eMedicine Specialties > Rheumatology > Vasculitis

Polymyalgia Rheumatica

Author: Ehab R Saad, MD, FACP, FASN, MA, Assistant Professor, Department of Medicine, Medical College of Wisconsin
Coauthor(s): Gloria Fioravanti, DO, Program Director, Clinical Assistant Professor, Department of Internal Medicine, Saint Luke's Hospital of Bethlehem, Temple University School of Medicine; Allen Samuels, MD, Consulting Staff, Department of Internal Medicine, Division of Rheumatology, St Luke's Hospital, Lehigh Valley Hospital, Pocono Medical Center
Contributor Information and Disclosures

Updated: Aug 20, 2009

Introduction

Background

Polymyalgia rheumatica (PMR) is a relatively common clinical syndrome of unknown etiology. It is characterized by proximal myalgia of the hip and shoulder girdles with accompanying morning stiffness that lasts for more than 1 hour. Approximately 15% of patients with polymyalgia rheumatica develop giant cell arteritis (GCA), and approximately 50% of patients with giant cell arteritis have associated polymyalgia rheumatica.

Pathophysiology

The cause of polymyalgia rheumatica is unknown. HLA-DR4 is found with increased frequency in persons with polymyalgia rheumatica and in those with giant cell arteritis, and systemic monocyte activation is characteristic of both conditions. Both diseases show a sequence polymorphism encoded within the second hypervariable region of the HLA-DRB1 gene.

The pattern of T-cell–derived cytokines distinguishes the two patient populations. Patients with polymyalgia rheumatica often have elevated interleukin-2 (IL-2) and interleukin-6 (IL-6) levels. One hypothesis holds that, in a genetically predisposed patient, an environmental factor, possibly a virus, causes monocyte activation, which helps determine the production of cytokines that induce manifestations characteristic of polymyalgia rheumatica and giant cell arteritis.1 The prevalence of antibodies to adenovirus and respiratory syncytial virus was reportedly higher in patients with polymyalgia rheumatica.2 Occurrence in siblings suggests a genetic role in the pathophysiology of the disease.

Frequency

United States

The average annual incidence is 52.5 cases per 100,000 persons aged 50 years and older. The prevalence is approximately 0.5-0.7%.

International

The frequency varies by country; highest rates occur in northern Europe. For example, in Italy, the incidence is 12.7 cases per 100,000 persons.

Mortality/Morbidity

  • With appropriate treatment, the survival rate is similar to that of unaffected persons of the same age; however, some reports document increased mortality from vascular disease among men with polymyalgia rheumatica after the initial 2 years following diagnosis.
  • Polymyalgia rheumatica is self-limited and often remits in 1-3 years. Untreated patients, however, often feel unwell and have an impaired quality of life.

Race

Polymyalgia rheumatica almost always affects whites but is also occasionally reported in African American persons.

Sex

Polymyalgia rheumatica is twice as common in females.

Age

The incidence increases with advancing age. Polymyalgia rheumatica rarely affects persons younger than 50 years. The median age at diagnosis is 72 years.

Clinical

History

Patients are often in good health prior to disease onset, which is abrupt in about 50% of patients. In most patients, the shoulder girdle is first to become symptomatic. In the remainder, the hip or neck is involved at onset. At presentation, symptoms may be unilateral but usually become bilateral within a few weeks.

  • Criteria for diagnosis
    • Age 50 years or older at onset
    • Bilateral aching and morning stiffness for at least 1 month and involving at least 2 of 3 areas: neck or torso, shoulders or arms, hips or thighs
    • Westergren erythrocyte sedimentation rate (ESR) 40 mm/h or greater
    • Prompt response of symptoms to corticosteroids (15 mg/d)
  • Systemic
    • Low-grade fever and weight loss
    • Malaise, fatigue, and depression
    • Difficulty rising from bed in the morning
    • Difficulty getting up from the toilet
    • Difficulty completing daily life activities
    • High, spiking fevers (rare)
  • Musculoskeletal
    • Morning stiffness for more than 1 hour, often more prolonged
    • Muscle stiffness after prolonged inactivity
    • Carpal tunnel syndrome (in about 15% of patients)
    • Distal extremity swelling (uncommon)
    • Possible development of arthralgia and myalgia up to 6 months after onset of systemic symptoms

Physical

Polymyalgia rheumatica is a clinical diagnosis based on the complex of presenting symptoms and exclusion of the other potential diseases.

