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Polymyalgia rheumatica - Management
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How do I know my patient has it?
- A diagnosis of polymyalgia rheumatica is mainly based on symptoms and signs, although there are some investigations that may help support the diagnosis.
- A common presentation is a person older than 50 years of age with [Dasgupta et al, 2007]:
- Bilateral shoulder and/or pelvic girdle aching lasting more than 2 weeks.
- Morning stiffness (for more than 45 minutes).
- Raised erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP).
- If symptoms or signs of giant cell arteritis are present (such as headache, scalp tenderness, visual symptoms), urgent management is needed (see the CKS topic on Giant cell arteritis).
- People with polymyalgia rheumatica usually respond rapidly to systemic corticosteroids, but this is not diagnostic as other conditions with similar presentation can also respond rapidly to steroids.
- Many features of polymyalgia rheumatica can lead to diagnostic error because the main symptoms occur in many other illnesses [Dasgupta et al, 2007]. For more information, see differential diagnosis.
- Several sets of criteria have been proposed to increase the uniformity and accuracy of diagnosis and are discussed in Diagnostic criteria.
- Consider referral to a rheumatologist to confirm the diagnosis.
What are the symptoms of polymyalgia rheumatica?
- Onset — the onset of polymyalgia rheumatica is usually rapid, but may be insidious. However, symptoms may have been present for weeks or months before the diagnosis is made .
- Muscle pain — shoulder pain is the presenting feature in 70–95% of people, with the hips and neck being less frequently involved (50–70%). The pain usually radiates distally towards the elbows and knees. It can begin in one shoulder or hip but soon becomes bilateral. Pain is more severe with movement and interferes with sleep at night.
- Stiffness — stiffness after periods of rest, and morning stiffness of more than 45 minutes are typical. The stiffness may be so profound that the person may have great difficulty turning over in bed, rising from a bed or a chair, or raising their arms above shoulder height (for example to comb their hair).
- Low-grade fever, fatigue, anorexia, weight loss, and depression (systemic symptoms) occur in up to 40% of people.
- Headache, scalp tenderness, and visual disturbances, (symptoms indicative of giant cell arteritis) may be present in 10–20% of people with polymyalgia rheumatica.
Basis for recommendation
These recommendations are based on published expert opinion [Salvarani et al, 2002; Michet and Matteson, 2008; Salvarani et al, 2008], and published guidelines that are based on draft guidance from the British Rheumatology [Dasgupta et al, 2007].
What are the signs of polymyalgia rheumatica?
- Bilateral upper arm tenderness is sometimes present. Shoulder abduction is often uncomfortable and may be limited by pain.
- Muscle strength is not usually impaired, but muscle pain may make testing difficult. If symptoms are protracted, disuse atrophy of muscle can occur, leading to muscle weakness.
- Peripheral musculoskeletal signs are seen in approximately 50% of people. They include:
- Carpal tunnel syndrome.
- Peripheral arthritis (predominantly affecting the knees and wrists), which is asymmetric, non-erosive, and self-limiting.
- Swelling with pitting oedema of hands, wrists, feet, and ankles.
- Scalp tenderness and visibly thickened and tender temporal arteries are signs indicative of giant cell arteritis, and may be present in 10–20% of people with polymyalgia rheumatica.
Basis for recommendation
These recommendations are based on published expert opinion [Michet and Matteson, 2008; Salvarani et al, 2008].
What investigations should I do?
- There are no diagnostic tests that can be routinely carried out in primary care to diagnose polymyalgia rheumatica. However, the following may help to support a diagnosis:
- Erythrocyte sedimentation rate (ESR).
- The ESR is typically higher than 40 mm per hour.
- However, the ESR may be normal at presentation and even during a flare of disease activity (relapse). At presentation, 7–20% of people with polymyalgia rheumatica have an ESR less than 40 mm per hour.
- C-reactive protein (CRP) is typically elevated.
- Full blood count.
- Normochromic normocytic anaemia is common.
- Consider investigations that help rule out other conditions with similar presentations — for more information, see differential diagnosis.
- With respect to other investigations that may be carried out, note that:
- Mild abnormalities in liver function tests are common (for example raised alkaline phosphatase is present in about 30% of people).
- Serum protein electrophoresis may show increased alpha1 and alpha2 globulins.
Basis for recommendation
These recommendations are based on published expert opinion [Dasgupta and Hassan, 2007; Salvarani et al, 2008].
How should I interpret the response to systemic corticosteroids?
