Polymyalgia Rheumatica Clinical Presentation

Updated: Nov 16, 2017
  • Author: Ehab R Saad, MD, MA, FACP, FASN; Chief Editor: Herbert S Diamond, MD  more...
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Presentation

History

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

The symptoms include pain and stiffness of the shoulder and hip girdle. The stiffness may be so severe that the patient may have a great difficulty rising from a chair, turning over in bed, or raising the arms above shoulder height. Stiffness after periods of rest (gel phenomenon) as well as morning stiffness of more than 1 hour typically occurs.

Muscle weakness is not a feature of PMR. Howeve, this can be difficult to assess in the setting of pain, especially if symptoms are protracted and untreated, resulting in disuse atrophy.

Patients may also describe distal peripheral joint swelling or, more rarely, limb edema. Carpal tunnel syndrome can occur in some patients. Most patients report systemic features as listed below.

Several diagnostic criteria for PMR exist. One set of diagnostic criteria is as follows [18] :

  • Age of onset 50 years or older
  • Erythrocyte sedimentation rate ≥40 mm/h
  • Pain persisting for ≥1 month and involving 2 of the following areas: neck, shoulders, and pelvic girdle
  • Absence of other diseases capable of causing the same musculoskeletal symptoms
  • Morning stiffness lasting ≥1 hour
  • Rapid response to prednisone (≤20 mg)

In 2012, the European League Against Rheumatism and the American College of Rheumatology published new provisional classification criteria for PMR in patients aged 50 or older with bilateral shoulder aching and elevated inflammatory markers. These classification criteria are not diagnostic criteria, but rather designed for enrolling patients into clinical trials of new treatments for PMR. [19] This collaborative initiative resulted in a scoring algorithm based on the following criteria:

  • Morning stiffness >45 minutes (2 points)
  • Hip pain/limited range of motion (1 point)
  • Absence of rheumatoid factor and/or anti-citrullinated protein antibody (anti-CCP) (2 points)
  • Absence of peripheral joint pain (1 point)

A score of ≥4 points has a 68% sensitivity and 78% specificity for discriminating PMR from other comparison patients. There is also an additional ultrasound criteria (1 point if positive findings), which can add up to a score of ≥5 points that is associated with a 66% sensitivity and 81% specificity for PMR. [20]

Systemic findings in more than 50% of patients are as follows:

  • Low-grade fever and weight loss
  • Malaise, fatigue, and depression
  • Difficulty rising from bed in the morning
  • Difficulty getting up from the toilet or out of a chair
  • Difficulty completing daily life activities
  • High, spiking fevers (rare, should prompt evaluation for underlying infection, malignancy, or vasculitis)

Musculoskeletal findings are as follows [21] :

  • Morning stiffness for ≥1 hour, often more prolonged
  • Muscle stiffness after prolonged inactivity
  • Synovitis of proximal joints and periarticular structures
  • Peripheral arthritis (in 25% of patients)
  • Carpal tunnel syndrome (in about 15% of patients)
  • Distal extremity swelling (in approximately 12%)
  • Possible development of arthralgia and myalgia up to 6 months after onset of systemic symptoms
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Causes

The exact causes of PMR are unknown. The disease is more common among northern Europeans, which may indicate a genetic predisposition.

An autoimmune process may play a role in PMR development. PMR is associated with the HLA-DR4 haplotype. High level of IL-6 is associated with increased disease activity.

Many investigators believe that nonerosive synovitis and tenosynovitis are responsible for many symptoms of PMR

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Physical Examination

PMR is a clinical diagnosis based on the complex of the presenting symptoms and exclusion of the other potential diseases. The symptoms and signs of PMR are nonspecific, and objective findings on physical examination are often lacking.

General symptoms are as follows:

  • Fatigued appearance
  • Low-grade temperature
  • Distal extremity swelling with pitting edema

Musculoskeletal findings are as follows:

  • Normal muscle strength; no muscle atrophy typically present at initial presentation
  • Pain in the shoulder and hip with movement; active range of motion may be decreased because of pain
  • Transient synovitis of the knee, wrist, and sternoclavicular joints; a more peripheral nonerosive arthritis may be seen in some cases
  • Tenderness to palpation with decreased active range of motion in the musculature of the proximal hip/leg and/or shoulder/arm girdle

In later stages, disuse muscle atrophy with proximal muscle weakness may occur. Contractures of the shoulder capsule may lead to limitation of the passive and active movements.

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