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Polymyalgia rheumatica - Management
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What initial management is required for someone with polymyalgia rheumatica?
If the person has symptoms or signs suggestive of giant cell arteritis, see the CKS topic on Giant cell arteritis. Visual symptoms need urgent (same day) referral.
- Establish a baseline against which to assess response to treatment by documenting:
- Current symptoms and signs of polymyalgia rheumatica.
- Symptoms or disability present before the onset of the current problem.
- Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP).
- Prescribe prednisolone 15 mg each day. Seek specialist advice if the person is unable to take oral prednisolone.
- Within 1 week, assess the person's response to prednisolone (symptoms should dramatically resolve within a few days).
- Consider early referral for people with atypical features and treatment dilemmas.
- Consider referring all people with suspected polymyalgia rheumatica to rheumatology for confirmation of the diagnosis (corticosteroids can be started while the person is awaiting specialist assessment).
- Ensure that the person understands the importance of new headaches, visual disturbances, and other symptoms and signs of giant cell arteritis and the need for urgent medical review if these develop.
- Do not prescribe nonsteroidal anti-inflammatory drugs (NSAIDs) as these are not thought to be effective for polymyalgia rheumatica.
Basis for recommendation
- Unblinded, uncontrolled, prospective, and retrospective cohort studies suggest that corticosteroids are effective treatment for polymyalgia rheumatica. Corticosteroids have not been evaluated in placebo-controlled clinical trials, and it is unlikely these would ever take place because the effect of corticosteroids is so dramatic.
- The initial dose of prednisolone of 15 mg each day is recommended by published guidelines that are based on draft guidance from the British Society for Rheumatology [Dasgupta et al, 2007].
- Some experts recommend the use of intramuscular methylprednisolone in milder cases of polymyalgia rheumatica [Dasgupta et al, 2007]. However intramuscular methylprednisolone is not routinely used in primary care.
- Rheumatology assessment is recommended in people with suspected polymyalgia rheumatica, because many features of the condition can lead to diagnostic error.
- Among other assessments, a specialist may use a standardized scoring system to assess the response to corticosteroid treatment [Dasgupta et al, 2007; Michet and Matteson, 2008].
- A diagnosis of polymyalgia rheumatica is likely if the person has a global response of greater than 70% improvement within 1 week and if inflammatory markers (ESR and CRP) return to normal in 3–4 weeks.
- A lesser response may suggest that the cause of the symptoms is another condition, such as osteoarthritis, rheumatoid arthritis, cancer, or infection. Symptoms due to these conditions respond to corticosteroids, but the response is not as dramatic as that of polymyalgia rheumatica.
- The diagnosis of polymyalgia rheumatica is not confirmed or sustained in up to 23% of people with suspected polymyalgia rheumatica when they are followed up [Dasgupta et al, 2007].
- The recommendation that NSAIDs are not effective for treating polymyalgia rheumatica is based on published guidelines that are based on draft guidance from the British Society for Rheumatology [Dasgupta et al, 2007]. CKS found no trial evidence that NSAIDS were effective or not effective.
What ongoing management is required for someone with polymyalgia rheumatica?
- If the symptoms of polymyalgia rheumatica are controlled, reduce the dose of prednisolone slowly.
- The dose may have to be titrated individually in each person with either smaller reductions or longer periods at each level (treatment duration may range from 1 year to 3 years).
- A suggested schedule is to:
- Continue prednisolone 15 mg each day until symptoms have returned to normal (usually 3 weeks), then
- Reduce the dose to 12.5 mg each day for 3 weeks, then
- Reduce the dose to 10 mg each day for 4–6 weeks, then
- Reduce the dose by 1 mg every 4–8 weeks until treatment is stopped.
- A week after any change in dose, review the person to exclude any relapse of symptoms. It may be possible to do this by telephone.
- If symptoms worsen following a dose reduction, treat with the previous higher dose. For more information on how to manage relapses, see Managing relapses.
- Start osteoporosis prophylaxis if the person is 65 years of age or older, or has a history of fragility fracture.
- In other individuals, measure bone mineral density using dual energy X-ray absorptiometry (DXA) to assess fracture risk. If the T score is –1.5 or lower, consider prophylaxis.
- For more information, see the CKS topic on Osteoporosis - preventing steroid-induced.
- Monitor the person regularly to assess for disease relapse (including symptoms of giant cell arteritis, see the CKS topic on Giant cell arteritis) and steroid-related adverse effects.
