


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Who is most affected
• Age >50 years; females; almost exclusively in whites

Incidence/prevalence
• Incidence varies from 12.7/100,000 in Italy to 112.6/100,000 in Norway
• Average annual incidence in Olmsted County, Minn., per 100,000 persons >50 years is 52.5 (62 in women and 40 in men)

Associated conditions
• Giant cell arteritis (temporal arteritis)
— 40%-60% with giant cell arteritis have PMR symptoms
— 10%-33% of PMR patients have giant cell vasculitis(temporal arteritis)
• Bursitis, synovitis
— peripheral joints, upper extremity
• Degenerative disease of other joints

History
• Severe aching and stiffness (without weakness) for more than one month
— Diffuse aching pain (symmetrical polymyalgias and arthralgias)
— Often neck and shoulders; sometimes hips, thighs, arms
— Distal musculoskeletal involvement common
— Usually insidious onset but can be very acute
— Worse after motion, more severe in morning; morning stiffness lasts about an hour.
• Proximal muscle pain: difficulty getting out of bed, rising from chair, climbing onto a train, combing hair; unlike problems with small-joint function, e.g., buttoning clothes
• Almost all patients have systemic symptoms.
— Most frequently fever
— Fatigue and malaise are prominent in older patients.
— Weight loss common
— Night pain and night sweats
• Headache suggests temporal arteritis.
• Ask about functional impact, e.g., limited ability to work, meet family responsibilities, or enjoy leisure time; in one third, aching and stiffness severe enough to limit self-care

Physical exam
• May have tender, stiff muscles
• Not truly weak; apparent weakness related to pain
• Occasionally synovitis of wrists and hands
• Diffuse edema of hands and feet (more common in men)
• Check temporal artery pulse; palpate scalp for tenderness.

Diagnostic criteria
• Jones and Hazleman criteria (most PMR patients have five or more) — Muscular shoulder and pelvic-girdle pain
— Morning stiffness
— Symptoms for at least two months
— Erythrocyte sedimentation rate (ESR) >30 mm/hr
or C-reactive protein >6 µg/µL
— Absence of inflammatory arthritis or malignancy
— Absence of objective signs of muscle disease
— Prompt dramatic response to systemic corticosteroids
• Bird/Wood 1979 criteria may be most sensitive diagnostic criteria for PMR (three or more required for diagnosis) — Bilateral shoulder pain or stiffness
— Duration of illness less than two weeks
— Initial ESR >40 mm/hr
— Stiffness lasting longer than one hour
— Age >65 years
— Depression and/or weight loss
— Bilateral upper arm tenderness

Differential diagnosis
• Other rheumatic conditions: rheumatoid arthritis, polymyositis, systemic lupus erythematosus, polyarteritis nodosa, dermatomyositis, fibromyalgia
• Other neuromusculoskeletal conditions: cervical spondylosis, cervical myelopathy, adhesive capsulitis, osteoarthritis of shoulder, myopathies, Parkinson’s disease
• Malignancies, including multiple myeloma
• Depression
• Thyrotoxicosis, hypothyroidism
• Infections, e.g., viral myalgias, subacute bacterial endocarditis

Testing to consider
• ESR, may be normal (<30 mm/hr).
• Rule out other disorders: complete blood count, platelets, creatine kinase, rheumatoid factor, antinuclear antibody.
• Temporal artery biopsy if headache, jaw claudication, temporal artery pain, scalp tenderness, visual loss, or thrombocytosis
• Temporal artery ultrasound to diagnose giant cell arteritis

Prognosis
• Up to one third of patients develop temporal arteritis within one year; risk factors include new headache, onset after age 70 years, abnormal temporal arteries, elevated liver enzymes, jaw claudication, and amaurosis.

Treatment overview
• Instruct patient to immediately report any visual or neurologic symptoms.
• Low-dose corticosteroids (e.g., prednisone 10-20 mg/day initially, then tapered as tolerated)
— IM methylprednisolone acetate every three weeks may achieve similar remission rates with fewer fractures and less weight gain than oral prednisone daily.
— Shoulder corticosteroid injections may reduce pain and duration of morning stiffness.
—Duration of corticosteroid therapy varies.

  • One to two years (but mean 28 months in one series)
  • Slower tapering associated with lower relapse rate
  • Discontinue corticosteroids after about 6-12 symptomless months and normal ESR on low maintenance dose (e.g., prednisone 2.5-5 mg once daily).

• Methotrexate 10 mg weekly may allow shorter duration of steroids and reduced steroid dose.
• Aggressive osteoporosis prevention

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