Changes

February 2024 — reviewed. A literature search was conducted in January 2024 to identify evidence-based guidelines, UK policy, systematic reviews, and key randomized controlled trials published since the last revision of the topic. No major changes to recommendations have been made.

Update

Goals

To support primary healthcare professionals to:

• Make a diagnosis of polymyalgia rheumatica. 
• Control symptoms of polymyalgia rheumatica.
• Preserve mobility, independence, and quality of life.
• Minimize the risk of complications, for example, blindness.
• Minimize the risks of corticosteroid treatment.
• Provide appropriate information and advice.

Outcome measures

Audit criteria

QOF indicators

QIPP - Options for local implementation

NICE quality standards

What is it?

• Polymyalgia rheumatica is a chronic, systemic rheumatic inflammatory disease characterized by pain and morning stiffness in the neck, shoulder, and pelvic girdle in people older than 50 years of age. It is best understood as a syndrome that is associated with synovitis of proximal large joints, tenosynovitis, and bursitis.

[Mahmood, 2020; Lundberg, 2022; BMJ Best Practice, 2023; Espígol-Frigolé, 2023; Florescu, 2023]

What are the causes and risk factors?

• The cause of polymyalgia rheumatica (PMR) is unknown, although genetic and environmental factors are thought to contribute to disease susceptibility and severity.
• Risk factors for PMR include:
  • Older age — the incidence of PMR increases progressively with age in both men and women. The highest incidence is in people older than 65, with a peak in the 70–80 age group. It is seldom diagnosed in people younger than 50 years of age. 
  • Female sex — PMR is more common in women than men. Over 65% of people who have PMR are women, and the lifetime risk for developing PMR is estimated at 2.4% for women and 1.7% for men. 
  • Northern European ancestry — PMR is most common in people of Northern European ancestry, with an incidence of 41–113 cases per 100,000 persons and a prevalence of about 6 cases per 1000 persons in those older than 50 years. It is uncommon in people of Middle Eastern, Asian, African, and Hispanic descent. 
  • Infection — cyclic fluctuations and peaks in incidence have been observed in the winter and associated with epidemics of mycoplasma, chlamydia pneumonia, and parvovirus B19 infections.

[González-Gay, 2017; Matteson, 2017; Camellino, 2019; Mahmood, 2020; Rosenberg, 2021; Lundberg, 2022; BMJ Best Practice, 2023; Florescu, 2023]

How common is it?

• Polymyalgia rheumatica (PMR) is among the most common inflammatory rheumatic diseases in older people [Matteson, 2017; Partington, 2018; Mahmood, 2020; Lundberg, 2022; Florescu, 2023], and is one of the most common indications for long-term corticosteroid treatment in the UK, accounting for 22% of prescriptions [Dasgupta, 2007; Ameer, 2014].
• The incidence varies depending on geographical location, with more cases in Northern Europe (113 per 100,000 people per year in Norway) than in Southern Europe (13 per 100,000 people per year in Italy) [Lundberg, 2022; Espígol-Frigolé, 2023].
• A large population-based study (n = 5,364,005) assessed the incidence, prevalence, and treatment burden of PMR in the UK over two decades (1990–2016) in people aged over 40 years [Partington, 2018]:
  • During the period, 42,125 people had an incident diagnosis of PMR. The overall incidence rate of PMR was 95.9 per 100,000, and the incidence was highest in women, older age groups, and those living in the South of England. 
  • The prevalence of PMR in 2015 was 0.85%.
• A study of primary care computer records assessed the prevalence of PMR and giant cell arteritis (GCA) in a GP practice in Norfolk by reviewing clinical data for diagnoses of both conditions. This was supplemented by postal survey (to identify potentially undiagnosed cases within the practice population) and subsequent clinical review (for those screening positive for potential diagnoses of PMR and GCA [Yates, 2016]. 
  • From the GP case records (n = 5159), 117 people had a recorded diagnosis of PMR, and 21 people had GCA.
  • From the population survey (n = 4728), no new cases of PMR and GCA were identified among the 2227 completed questionnaires returned.
  • The cumulative prevalence estimate in those aged 55 years and older meeting 5 published criteria sets for PMR ranged from 0.91–1.53%.
  • The prevalence of both conditions was higher in women than in men and in older age groups.

What are the complications?

• Giant cell arteritis (GCA) can co-develop with polymyalgia rheumatica (PMR) or can occur at any time during the course of PMR [Espígol-Frigolé, 2023]. 
  • About 15–20% of people with PMR develop GCA, and up to 60% of people with GCA have symptoms of PMR [Camellino, 2019].
  • See the CKS topic on Giant cell arteritis for more information.
• Complications of long-term corticosteroid treatment may occur in up to 65% of people [Camellino, 2019].
  • See the CKS topic on Corticosteroids - oral for more information on complications and adverse effects of oral corticosteroids.

