Clinical Topic A-Z Clinical Speciality

Polymyalgia rheumatica

Polymyalgia rheumatica
D011111Polymyalgia Rheumatica
CardiovascularEyesMusculoskeletalNeurological
2013-08-01Last revised in August 2013

Polymyalgia rheumatica - Summary

Polymyalgia rheumatica (PMR) is an inflammatory condition of unknown cause, characterized by aching and morning stiffness in the neck, shoulder, and pelvic girdle.

The average age of onset is just over 70 years of age.

It occurs about two to three times as frequently in women as it does in men.

Complications of PMR include:

Giant cell arteritis, which may occur abruptly and without warning and occurs in approximately 10–20% of people taking corticosteroids for PMR.

Complications of long-term corticosteroid treatment.

The prognosis of PMR is variable:

The response to systemic corticosteroids is usually rapid and dramatic, with many symptoms resolving after a few days of treatment.

Treatment for 1–3 years is often required, and some people may need low-dose corticosteroids for several years.

Relapse is common, but responds to restarting or increasing the dose of systemic corticosteroids.

PMR should be suspected in a person older than 50 years of age presenting with:

Bilateral shoulder and/or pelvic girdle aching lasting more than 2 weeks.

Morning stiffness (for more than 45 minutes).

Evidence of an acute phase response.

Other more general symptoms such as low-grade fever, fatigue, anorexia, weight loss, or depression.

A working diagnosis of PMR is made from the combination of:

Presence of core features of the condition.

Exclusion of conditions that mimic PMR such as rheumatoid arthritis, polymyositis, and fibromyalgia.

A positive response to oral corticosteroids within a week.

Normalization of inflammatory markers within 4 weeks.

Referral to a rheumatologist should be made if there is doubt about the diagnosis, for example because:

There are atypical features of PMR, such as no evidence of an acute phase response, and no clear alternative diagnosis.

There is a poor response to corticosteroids.

Management of a person with a working diagnosis of PMR involves:

Gradually reducing the dose of corticosteroids when symptoms are fully controlled, adjusting the size of each dose reduction and the duration at each dose to avoid relapses.

Assessing and managing the risk of osteoporosis.

Assessing for, and managing symptoms of relapse, giant cell arteritis, and steroid-related adverse effects.

Referral to a rheumatologist is recommended for people diagnosed with PMR if:

It is not possible to reduce corticosteroids at reasonable intervals without causing relapse.

Corticosteroids are required for more than 2 years.

Have I got the right topic?

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This CKS topic is based on published guidelines from the British Society for Rheumatology and British Health Professionals in Rheumatology for the management of polymyalgia rheumatica [Dasgupta et al, 2009; Dasgupta et al, 2010].

This CKS topic covers the management of polymyalgia rheumatica.

This CKS topic does not cover the management of giant cell arteritis.

There are separate CKS topics on Ankylosing spondylitis, Back pain - low (without radiculopathy), Giant cell arteritis, Neck pain - acute torticollis, Neck pain - cervical radiculopathy, Neck pain - non-specific, Neck pain - whiplash injury, Osteoarthritis, Rheumatoid arthritis, Shoulder pain, and Sciatica (lumbar radiculopathy).

The target audience for this CKS topic is healthcare professionals working within the NHS in the UK, and providing first contact or primary health care.

How up-to-date is this topic?

How up-to-date is this topic?

Changes

Last revised in August 2013

August 2013 — reviewed. A literature search was conducted in July 2013 to identify evidence-based guidelines, UK policy, systematic reviews, and key RCTs published since the last revision of this topic. Changes have been made to the diagnosis and management sections to provide a more stepped approach to the assessment and management of a person with suspected polymyalgia rheumatica. These changes reflect recommendations made in the British Society for Rheumatology (BSR) and British Health Professionals in Rheumatology (BHPR) guidelines for the management of polymyalgia rheumatica.

Previous changes

August 2012 — minor update. Minor typographical errors corrected.

February 2010 — topic structure revised to ensure consistency across CKS topics — no changes to clinical recommendations have been made.

December 2008 to May 2009 — converted from CKS guidance to CKS topic structure. The evidence-base has been reviewed in detail, and are more clearly justified and transparently linked to the supporting evidence.

Together with the CKS topic on Giant cell arteritis, this CKS topic replaces the former topic on PMR and GCA.

There have been no major changes to the recommendations.

September 2008 — minor correction to the Changes section. Issued September 2008.

July 2006 — minor update to include the Commission on Human Medicine's warning for bisphosphonates and associated osteonecrosis of the jaw. Issued in July 2006.

June 2005 — reviewed. Validated in September 2005 and issued in November 2005.

January 2004 — updated to incorporate the safety update from the Committee on Safety of Medicines (CSM) advising that hormone replacement therapy (HRT) should no longer be used first-line for the prevention of osteoporosis. Issued in February 2004.

June 2003 — updated to incorporate new guidance from the Royal College of Physicians: Glucocorticoid-induced osteoporosis. Validated in September 2003 and issued in October 2003.

January 2002 — reviewed. Validated in March 2002 and issued in April 2002.

June 1999 — written. Validated in October 1999 and issued in January 2000.

Update

New evidence

Evidence-based guidelines

New new evidence-based guidelines since 1 July 2013.

HTAs (Health Technology Assessments)

No new HTAs since 1 July 2013.

