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Author: Geofrey Nochimson, MD, Consulting Staff, Department of Emergency Medicine, Sentara Careplex Hospital

Geofrey Nochimson is a member of the following medical societies: American College of Emergency Physicians

Editors: Michael S Beeson, MD, MBA, FACEP, Professor of Emergency Medicine, Northeastern Ohio Universities College of Medicine; Program Director, Emergency Medicine Residency, Summa Health System; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; Gino A Farina, MD, Program Director, Associate Professor of Clinical Emergency Medicine, Department of Emergency Medicine, Long Island Jewish Medical Center, Albert Einstein College of Medicine; John D Halamka, MD, MS, Associate Professor of Medicine, Harvard Medical School, Beth Israel Deaconess Medical Center; Chief Information Officer, CareGroup Healthcare System and Harvard Medical School; Attending Physician, Division of Emergency Medicine, Beth Israel Deaconess Medical Center; Robert E O'Connor, MD, MPH, Professor and Chair, Department of Emergency Medicine, University of Virginia Health System

Author and Editor Disclosure

Synonyms and related keywords: PMR, giant cell arteritis, rheumatic disease, temporal arteritis, stiffness, aching, pain in the proximal muscle groups, stiffness in the proximal muscle groups, Gel phenomenon, stiffness after prolongedinactivity, polymyalgia rheumatica

Background

Polymyalgia rheumatica (PMR) is a clinical syndrome characterized by severe aching and stiffness in the neck, shoulder girdle, and pelvic girdle. It is classified as a rheumatic disease, although the etiology is undetermined.

Pathophysiology

PMR causes severe pain in the proximal muscle groups; however, no evidence of disease is present at muscle biopsy. Muscle strength and electromyographic findings are normal. Some evidence suggests the presence of cell-mediated injury to the elastic lamina in the blood vessels in the affected muscle groups.

PMR is closely linked to giant cell arteritis (temporal arteritis), but this is believed to be a separate disease process.

Frequency

United States

One study revealed a prevalence of 1 in 200 people aged 50 years or older.

Mortality/Morbidity

PMR is not a life-threatening disease, but it does require treatment for 2-4 years.

Race

Whites are affected more than other ethnic groups.

Sex

Females are affected twice as often as males.

Age

PMR usually affects people older than 50 years.



History

The patient's history may include the following features:

  • Pain and stiffness in the proximal muscle groups that usually is symmetrical and worse in the morning
  • Gel phenomenon (stiffness after prolonged inactivity)
  • Fever (low grade)
  • Weight loss
  • Fatigue
  • Depression
  • No weakness
  • Abrupt onset of symptoms

Physical

The signs and symptoms of PMR are nonspecific, and objective findings on physical examination often are lacking. If present, findings may include the following:

  • No muscle atrophy
  • Muscle tenderness
  • Decreased active range of motion of joints secondary to pain

Causes

The etiology is unknown; however, risk factors include the following:

  • Age of 50 years or older
  • Presence of giant cell arteritis



Arthritis, Rheumatoid
Depression and Suicide
Hypothyroidism and Myxedema Coma
Polymyositis
Systemic Lupus Erythematosus
Temporal Arteritis

Other Problems to be Considered

Dermatomyositis
Fibromyalgia
Osteoarthritis
Occult infection
Myopathy



Lab Studies

  • Erythrocyte sedimentation rate greater than 50 mm/h
  • Normochromic normocytic anemia in 50% of cases
  • Normal creatinine kinase level
  • Negative finding for rheumatoid factor
  • Mild elevations with liver function test results
  • Mild nonspecific synovitis
  • Negative muscle biopsy findings



Emergency Department Care

  • No emergent therapy is required.
  • Physical therapy may be administered for range of motion, if necessary.

Consultations

Consult the primary care physician and/or a rheumatologist.



The goal of therapy is to suppress autoimmune activity.

Drug Category: Corticosteroids

These agents have anti-inflammatory properties and may cause profound and varied metabolic effects. They modify the body's immune response to diverse stimuli.

Drug NamePrednisone (Deltasone, Orasone, Sterapred)
DescriptionUseful in the treatment of inflammatory and allergic reactions. By reversing increased capillary permeability and suppressing PMN activity, it may decrease inflammation. PMR is rapidly responsive to low doses of prednisone. Patients may require treatment for several months to several years.
Adult Dose5-60 mg/d PO qd or divided bid/qid; taper over 2 wk as symptoms resolve
Pediatric Dose4-5 mg/m2/d or 1-2 mg/kg PO qd; taper over 2 wk as symptoms resolve
ContraindicationsDocumented hypersensitivity; viral infection, peptic ulcer disease, hepatic dysfunction, connective tissue infections, and fungal or tubercular skin infections; GI disease
InteractionsCoadministration with estrogens may decrease prednisone clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics
PregnancyB - Usually safe but benefits must outweigh the risks.
PrecautionsAbrupt discontinuation of glucocorticoids may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur with glucocorticoid use



Further Outpatient Care

  • Perform follow-up monthly initially and during steroid tapering; then follow up quarterly.
  • Monitor erythrocyte sedimentation rate while patient is receiving steroids.

Complications

  • Complications related to steroid therapy
  • Exacerbation of disease with taper of steroids

Prognosis

  • The average length of disease is 3 years.
  • Exacerbations may occur if steroids are tapered too rapidly.
  • Relapse is common.

Patient Education



Medical/Legal Pitfalls

  • Failure to consider giant cell arteritis
  • Failure to strongly consider other diagnoses in patients younger than 50 years
  • Failure to prescribe prednisone for a patient in whom PMR is strongly suspected



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Polymyalgia Rheumatica excerpt

Article Last Updated: Jun 13, 2006