- Do not prescribe nonsteroidal anti-inflammatory drugs (NSAIDs) as these are not effective.
Additional information
- A suggested monitoring schedule is to see the person at 3 and 6 weeks, and then at 3, 6, 9, and 12 months. After the first year, see the person every 3–6 months (with extra visits for relapses or adverse events).
- At each visit:
- Assess symptoms and review results of inflammatory markers (erythrocyte sedimentation rate [ESR] and/or C-reactive protein [CRP]).
- In the absence of symptoms, raised inflammatory markers alone are not a valid reason to increase the dose of corticosteroid.
- If inflammatory markers are raised and the person is scheduled to have their dose reduced, consider rechecking the inflammatory markers before deciding to reduce the dose.
- If inflammatory markers are persistently elevated, consider other possible diagnoses (such as malignancy, multiple myeloma, infection, or connective tissue disease).
- If inflammatory markers decrease but the person continues to complain of symptoms, consider other possible causes of the symptoms.
- Assess for adverse effects related to steroids (such as weight gain, raised blood pressure, diabetes, osteoporosis, and abnormal lipid profile).
- For more information on how to monitor the adverse effects of long-term corticosteroids, see the CKS topic Corticosteroids - oral.
Basis for recommendation
- The recommendation regarding osteoporosis prophylaxis is based on published guidance from the Royal College of Physicians [RCP, 2002]. Prophylaxis may be required because corticosteroids can cause osteoporosis, especially if taken over long periods. Most people with polymyalgia rheumatica need to take corticosteroids for 1–3 years. Some people have a chronic-relapsing course and may need low doses of prednisolone for several years [Salvarani et al, 2008].
- These recommendations are based on expert opinion in published guidelines [Michet and Matteson, 2008; Salvarani et al, 2008], and by published guidelines that are based on draft guidance from the British Society for Rheumatology [Dasgupta et al, 2007].
- The dose of prednisolone should be reduced slowly because relapses are common and are more likely to occur if corticosteroids are reduced or withdrawn too quickly [Salvarani et al, 2008]. Rapid tapering of corticosteroids has been associated with longer duration of therapy [Dasgupta et al, 2007].
- The suggested tapering schedule is that proposed by the British Society for Rheumatology Guidelines Group [Dasgupta et al, 2007]. The schedules suggested by CKS expert reviewers varied considerably and locally recommended schedules may vary from the one proposed.
- Some experts recommend the use of intramuscular methylprednisolone in milder cases of polymyalgia rheumatica [Dasgupta et al, 2007]. However, intramuscular methylprednisolone is not routinely used in primary care.
- Complications of long-term corticosteroid treatment are common and may occur in up to 60% of people [Kremers et al, 2005; Michet and Matteson, 2008]. Adverse effects include osteoporosis, avascular necrosis, infections, diabetes, hypertension, and cataracts [Michet and Matteson, 2008].
- In 56% of people, the ESR returns to normal within 2 weeks, and in 76% of people within 5 weeks. CRP decreases more rapidly, with 67% normalizing in 2 weeks and 75% in 3 weeks [Andersson et al, 1986a; Leeb et al, 2003].
How should I manage relapses in someone with polymyalgia rheumatica?
- If the person develops symptoms or signs suggestive of giant cell arteritis, see the CKS topic on Giant cell arteritis.
- Features of giant cell arteritis include headache; scalp tenderness; jaw claudication; tender, thickened temporal arteries; and visual symptoms such as diplopia and visual loss.
- Visual symptoms need urgent (same day) referral to an ophthalmologist.
- If the person has features that suggest a relapse of polymyalgia rheumatica alone (such as shoulder or pelvic girdle pain and stiffness):
- Increase prednisolone to the previous higher dose and monitor for response.
- ▪If symptoms settle, it may be wise to continue on the higher dose for longer than usual before following the suggested tapering schedule — see Ongoing management.
- ▪If symptoms do not improve, increase the dose of prednisolone further and seek specialist advice.
- If the person has frequent relapses, or it is not possible to taper steroids, specialist input is strongly recommended so that the diagnosis can be reviewed and adjuvant therapy considered.
- A relapse should be evaluated with regard to symptoms and signs, as well as assessment for related conditions (especially giant cell arteritis), and not solely on the basis of raised inflammatory markers (erythrocyte sedimentation rate [ESR] and C-reactive protein [CRP]) [Dasgupta et al, 2007].