Economic appraisals

No new economic appraisals relevant to England since 1 July 2013.

Systematic reviews and meta-analyses

No new systematic reviews since 1 July 2013.

Primary evidence

No new randomized controlled trials published in the major journals since 1 July 2013.

New policies

No new national policies or guidelines since 1 July 2013.

New safety alerts

No new safety alerts since 1 July 2013.

Changes in product availability

No changes in product availability since 1 July 2013.

Goals and outcome measures

Goals

To control symptoms

To preserve mobility, independence, and quality of life

To minimize complications, e.g. blindness

To minimize the risks of corticosteroid treatment

Background information

Definition

What is it?

Polymyalgia rheumatica is an inflammatory condition of unknown cause, characterized by aching and morning stiffness in the neck, shoulder, and pelvic girdle [Salvarani et al, 2002].

Prevalence

How common is it?

Polymyalgia rheumatica is the most common cause of new inflammatory rheumatic disease in elderly people, and represents one of the most common indications for long-term corticosteroid therapy in the community [Dasgupta et al, 2007].

Epidemiological studies show marked variations in the incidence and prevalence of polymyalgia rheumatica. The variability is partly due to differences in case definition and study methodology, but there are also differences due to age, sex, and genetic factors.

Age — the average age of onset is just over 70 years of age. It is seldom diagnosed in people younger than 50 years of age [Michet and Matteson, 2008].

Sex — it occurs about two to three times as frequently in women as it does in men [Salvarani et al, 2002].

Genetic variation — prevalence varies between different populations, with Scandinavian people having a higher rate than other white populations, black people and Japanese people [Gran, 2010].

In the UK, a study of primary care computer records found the annual incidence of polymyalgia rheumatica in the general population to be 84 per 100,000 [Smeeth et al, 2006].

Complications

What are the complications?

Giant cell arteritis and its complications can occur, abruptly and without warning, early in the course of polymyalgia rheumatica [Hunder, 1997].

A cohort study in the US enrolled 364 people with polymyalgia rheumatica over a period of 30 years. Of the 53 people who also had giant cell arteritis, 37 of them had developed this while being treated for polymyalgia rheumatica [Kremers et al, 2005].

A hospital-based study in the UK reported that 20% of people with polymyalgia rheumatica developed giant cell arteritis [Kyle and Hazleman, 1993].

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].

Prognosis

What is the prognosis?

Response to systemic corticosteroids is rapid and dramatic, with many symptoms resolving after a few days of treatment [Salvarani et al, 2002].

Treatment for 1–3 years is often required [Dasgupta et al, 2009], and some people may need low-dose corticosteroids for several years [Salvarani et al, 2008].

Relapse is common, but responds to restarting or increasing the dose of systemic corticosteroids.

Higher initial doses and faster tapering are predictors of future relapses [Salvarani et al, 2008].

A high baseline erythrocyte sedimentation rate (ESR), and persistently elevated ESR and serum levels of C-reactive protein (CRP), may be associated with increased risks of relapse [Salvarani et al, 2005].

Polymyalgia rheumatica is not associated with increased mortality [Andersson et al, 1986; Gran et al, 2001].

Diagnosis

Diagnosis of polymyalgia rheumatica

When to suspect PMR

When should I suspect polymyalgia rheumatica?

Suspect polymyalgia rheumatica (PMR) in a person over 50 years of age, presenting with at least 2 weeks of the core symptoms of:

Bilateral shoulder and/or pelvic girdle pain. Initially this may be unilateral but quickly becomes bilateral, is worse with movement, and interferes with sleep.

Shoulder pain may radiate to the elbows and is the presenting feature in 70–95% of people.

Hip and neck pain is the presenting feature in 50–70% of people. Hip pain may radiate to the knees.

Stiffness lasting for at least 45 minutes after waking or periods of rest that may cause the person to have difficulty turning over in bed, rising from a bed or a chair, or raising their arms above shoulder height.

Additional features that may accompany these core symptoms include:

Low-grade fever, fatigue, anorexia, weight loss, and depression — systemic symptoms occur in up to half of people with PMR.

Bilateral upper arm tenderness — sometimes present.

Peripheral musculoskeletal signs — seen in approximately 50% of people and include:

Carpal tunnel syndrome.

Peripheral arthritis (predominantly affecting the knees and wrists), which is asymmetric and self-limiting.

Swelling with pitting oedema of hands, wrists, feet, and ankles.

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.

Basis for recommendation

Basis for recommendation

This recommendation is based on published expert opinion [Salvarani et al, 2002; Michet and Matteson, 2008; Salvarani et al, 2008; van Hecke, 2011; Kermani and Warrington, 2013], and guidelines from the British Society for Rheumatology and British Health Professionals in Rheumatology [Dasgupta et al, 2009].

Diagnosis

How do I know my patient has it?

Polymyalgia rheumatica (PMR) is diagnosed by identifying core features of the condition, excluding conditions that mimic PMR, and by a positive response to oral corticosteroids. For a person with core symptoms of PMR who is over 50 years of age:

Request an ESR/plasma viscosity and/or CRP (in addition to blood tests to help exclude other conditions).

Raised inflammatory markers are supportive of a diagnosis of PMR, although if the clinical picture and response to steroids are typical the diagnosis can be made with normal inflammatory markers.