What is the prognosis?

• The overall prognosis of polymyalgia rheumatica (PMR) is good. 
• Response to systemic corticosteroids is often rapid and dramatic, with many symptoms resolving within 24–72 hours [BMJ Best Practice, 2023]. However:
  • Up to 50% of people with PMR do not adequately respond to systemic corticosteroids within four weeks of treatment [Camellino, 2019; Espígol-Frigolé, 2023].
  • The minimum expected course of corticosteroid treatment is 9–12 months. However, treatment for three to five years is not uncommon and in some cases, may be lifelong [Camellino, 2019; Buttgereit, 2020; Espígol-Frigolé, 2023].
  • Relapse is common [González-Gay, 2017; BMJ Best Practice, 2023], but usually responds to restarting or increasing the dose of systemic corticosteroids. Less commonly, there is a chronic relapsing course that may require prolonged treatment [BMJ Best Practice, 2023].
    • The role of risk factors for relapse or prolonged treatment of PMR is unclear: evidence from some low-to moderate-quality PMR studies suggests that female sex, high erythrocyte sedimentation rate (more than 40 mm/hour), and peripheral arthritis are associated with a higher relapse rate and/or prolonged treatment. However, other studies (also low-to-moderate-quality) failed to demonstrate an association between these factors and relapse or prolonged treatment [Dejaco, 2015; Camellino, 2019].
• PMR is not associated with increased mortality, but morbidity and mortality may occur due to corticosteroid adverse effects [BMJ Best Practice, 2023; Espígol-Frigolé, 2023].

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, worsens 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 40–50% 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.

How should I diagnose polymyalgia rheumatica?

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 erythrocyte sedimentation rate (ESR)/plasma viscosity and/or C-reactive protein (CRP), in addition to other blood tests to help exclude other conditions that can cause similar symptoms to PMR.
  • 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, such as vision loss.
    • 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; and 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 that can cause similar symptoms to PMR such as arthritis, thyroid disease, and statin-induced myalgia or myositis. For more information on other conditions to consider, see the section on 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 antinuclear antibody and anti-cyclic citrullinated peptide antibody, and chest X-ray.
• If PMR is the most likely diagnosis:
  • Prescribe a trial of oral prednisolone 15 mg daily and arrange a 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.
• Refer to a rheumatologist people with atypical features of PMR who do not have a clear alternative cause for their symptoms, including people who:
  • Are younger 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.
    • A chronic onset of symptoms.
• Make a working diagnosis of PMR if there is a patient-reported global improvement of 70% or more within a week of corticosteroid treatment 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.

What else might it be?

Exclusion of other conditions is essential to making a working diagnosis of polymyalgia rheumatica (PMR).

• Disorders that can cause similar symptoms to PMR include:
  • Degenerative disorders (may coexist with PMR and increase the need for steroid treatment). These include:
    • 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 the 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) and 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, including:
    • Thyroid disease — wide range of symptoms and signs, including tiredness, weakness, weight gain, paraesthesias, and abnormal thyroid stimulating hormone (TSH) levels. For more information, see the CKS topics on Hyperthyroidism and Hypothyroidism.
    • Parathyroid disease (hyperparathyroidism causing hypercalcaemia) — bone pain, muscular weakness, gastrointestinal symptoms (for example, anorexia and nausea), renal stones, cardiac arrhythmias, and neurological symptoms (for example, depression and confusion).
  • Infection, including:
    • Viral illness — variety of symptoms, for example, influenza commonly includes myalgia, weakness and fatigue, malaise, sore throat, and dry cough.
    • Chronic osteomyelitis — variable pain and disability and possible evidence of soft tissue swelling and bony tenderness. Systemic features (such as weight loss and malaise) are common.
    • Tuberculosis — variable presentation but may include malaise, night sweats, fever, and weight loss. 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, including:
    • Rheumatoid arthritis, often seronegative for rheumatoid factor (common) — deforming and destructive polyarthritis. Sometimes extra-articular manifestations (for example, subcutaneous nodules and vasculitis) are present. For more information, see the CKS topic on Rheumatoid arthritis.
    • Spondyloarthropathy (rare) — predominant involvement of axial and peripheral joints and tendon insertions. Other clinical features include inflammatory back pain, sacroiliitis, peripheral arthritis, and, sometimes, skin, gastrointestinal and eye involvement.
    • Remitting seronegative symmetric synovitis with pitting oedema (rare) — morning stiffness, joint swelling, and pitting oedema of the hands and feet.
    • Polymyositis/dermatomyositis — painless muscle weakness, cutaneous changes in dermatomyositis, and typically increased serum creatine kinase (CK) levels.
    • Systemic lupus erythematosus — constitutional symptoms common. There are also musculoskeletal (for example, arthralgia, arthritis, and myalgia), skin (for example, alopecia and butterfly rash), cardiopulmonary, and neurological features.
  • Cancer, including:
    • Multiple myeloma — weakness, fatigue, bone pain. Less commonly, renal failure, hypercalcaemia, and acute infection.
    • Acute leukaemia — pallor, petechiae, bruising, and fever.
    • Lymphoma — palpable non-tender lymphadenopathy. Less commonly, fever, night sweats, and weight loss.
    • Lung carcinoma — apical (Pancoast) tumour may cause progressive, constant pain in the shoulder, upper chest or interscapular region. There may also be weakness of hand muscles, Horner's syndrome, and hoarseness.
  • Drug-related adverse effects, including:
    • Myositis or myalgia due to statins — increased CK.
    • PMR-like syndrome due to quinidine.
  • Other conditions including:
    • Osteomalacia — bone pain and tenderness, skeletal deformity, and proximal muscle weakness. Frequently results from lack of vitamin D. For more information, see the CKS topic on Vitamin D deficiency in adults - treatment and prevention.
    • Fibromyalgia — tender spots; there is usually a longstanding history. 
    • Chronic fatigue syndrome — mental and physical fatigue exacerbated by activity. May be associated with muscle and joint pain; depression and anxiety are common.