- Response to systemic corticosteroids is usually rapid and dramatic; symptoms largely resolve in most people within a week, with resolution of laboratory abnormalities in 3–4 weeks.
- Response to treatment with corticosteroids cannot be used as a diagnostic test, as other conditions that can present with features of polymyalgia rheumatica also respond to treatment with corticosteroids (for example rheumatoid arthritis).
- Lack of response suggests that an alternative diagnosis should be considered.
Basis for recommendation
These recommendations are based on published guidelines that are based on draft guidance from the British Rheumatology [Dasgupta et al, 2007], and published expert opinion [Michet and Matteson, 2008].
What diagnostic criteria are used for diagnosing polymyalgia rheumatica?
- Several sets of criteria for diagnosing polymyalgia rheumatica have been proposed, principally to aid research by ensuring uniformity within trials and to allow comparison of results from different studies. However, the criteria have also been used for clinical purposes.
- One report examined the performance of individual criteria and sets of criteria for making a diagnosis of polymyalgia rheumatica . The set of criteria proposed was found to be most useful (sensitivity 92%, specificity 80%, likelihood ratio 5).
- Polymyalgia rheumatica is diagnosed by the Bird criteria if three or more of the following are present:
- Bilateral shoulder pain or stiffness.
- Onset of illness less than 2 weeks previously.
- Initial erythrocyte sedimentation rate (ESR) greater than 40 mm per hour.
- Morning stiffness lasting longer than an hour.
- 65 years of age or more.
- Depression and/or weight loss.
- Bilateral tenderness in the upper arms.
- Diagnostic criteria drafted at the Third International Conference on Polymyalgia Rheumatica and Giant Cell Arteritis (July 2005) have also been published, and are currently being tested in a clinical trial.
Basis for recommendation
This information is taken from a narrative review [Bird et al, 2005] of the literature, a diagnostic study[Bird et al, 1979] and published expert opinion [Dasgupta et al, 2008].
What else might it be?
- It is important to rule out conditions that can present with features similar to those of polymyalgia rheumatica, particularly conditions where use of systemic corticosteroids could have harmful consequences.
- The process of ruling out other possible conditions is lengthy, because diseases such as rheumatoid arthritis can take many months to become apparent, especially if treatment with corticosteroids is started. The initial diagnosis of polymyalgia rheumatica is thus, in effect, a 'working diagnosis'.
- Features that indicate increased likelihood of the diagnosis being a condition other than polymyalgia rheumatica include:
- Onset at less than 65 years of age.
- Incomplete response to treatment with corticosteroids.
- Weakness — suggests polymyositis.
- Prominent peripheral joint symptoms — suggest rheumatoid arthritis or remitting seronegative symmetrical synovitis with pitting oedema syndrome.
- Presence of other atypical clinical features (for example lack of shoulder pain, no muscle stiffness, and unilateral rather than bilateral girdle pain).
- See Table 1 for examples of disorders that can cause similar symptoms.
Table 1. Examples of disorders that can cause similar symptoms to polymyalgia rheumatica.
Differential diagnosis | Examples |
|---|
Degenerative disorders (may coexist with polymyalgia rheumatica and increase the need for steroid therapy) | Cervical and lumbar spondylosis Osteoarthritis Bilateral adhesive capsulitis (frozen shoulder), rotator cuff disorders Osteoporosis |
Endocrine disorders | Thyroid disease Parathyroid disease |
Infection | Chronic osteomyelitis Viral illness Tuberculosis Infective endocarditis |
Inflammatory disorders | Rheumatoid arthritis, often seronegative for rheumatoid factor (common) Spondyloarthropathy (rare) Remitting seronegative symmetric synovitis with pitting oedema (rare) Polymyositis/dermatomyositis Systemic lupus erythematosus Other connective tissue disorders |
Muscle disorders | Proximal myopathy (for example, corticosteroid-induced) Muscular dystrophy |
Malignancy | Multiple myeloma Leukaemia Lymphoma Lung carcinoma Other occult carcinomas |
Neurological disorders | Parkinson's disease Myasthenic syndromes |
Psychiatric disorders | Depression |
Drug adverse effects | Myositis or myalgia due to statins Polymylagia rheumatica-like syndrome due to quinidine |
Miscellaneous | Osteomalacia Fibromyalgia Chronic fatigue syndrome Chronic pain syndromes |
|
Basis for recommendation
These recommendations are based on published expert opinion [Hazleman, 1998; Salvarani et al, 2008], and published guidelines that are based on draft guidance from the British Rheumatology [Dasgupta et al, 2007].
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