  • General
    • Fatigued appearance
    • Low-grade temperature
    • Distal extremity swelling with pitting edema, which should be distinguished from remitting seronegative symmetrical synovitis with pitting edema (RS3PE)3
  • Musculoskeletal
    • Normal muscle strength
    • Pain in the shoulder and hip with movement without significant clinical swelling
    • Transient synovitis of the knee, wrist, and sternoclavicular joints
    • Tenderness to palpation with decreased active range of motion in the proximal hip/leg and/or shoulder/arm girdle musculature
    • In later stages: Disuse muscle atrophy with proximal muscle weakness and even contractures of the shoulder capsule may lead to limitation of passive and active movement.

Causes

  • Exact causes are unknown. The disease is more common among northern Europeans, which may indicate a genetic predisposition.
  • An autoimmune process may play a role in polymyalgia rheumatica development. Polymyalgia rheumatica is associated with the HLA-DR4 haplotype. High levels of IL-2 are associated with polymyalgia rheumatica, and high serum levels of IL-6 correlate with increased disease activity.
  • T2-weighted MRI of the shoulders may reveal subdeltoid, subacromial, and bicipital tenosynovitis. These findings have led many investigators to believe that nonerosive synovitis and tenosynovitis are responsible for many symptoms of polymyalgia rheumatica.

More on Polymyalgia Rheumatica

Overview: Polymyalgia Rheumatica
Differential Diagnoses & Workup: Polymyalgia Rheumatica
Treatment & Medication: Polymyalgia Rheumatica
Follow-up: Polymyalgia Rheumatica
References
Further Reading
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References

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Keywords

polymyalgia rheumatica, PMR, giant cell arteritis, GCA, collagen diseases, muscle pain, morning stiffness, HLA-DR4, interleukin-2, IL-2, interleukin-6, IL-6, remitting seronegative symmetrical synovitis with pitting edema, RS3PE, carpal tunnel syndrome, malaise, fatigue, depression, nonerosive synovitis, tenosynovitis, bursitis, proximal myalgia of the hip, shoulder girdles, HLA-DR4 haplotype

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Contributor Information and Disclosures

Author

Ehab R Saad, MD, FACP, FASN, MA, Assistant Professor, Department of Medicine, Medical College of Wisconsin
Ehab R Saad, MD, FACP, FASN, MA is a member of the following medical societies: American College of Physicians-American Society of Internal Medicine, American Geriatrics Society, American Society of Nephrology, and National Kidney Foundation
Disclosure: Nothing to disclose.

Coauthor(s)

Gloria Fioravanti, DO, Program Director, Clinical Assistant Professor, Department of Internal Medicine, Saint Luke's Hospital of Bethlehem, Temple University School of Medicine
Gloria Fioravanti, DO is a member of the following medical societies: American College of Physicians-American Society of Internal Medicine and American Osteopathic Association
Disclosure: Nothing to disclose.

Allen Samuels, MD, Consulting Staff, Department of Internal Medicine, Division of Rheumatology, St Luke's Hospital, Lehigh Valley Hospital, Pocono Medical Center
Allen Samuels, MD is a member of the following medical societies: American College of Rheumatology
Disclosure: Nothing to disclose.

Medical Editor

Kristine M Lohr, MD, MS, Program Director, Professor, Department of Internal Medicine, Division of Rheumatology and Women's Health, University of Kentucky School of Medicine
Kristine M Lohr, MD, MS is a member of the following medical societies: American College of Physicians, American College of Rheumatology, and American Medical Women's Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Elliot Goldberg, MD, Dean of the Western Pennsylvania Clinical Campus, Professor, Department of Medicine, Temple University School of Medicine
Elliot Goldberg, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, and American College of Rheumatology
Disclosure: Nothing to disclose.

CME Editor

Alex J Mechaber, MD, FACP, Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine
Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine
Disclosure: Nothing to disclose.

Chief Editor

Herbert S Diamond, MD, Professor of Medicine, Temple University School of Medicine; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital
Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, and Phi Beta Kappa
Disclosure: medifocus Honoraria Review panel membership; health dialogs Honoraria Consulting; West Penn Allegheny Health System None Board membership

 
 
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