Exclude:

Giant cell arteritis because immediate high doses of oral steroids are required to reduce the risk of serious complications.

Features include abrupt onset headache (usually temporal) and temporal tenderness; visual disturbance, including diplopia; jaw or tongue claudication; prominence, beading, or diminished pulse on examination of the temporal artery; upper cranial nerve palsies; limb claudication or other evidence of large vessel involvement.

For more information on diagnosis and management, see the CKS topic on Giant cell arteritis.

Active infection or cancer because treatment with corticosteroids may mask these conditions.

Other conditions associated with pain and/or stiffness that could be mistaken for PMR such as arthritis, or muscle diseases such as statin-induced myalgia or myositis.

For more information about other conditions to consider and tests to identify them, see Differential diagnosis.

Arrange the following tests in all people with suspected PMR to rule out other conditions before starting corticosteroids: full blood count, urea and electrolytes, liver function tests, calcium, alkaline phosphatase, protein electrophoresis, thyroid stimulating hormone, creatine kinase, rheumatoid factor, and dipstick urinalysis.

Consider the following tests depending on the clinical features: a urine specimen for Bence Jones protein; blood tests for anti-nuclear antibody and anti-cyclic citrullinated peptide antibody; and chest X-ray.

Refer people with atypical features of PMR who do not have a clear alternative cause for their symptoms including people who:

Are less than 60 years of age.

Have red flags suggestive of a serious underlying condition such as weight loss, night pain, or neurological features. 

Do not have the core features of PMR including:

Bilateral shoulder or pelvic girdle aching.

Stiffness lasting for at least 45 minutes after waking or periods of rest.

Have clinical features that are uncommon with PMR including people:

With normal inflammatory markers; or ESR of more than 100 mm/hour; or very high CRP.

With a chronic onset of their symptoms.

If PMR is the most likely diagnosis:

Prescribe a trial of oral prednisolone 15 mg daily and arrange follow up after 1 week to assess clinical response.

After 3–4 weeks of treatment:

Consider reducing the dose of prednisolone.

Recheck ESR/plasma viscosity and/or CRP to assess response to treatment.

Make a working diagnosis of PMR if there is a patient-reported global improvement of 70% or more within a week, and normalization of inflammatory markers within 4 weeks.

If there is a lesser response, consider increasing the dose to 20 mg prednisolone and reassess response.

If, despite increasing the dose of prednisolone, response is still less than 70%, reconsider the diagnosis and refer to an appropriate specialist.

Basis for recommendation

Basis for recommendation

These recommendations are largely based on the British Society for Rheumatology (BSR) and British Health Professionals in Rheumatology (BHPR) guidelines for the management of polymyalgia rheumatica (PMR). These guidelines recommend an approach to the diagnosis of PMR that discourages starting corticosteroids before a full assessment of the underlying cause has been made. This approach aims to avoid diagnostic confusion in people with other conditions that respond to corticosteroids and has been agreed by the International PMR Classification Criteria Group [Dasgupta et al, 2009].

Diagnosing PMR

There is no clinical feature, test result or response to treatment that is unique to PMR. The diagnosis requires identification of the characteristic clinical features of PMR (including a characteristic acute phase response and a positive response to oral corticosteroids) while excluding other causes for these features.

Core inclusion criteria of PMR

The most useful clinical features supporting a diagnosis of PMR have been included in core inclusion criteria recommended in the BSR/BHPR guideline. These criteria are based on expert opinion and are in the process of being validated in a prospective case control study [Dasgupta et al, 2009]. They are largely consistent with diagnostic criteria proposed by other experts [Kermani and Warrington, 2013] and are as follows:

Age over 50 years.

Duration more than 2 weeks.

Bilateral shoulder and/or pelvic girdle aching.

Morning stiffness lasting more than 45 minutes.

Evidence of an acute phase response.

The BSR/BHPR guideline states that PMR can be diagnosed with normal inflammatory markers if the clinical picture and response to corticosteroid treatment are typical, but this will be in secondary care as referral is recommended for people with normal inflammatory markers [Dasgupta et al, 2010].

Excluding other conditions

The recommendation to exclude other conditions is based on the rationale that the symptoms of PMR (including proximal pain and stiffness) are not unique to this condition [Dasgupta et al, 2009].

Giant cell arteritis is associated with 10% of PMR cases [Dasgupta et al, 2009] and must always be identified because it requires urgent treatment with high-dose corticosteroids to reduce the risk of serious complications [Gran, 2010].

The BSR/BHPR guideline recommends undertaking an extensive list of other laboratory investigations before starting corticosteroids to exclude other conditions that can present in a similar way, respond to corticosteroids, and cause diagnostic confusion [Dasgupta et al, 2009].

Although people with atypical features (such as a normal ESR) may have PMR, there is an increased possibility that another condition may be the cause for their symptoms. Experts recommend that in this situation referral for specialist assessment of the cause is undertaken [Dasgupta et al, 2009].

Referral of people with atypical features of PMR

This recommendation reflects the BSR/BHPR guideline, and is based on evidence from non-analytic studies [Dasgupta et al, 2009].

The definition of a very high ESR as being more than 100 mm/hour is based on information in an implementation of the BSR/BHPR guideline from the Royal College of Physicians [RCP, 2010].