- If ESR or CRP are persistently elevated, consider other causes for the symptoms (for example malignancy, multiple myeloma, infection, or connective tissue disease).
- The dose of prednisolone should not be increased to try to reduce a persistently high ESR or CRP [Michet and Matteson, 2008].
Basis for recommendation
- These recommendations are based on expert opinion in published guidelines [Michet and Matteson, 2008; Salvarani et al, 2008], and published guidelines that are based on draft guidance from the British Society for Rheumatology [Dasgupta et al, 2007].
- Specialists may consider the use of steroid-sparing treatments such as disease-modifying antirheumatic drugs for people who experience frequent relapses.
What advice should I give someone with polymyalgia rheumatica?
- Advise the person:
- To seek urgent medical attention if they develop any visual disturbances.
- That the dose of prednisolone is normally reduced very slowly over several months. Treatment is often required for 1–2 years but some people may require low doses of corticosteroids for several years.
- That relapses may occur while taking prednisolone; they are more common when the dose is being reduced.
- That follow-up visits are required frequently in the initial phase of the condition to monitor for relapses and adverse effects of corticosteroids, then every few months as the condition stabilizes.
- Check the person has:
- A booklet on polymyalgia rheumatica and a contact for a regional patient support group if available (for example, see www.pmr-gca.org.uk).
- A blue steroid card, and discuss any potential adverse effects with them. For more information, see the CKS topic on Corticosteroids - oral.
- Give general and lifestyle recommendations to minimize adverse effects of corticosteroid treatment.
- Encourage adequate dietary calcium intake and good nutrition.
- Advise on maintaining a healthy body weight where possible.
- Advise on smoking cessation. See the CKS topic on Smoking cessation for more information.
- Advise on moderate alcohol consumption. See the CKS topic on Alcohol - problem drinking for more information.
- Encourage physical exercise.
- Perform a falls risk assessment, where appropriate, and advise those at increased risk of fractures from falling. See the CKS topic on Falls - risk assessment for more information.
- Advise the person that if they experience any adverse effects, they should not stop taking their prednisolone abruptly and that they should seek medical advice.
- Advise all people without a history of chickenpox or measles who are taking systemic prednisolone to avoid close contact with people who have chickenpox, shingles, or measles, and to seek urgent medical advice if they are exposed.
Basis for recommendation
- CKS recommends this advice which reflects good medical practice.
- The recommendation regarding exposure to chickenpox while taking corticosteroids is based on advice from the Medicines and Healthcare products Regulatory Agency [MHRA, 2007].
- Prolonged courses of corticosteroids increase susceptibility to, and severity of, infection. Unless people taking long-term corticosteroids have had chickenpox, they should be regarded as being at risk of severe chickenpox. Manifestations of fulminant illness include pneumonia, hepatitis, and disseminated intravascular coagulation [BNF 56, 2008].
When should I refer?
- Consider referring all people with suspected polymyalgia rheumatica to rheumatology for confirmation of the diagnosis.
- Refer early to a rheumatologist if there are:
- Atypical features or features that increase likelihood of an alternative diagnosis, such as:
- The person is younger than 60 years of age.
- Insidious onset.
- Lack of shoulder involvement.
- Lack of morning stiffness.
- Red flag features, such as prominent systemic features, weight loss, night pain, neurological signs.
- Peripheral arthritis or other features of rheumatic disease.
- Normal erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP), or very high ESR and CRP.
- Treatment dilemmas, such as:
- Incomplete or non-response to corticosteroids.
- Poorly-sustained response to corticosteroids.
- Being unable to reduce corticosteroids.
- Frequent relapses.
- Contraindications to corticosteroids. For more information, see the CKS topic Corticosteroids - oral.
Basis for recommendation
- These referral recommendations are based on published guidelines [Dasgupta et al, 2007], which are based on draft guidelines from the British Society for Rheumatology.
- Many conditions can mimic polymyalgia rheumatica, and it is generally recommended that people with suspected polymyalgia rheumatica should obtain at least one rheumatology opinion.
- Treatment with corticosteroids can be started while the person is waiting to be seen by a specialist.
- People with typical features of polymyalgia rheumatica who show a complete sustained response to corticosteroids may then be managed in primary care.
Prescriptions
Start corticosteroid
Age from 40 years onwards
Prednisolone tablets: 15mg each morning
Prednisolone 5mg tablets
Take three tablets each morning (as a single dose).
Supply 84 tablets.
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