Trial of corticosteroids

Unblinded, uncontrolled, prospective and retrospective cohort studies suggest that corticosteroids are an effective treatment for polymyalgia rheumatica. Corticosteroids have not been evaluated in placebo-controlled clinical trials, and it is unlikely that these would ever take place because the effect of corticosteroids is so dramatic.

Although a response to corticosteroids is considered by some to be the main defining feature of PMR, there were concerns from the BSR and BHPR that the anti-inflammatory properties of corticosteroids can mask symptoms of a number of other conditions (including cancer, infection, osteoarthritis, rheumatoid arthritis, and rotator cuff problems) [Dasgupta et al, 2009].

A consensus of 90% of the experts contributing to the BSR/BHPR guideline agreed that 15 mg of oral prednisolone daily is the most appropriate starting dose for someone with suspected PMR. This is supported by observational studies using doses between 10 mg and 20 mg. Doses of less than 10 mg were associated with a higher rate of relapse, and doses higher than 20 mg were associated with more adverse events [Dasgupta et al, 2009].

The definition of a response to corticosteroids (global improvement of 70% within a week and normalization of inflammatory markers within 4 weeks) was agreed by more than three quarters of the BSR/BHPR guideline consensus group [Dasgupta et al, 2009].

The BSR/BHPR guideline recommends the use of intramuscular methylprednisolone in milder cases of polymyalgia rheumatica [Dasgupta et al, 2009]. However, CKS does not recommend this because intramuscular methylprednisolone is not routinely used in primary care.

Early referral for people with a poor response to corticosteroids is based on the expert opinion of the authors of the BSR/BHPR guideline that a poor response to treatment may be indicative of a serious underlying condition being the cause of symptoms [Dasgupta et al, 2009].

Differential diagnosis

What else might it be?

Exclusion of other conditions is essential to making a working diagnosis of polymyalgia rheumatica (PMR). See Table 1 for examples of disorders that can cause similar symptoms to PMR.

Table 1 . Disorders that can cause similar symptoms to polymyalgia rheumatica.
Differential diagnosis Examples Clinical features
Degenerative disorders (may coexist with polymyalgia rheumatica and increase the need for steroid therapy) Cervical and lumbar spondylosis Neck and back pain. For more information, see the CKS topics on Neck pain - cervical radiculopathy and Back pain - low (without radiculopathy).
Osteoarthritis Commonly involves hands, hips, knees, and spine. Pain improves with rest and increases with joint use and at the end of the day. For more information, see the CKS topic on Osteoarthritis.
Bilateral adhesive capsulitis (frozen shoulder), rotator cuff disorders Pain, stiffness, and limitation of active and passive movement with frozen shoulder. Painful arc of movement and limited active range of movement with rotator cuff disorders. For more information, see the CKS topic on Shoulder pain.
Endocrine disorders Thyroid disease Wide range of symptoms and signs including tiredness, weakness, weight gain, paraesthesias; abnormal TSH levels. For more information, see the CKS topics on Hyperthyroidism and Hypothyroidism.
Parathyroid disease (hyperparathyroidism causing hypercalcaemia) Bone pain, muscular weakness, gastrointestinal symptoms (e.g. anorexia, nausea), renal stones, cardiac arrhythmias, neurological symptoms (e.g. depression, confusion).
Infection Viral illness Variety of symptoms, for example influenza commonly includes myalgia, weakness and fatigue, malaise, sore throat, dry cough.
Chronic osteomyelitis Variable pain and disability, possibly evidence of soft tissue swelling and bony tenderness. Systemic features common (e.g. weight loss, malaise).
Tuberculosis Variable presentation, but may include malaise, night sweats, fever, loss of weight. For more information, see the CKS topic on Tuberculosis.
Infective endocarditis Night sweats, anorexia, weight loss, splinter haemorrhages, Osler nodes, finger clubbing, and splenomegaly.
Inflammatory disorders Rheumatoid arthritis, often seronegative for rheumatoid factor (common) Deforming and destructive polyarthritis. Sometimes extra-articular manifestations (e.g. subcutaneous nodules, vasculitis). For more information, see the CKS topic on Rheumatoid arthritis.
Spondyloarthropathy (rare) Predominant involvement of axial and peripheral joints and tendon insertions. Inflammatory back pain, sacroiliitis, peripheral arthritis; sometimes skin, gastrointestinal and eye involvement.
Remitting seronegative symmetric synovitis with pitting oedema (rare) Morning stiffness and joint swelling and pitting oedema of the hands and feet.
Polymyositis/dermatomyositis Painless muscle weakness; cutaneous changes in dermatomyositis. Typically increased serum creatine kinase.
Systemic lupus erythematosus Constitutional symptoms common; also musculoskeletal (e.g. athralgia, arthritis, myalgia); skin (e.g. alopecia, butterfly rash); cardiopulmonary; and neurological features.
Malignancy Multiple myeloma Weakness, fatigue, bone pain. Less commonly renal failure, hypercalcaemia, and acute infection.
Acute leukaemia Pallor, petichiae, bruising, fever.
Lymphoma Palpable non-tender lymphadenopathy. Less commonly fever, night sweats, weight loss.
Lung carcinoma Apical (Pancoast) tumour may cause progressive, constant pain in the shoulder, upper chest or interscapular region. Also possibly weakness of hand muscles, Horner's syndrome, hoarseness.
Drug adverse effects Myositis or myalgia due to statins Drug history, increased creatine kinase.
Polymyalgia rheumatica-like syndrome due to quinidine Drug history.
Miscellaneous Osteomalacia Bone pain and tenderness, skeletal deformity, and proximal muscle weakness. Frequently results from lack of vitamin D.
Fibromyalgia Tender spots, longstanding history.
Chronic fatigue syndrome Mental and physical fatigue which is exacerbated by activity. May be associated with muscle and joint pain; depression and anxiety common.
[Hazleman, 1998; Jennings and Read, 2005; Aronson, 2006; Salvarani et al, 2008; Dasgupta et al, 2009; Derlet, 2009; Armitage, 2010; Berendt and McNally, 2010; Braun and Sieper, 2010; Brion and Kalunian, 2010; Chaisson and Nachega, 2010; Littler, 2010; Nath Maini, 2010; Rahman and Isenberg, 2010; Sharpe, 2010; Smith and Wordsworth, 2010; Spiro, 2010; Stone, 2010; Thakker, 2010; Weetman, 2010]

Basis for recommendation

Basis for recommendation

These recommendations are based on British Society for Rheumatology (BSR) and British Health Professionals in Rheumatology (BHPR) guidelines for the management of polymyalgia rheumatica [Dasgupta et al, 2009], published expert opinion in a textbook: The vasculitides: polymyalgia rheumatica and giant cell arteritis [Hazleman, 1998], and a review article [Salvarani et al, 2008].

Management

Management

Scenario: Management : covers the initial and ongoing management of polymyalgia rheumatica as well as how to manage relapses and when to refer.

Scenario: Management

Scenario: Management of polymyalgia rheumatica

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Management

How do I manage someone with polymyalgia rheumatica?

For people in whom a working diagnosis of polymyalgia rheumatica (PMR) has been made:

Reduce the dose of prednisolone slowly when symptoms are fully controlled. A suggested schedule for reducing prednisolone is provided below, but smaller dose reductions and longer durations at each dose may be needed to avoid relapses in some individuals. Typically, treatment is required for between 1 and 3 years.

For people who are well controlled on 15 mg daily 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.

Arrange routine reviews a week after any change in dose and at least every 3 months in the first year following diagnosis.

At all routine reviews ask about:

Symptoms of giant cell arteritis including an abrupt onset headache (usually temporal) and temporal tenderness; visual disturbance, or jaw or tongue claudication and, if present, see the CKS topic on Giant cell arteritis.

Relapsing symptoms of PMR and if present see 'If relapse occurs' below.

Adverse effects of corticosteroids such as weight gain, dyspepsia, muscle weakness, skin thinning and easy bruising.

At 3 monthly reviews assess blood pressure and glucose which may be adversely affected by corticosteroids.

Only consider monitoring of full blood count (FBC) and urea and electrolytes if there is a clinical indication (for example FBC if there is a suspicion of gastric bleeding secondary to corticosteroids; urea and electrolytes if concurrent prednisolone and diuretic use, or previously abnormal urea and electrolytes secondary to corticosteroids).

Advise the person to arrange a review at other times:

Urgently if they develop symptoms of giant cell arteritis, including an abrupt onset headache (usually temporal) and temporal tenderness; visual disturbance, or jaw or tongue claudication. If present see the CKS topic on Giant cell arteritis.

Routinely if they develop symptoms of relapsing PMR, including proximal pain, fatigue, and morning stiffness. If relapse occurs see below.

If relapse (recurrence of PMR symptoms) occurs:

Reassess the diagnosis.

Increase prednisolone to the previous dose that controlled symptoms and monitor the response.

If symptoms settle, continue on this dose and consider tapering the prednisolone dose more cautiously by increasing the scheduled dose durations or reducing the scheduled dose reductions to try to prevent further relapses.

If symptoms do not settle, seek specialist advice.

Refer for specialist management if:

It is not possible to reduce corticosteroids at reasonable intervals without causing relapse.

Corticosteroids are required for more than 2 years.

Assess and manage osteoporotic fracture risk. For more information see the CKS topic on Osteoporosis - prevention of fragility fractures.

Ensure the person is provided with a blue steroid card, and discuss potential adverse effects of corticosteroids. In particular, advise them:

Not to stop taking prednisolone abruptly and to seek medical advice if they are experiencing problems taking it.

To avoid close contact with people who have chickenpox, shingles, or measles if they do not have immunity to chickenpox or measles and to seek medical advice if they are exposed.

Provide written information on PMR and regional patient support groups. This is available at www.pmrgcauk.com.

Basis for recommendation

Basis for recommendation

These recommendations are based on the British Society for Rheumatology (BSR) and British Health Professionals in Rheumatology (BHPR) guidelines for the management of polymyalgia rheumatica (PMR) [Dasgupta et al, 2009] and expert opinion in review articles [Michet and Matteson, 2008; Salvarani et al, 2008].

Prednisolone dose reduction

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, 2009].

The suggested tapering schedule is that proposed by the guideline from the BSR/BHPR. The guideline working group found evidence for a steroid regimen to be lacking, and therefore based their suggested dosage regimen on a consensus decision. This guideline discussed the need for flexibility when tailoring treatment for an individual and acknowledges that, in practice, regimens may vary from the one suggested [Dasgupta et al, 2009]. The schedules suggested by CKS expert reviewers varied considerably and locally recommended schedules may differ.

Some experts recommend the use of intramuscular methylprednisolone in milder cases of polymyalgia rheumatica [Dasgupta et al, 2009]. However, intramuscular methylprednisolone is not routinely used in primary care.

Follow up

The guideline from the BSR/BHPR suggests, on the basis of expert opinion [Dasgupta et al, 2009]:

Early review at 1–3 and 6 weeks after commencement of corticosteroids (for more information, see Diagnosis).

Follow up at 3, 6, 9, and 12 months in the first year, with additional visits if the person relapses or experiences adverse events.

In addition, CKS recommends follow up after each dose change to identify any symptoms of relapse and to make any necessary adjustments to the withdrawal schedule.

The BHR/BHPR guideline recommends monitoring symptoms, adverse effects of corticosteroids, and osteoporotic risk; reassessing the diagnosis; and monitoring blood tests, based on evidence from observational studies and the expert opinion of the guideline development group.

The recommendation to monitor full blood count and urea and electrolytes depending on clinical judgement is pragmatic. CKS considers that routine monitoring of these parameters is unlikely to alter management of PMR, but these tests may help to inform the management of comorbid conditions.

Although a reduction in ESR in response to prednisolone is useful in making a working diagnosis of PMR, an isolated raised ESR or CRP once a diagnosis of PMR has been made is not a definite indicator of relapse (which is defined as recurrence of symptoms). CKS has therefore not recommended the routine monitoring of inflammatory markers.

Relapses

These recommendations are consistent with the guideline from the BSR and BHPR [Dasgupta et al, 2009], a summary of this guideline published by the Royal College of Physicians [RCP, 2010], and expert opinion in review articles [Michet and Matteson, 2008; Salvarani et al, 2008].

The guideline group based their recommendations on managing relapse on evidence from observational studies [RCP, 2010].

CKS recommends referral for third and subsequent relapses because specialists can reassess the diagnosis and may consider the use of steroid-sparing treatments such as disease-modifying antirheumatic drugs for people who experience frequent relapses, although there is a lack of good-quality evidence to support their use [Dasgupta et al, 2009].

Referral

These referral recommendations are extrapolated from the BSR/BHPR guideline [Dasgupta et al, 2009] and a summary of this guideline from the Royal College of Physicians [RCP, 2010]. The recommendations are also supported by expert opinion in a review article [Kermani and Warrington, 2013].

Osteoporosis prophylaxis

The recommendation to assess and treat osteoporotic fracture risk is consistent with UK guidelines from the National Institute for Health and Care Excellence [NICE, 2012] and the National Osteoporosis Guideline Group [NOGG, 2013]. More detail on the evidence influencing their recommendations can be found in the CKS topic on Osteoporosis - prevention of fragility fractures.

Advice and information

Advice on prednisolone treatment has been extrapolated by CKS from information in the guideline from the BSR and BHPR on the management of polymyalgia rheumatica [Dasgupta et al, 2009].

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 [BNF 65, 2013]. However, the Department of Health's Green Book states that immunosuppression should be considered in people receiving 40 mg prednisolone for more than a week (more than the dose recommended in this topic), but that there may occasionally be people on lower doses of corticosteroids who may be immunosuppressed and at increased risk from infections [DH, 2012].

Important aspects of prescribing information relevant to primary healthcare are covered in this section specifically for the drugs recommended in this CKS topic. For further information on contraindications, cautions, drug interactions, and adverse effects, see the electronic Medicines Compendium (eMC) (http://medicines.org.uk/emc), or the British National Formulary (BNF) (www.bnf.org).

Corticosteroids

Corticosteroids

For information about prescribing prednisolone, including contraindications, adverse effects, and drug interactions, see the CKS topic on Corticosteroids - oral.

Evidence

Evidence

Supporting evidence

Corticosteroids

Evidence on the use of corticosteroids for polymyalgia rheumatica

Unblinded, uncontrolled, prospective and retrospective cohort studies strongly suggest that corticosteroids are the only effective treatment for polymyalgia rheumatica. Corticosteroids have not been evaluated in placebo-controlled clinical trials. Because the effect of corticosteroids (both on starting treatment and on withdrawing treatment) is so dramatic, it is highly unlikely that placebo-controlled trials will ever be done.

Search strategy

Scope of search

A literature search was conducted for guidelines, systematic reviews and randomized controlled trials on primary care management of polymyalgia rheumatica.

Search dates

November 2008 - July 2013

Key search terms

Various combinations of searches were carried out. The terms listed below are the core search terms that were used for Medline.

exp Polymyalgia Rheumatica/, polymyalgia rheumatica.tw., PMR.tw.

Table 1 . Key to search terms.
Search commands Explanation
/ indicates a MeSH subject heading with all subheadings selected
.tw indicates a search for a term in the title or abstract
exp indicates that the MeSH subject heading was exploded to include the narrower, more specific terms beneath it in the MeSH tree
$ indicates that the search term was truncated (e.g. wart$ searches for wart and warts)
Sources of guidelines

National Institute for Health and Care Excellence (NICE)

Scottish Intercollegiate Guidelines Network (SIGN)

Royal College of Physicians

Royal College of General Practitioners

Royal College of Nursing

NICE Evidence

Health Protection Agency

National Guidelines Clearinghouse

Guidelines International Network

TRIP database

GAIN

Institute for Clinical Systems Improvement

National Health and Medical Research Council (Australia)

Royal Australian College of General Practitioners

British Columbia Medical Association

Canadian Medical Association

Towards Optimal Practice

University of Michigan Medical School

Michigan Quality Improvement Consortium

Patient UK Guideline links

Driver and Vehicle Licensing Agency

Medline (with guideline filter)

Sources of systematic reviews and meta-analyses

The Cochrane Library :

Systematic reviews

Protocols

Database of Abstracts of Reviews of Effects

Medline (with systematic review filter)

EMBASE (with systematic review filter)

Sources of health technology assessments and economic appraisals

The Cochrane Library :

NHS Economic Evaluations

Health Technology Assessments

Canadian Agency for Drugs and Technologies in Health

International Network of Agencies for Health Technology Assessment

NIHR Health Technology Assessment programme

Sources of randomized controlled trials

The Cochrane Library :

Central Register of Controlled Trials

Medline (with randomized controlled trial filter)

EMBASE (with randomized controlled trial filter)

Sources of evidence based reviews and evidence summaries

Drug & Therapeutics Bulletin

Central Services Agency COMPASS Therapeutic Notes

Sources of national policy

Department of Health

Health Management Information Consortium (HMIC)

Patient experiences

Healthtalkonline

BMJ - Patient Journeys

Patient.co.uk - Patient Support Groups

Sources of medicines information

The following sources are used by CKS pharmacists and are not necessarily searched by CKS information specialists for all topics. Some of these resources are not freely available and require subscriptions to access content.

British National Formulary (BNF)

electronic Medicines Compendium (eMC)

European Medicines Agency (EMEA)

LactMed

Medicines and Healthcare products Regulatory Agency (MHRA)

REPROTOX

Scottish Medicines Consortium

Stockley's Drug Interactions

TERIS

TOXBASE

Micromedex

UK Medicines Information

References

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Armitage, J.O. (2010) Lymphoma. In: Warrell, D.A., Cox, T.M., Firth, J.D. and Ogg.G.S. (Eds.) Oxford textbook of medicine. 5th edn. Oxford: Oxford University Press. 4317-4334.

Aronson, J.K. (Ed.) (2006) Meyler's side effects of drugs. The international encyclopedia of adverse drug reactions and interactions. Volume 5: P-S. 15th edn. Amsterdam: Elsevier.

Berendt, A.R. and McNally, M. (2010) Osteomyelitis. In: Warrell, D.A., Cox, T.M., Firth, J.D. and Ogg.G.S. (Eds.) Oxford textbook of medicine. 5th edn. Oxford: Oxford University Press. 3788-3795.

BNF 65 (2013) British National Formulary. 65th edn. London: British Medical Association and Royal Pharmaceutical Society of Great Britain.

Braun, J. and Sieper, J. (2010) Ankylosing spondylitis, other spondyloarthritides, and related conditions. In: Warrell, D.A., Cox, T.M., Firth, J.D. and Ogg.G.S. (Eds.) Oxford textbook of medicine. 5th edn. Oxford: Oxford University Press. 3603-3616.

Brion, P.H. and Kalunian, K.C. (2010) Osteoarthritis. In: Warrell, D.A., Cox, T.M., Firth, J.D. and Ogg.G.S. (Eds.) Oxford textbook of medicine. 5th edn. Oxford: Oxford University Press. 3628-3636.

Chaisson, R.E. and Nachega, J.B. (2010) Tuberculosis. In: Warrell, D.A., Cox, T.M., Firth, J.D. and Ogg.G.S. (Eds.) Oxford textbook of medicine. 5th edn. Oxford: Oxford University Press. 810-831.

Dasgupta, B., Matteson, E.L. and Maradit-Kremers, H. (2007) Management guidelines and outcome measures in polymyalgia rheumatica (PMR). Clinical & Experimental Rheumatology 25(6 Suppl 47), 130-136. [Abstract] [Free Full-text]

Dasgupta, B., Borg, F.A., Hassan, N. et al. (2009) BSR and BHPR guidelines for the management of polymyalgia rheumatica (full guideline). British Society for Rheumatology and British Health Professionals in Rheumatology. http://rheumatology.oxfordjournals.org [Free Full-text]

Dasgupta, B., Borg, F.A., Hassan, N. et al. (2010) BSR and BHPR guidelines for the management of polymyalgia rheumatica. Rheumatology 49(1), 186-190. [Free Full-text]

Derlet, R.W. (2009) Influenza. emedicineWebMD. www.emedicine.com [Free Full-text]

DH (2012) Immunisation against infectious disease- "The Green Book". Chapter 34 - Varicella. Department of Health. www.dh.gov.uk [Free Full-text]

Gran, J.T. (2010) Polymyalgia rheumatica and temporal arteritis. In: Warrell, D.A., Cox, T.M., Firth, J.D. and Ogg, G.S. (Eds.) Oxford textbook of medicine. 5th edn. Oxford: Oxford University Press. 3679-3684.

Gran, J.T., Myklebust, G., Wilsgaard, T. and Jacobsen, B.K. (2001) Survival in polymyalgia rheumatica and temporal arteritis: a study of 398 cases and matched population controls. Rheumatology 40(11), 1238-1242. [Abstract] [Free Full-text]

Hazleman, B.L. (1998) The vasculitides: polymyalgia rheumatica and giant cell arteritis. In: Klippel, J.H. and Dieppe, P.A. (Eds.) Rheumatology. 2nd edn. London: Mosby International. Chapter 21. 21.1-21.8.

Hunder, G.G. (1997) Giant cell arteritis in polymyalgia rheumatica. American Journal of Medicine 102(6), 514-516.

Jennings, R. and Read, R.C. (2005) Influenza in practice. London: Royal Society of Medicine Press Ltd.

Kermani, T.A. and Warrington, K.J. (2013) Polymyalgia rheumatica. Lancet 381(9860), 63-72. [Abstract]

Kremers, H.M., Reinalda, M.S., Crowson, C.S. et al. (2005) Relapse in a population based cohort of patients with polymyalgia rheumatica. Journal of Rheumatology 32(1), 65-73. [Abstract]

Kyle, V. and Hazleman, B.L. (1993) The clinical and laboratory course of polymyalgia rheumatica/giant cell arteritis after the first two months of treatment. Annals of the Rheumatic Diseases 52(12), 847-850. [Abstract] [Free Full-text]

Littler, W.A. (2010) Infective endocarditis. In: Warrell, D.A., Cox, T.M., Firth, J.D. and Ogg.G.S. (Eds.) Oxford textbook of medicine. 5th edn. Oxford: Oxford University Press. 2806-2822.

MHRA (2007) Steroids: ten questions and answers for patients. Medicines and Healthcare products Regulatory Agency. www.mhra.gov.uk [Free Full-text]

Michet, C.J. and Matteson, E.L. (2008) Polymyalgia rheumatica. British Medical Journal 336(7647), 765-769.

Nath Maini, R. (2010) Rheumatoid arthritis. In: Warrell, D.A., Cox, T.M., Firth, J.D. and Ogg.G.S. (Eds.) Oxford textbook of medicine. 5th edn. Oxford: Oxford University Press. 3579-3602.

NICE (2012) Osteoporosis: assessing the risk of fragility fracture (NICE guideline). . Clinical guideline 146. National Institute for Health and Care Excellence. www.nice.org.uk [Free Full-text]

NOGG (2013) Osteoporosis clinical guideline for prevention and treatment: executive summary. National Osteoporosis Guideline Group. www.shef.ac.uk/NOGG [Free Full-text]

Rahman, A. and Isenberg, D.A. (2010) Systemic lupus erythematosus and related disorders. In: Warrell, D.A., Cox, T.M., Firth, J.D. and Ogg.G.S. (Eds.) Oxford textbook of medicine. 5th edn. Oxford: Oxford University Press. 3652-3664.

RCP (2010) Diagnosis and management of polymyalgia rheumatica. Royal College of Physicians. www.rcplondon.ac.uk [Free Full-text]

Salvarani, C., Cantini, F., Boiardi, L. and Hunder, G.G. (2002) Polymyalgia rheumatica and giant-cell arteritis. New England Journal of Medicine 347(4), 261-271.

Salvarani, C., Cantini, F., Niccoli, L. et al. (2005) Acute-phase reactants and the risk of relapse/recurrence in polymyalgia rheumatica: a prospective followup study. Arthritis & Rheumatism 53(1), 33-38. [Abstract] [Free Full-text]

Salvarani, C., Cantini, F. and Hunder, G.G. (2008) Polymyalgia rheumatica and giant-cell arteritis. Lancet 372(9634), 234-245. [Abstract]

Sharpe, M. (2010) Chronic fatigue syndrome (postviral fatigue syndrome, neurosthenia, and myalgic encephalomyelitis). In: Warrell, D.A., Cox, T.M., Firth, J.D. and Ogg.G.S. (Eds.) Oxford textbook of medicine. 5th edn. Oxford: Oxford University Press. 5304-5308.

Smeeth, L., Cook, C. and Hall, A.J. (2006) Incidence of diagnosed polymyalgia rheumatica and temporal arteritis in the United Kingdom, 1990-2001. Annals of the Rheumatic Diseases 65(8), 1093-1098. [Abstract] [Free Full-text]

Smith, R. and Wordsworth, B.P. (2010) Skeletal disorders - general approach and clinical conditions. In: Warrell, D.A., Cox, T.M., Firth, J.D. and Ogg.G.S. (Eds.) Oxford textbook of medicine. 5th edn. Oxford: Oxford University Press. 3719-3770.

Spiro, S.G. (2010) Lung cancer. In: Warrell, D.A., Cox, T.M., Firth, J.D. and Ogg.G.S. (Eds.) Oxford textbook of medicine. 5th edn. Oxford: Oxford University Press. 3514-3533.

Stone, J.H. (2010) Polymyositis and dermatomyositis. In: Warrell, D.A., Cox, T.M., Firth, J.D. and Ogg.G.S. (Eds.) Oxford textbook of medicine. 5th edn. Oxford: Oxford University Press. 3692-3698.

Thakker, R.V. (2010) Parathyroid disorders and disease altering calcium metabolism. In: Warrell, D.A., Cox, T.M., Firth, J.D. and Ogg.G.S. (Eds.) Oxford textbook of medicine. 5th edn. Oxford: Oxford University Press. 1851-1868.

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