Scenario: Management of polymyalgia rheumatica

From age 40 years onwards.

Search strategy

Scope of search

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

Search dates

July 2013 - January 2019

Key search terms

The terms listed below are the core search terms that were used for EBSCOhost MEDLINE (searched 3rd January 2019). These terms were combined with search filters for systematic reviews and guidelines in EBSCOhost MEDLINE. The strategy was adapted for The Cochrane Library databases. 

S3    S1 OR S2 
S2    AB polymyalgia rheumatica OR TI polymyalgia rheumatica 
S1    (MH "Polymyalgia Rheumatica") 

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
• World Health Organization
• Guidelines International Network
• TRIP database
• Agency for Healthcare Research and Quality
• 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
• Alberta Medical Association
• Michigan Quality Improvement Consortium
• Singapore Ministry of Health
• National Resource for Infection Control
• RefHELP NHS Lothian Referral Guidelines
• Medline (with guideline filter)
• Driver and Vehicle Licensing Agency
• NHS Health at Work (occupational health practice)

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

• NIHR Health Technology Assessment programme
• 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

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

• Bandolier
• Drug and Therapeutics Bulletin
• TRIP database
• Central Services Agency COMPASS Therapeutic Notes

Sources of national policy

• Department of Health
• Health Management Information Consortium (HMIC)

Patient experiences

• Healthtalk
• BMJ - Patient Journeys

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

Stakeholder engagement

Our policy

The external review process is an essential part of CKS topic development. Consultation with a wide range of stakeholders provides quality assurance of the topic in terms of:

• Clinical accuracy.
• Consistency with other providers of clinical knowledge for primary care.
• Accuracy of implementation of national guidance (in particular NICE guidelines).
• Usability.

Principles of the consultation process

• The process is inclusive and any individual may participate.
• To participate, an individual must declare whether they have any competing interests or not. If they do not declare whether or not they have competing interests, their comments will not be considered.
• Comments received after the deadline will be considered, but they may not be acted upon before the clinical topic is issued onto the website.
• Comments are accepted in any format that is convenient to the reviewer, although an electronic format is encouraged.
• External reviewers are not paid for commenting on the draft topics.
• Discussion with an individual or an organization about the CKS response to their comments is only undertaken in exceptional circumstances (at the discretion of the Clinical Editor or Editorial Steering Group).
• All reviewers are thanked and offered a letter acknowledging their contribution for the purposes of appraisal/revalidation.
• All reviewers are invited to be acknowledged on the website. All reviewers are given the opportunity to feedback about the external review process, enabling improvements to be made where appropriate.

Stakeholders

• Key stakeholders identified by the CKS team are invited to comment on draft CKS topics. Individuals and organizations can also register an interest to feedback on a specific topic, or topics in a particular clinical area, through the Getting involved section of the Clarity Informatics website.
• Stakeholders identified from the following groups are invited to review draft topics:
  • Experts in the topic area.
  • Professional organizations and societies (for example, Royal Colleges).
  • Patient organizations, Clarity has established close links with groups such as Age UK and the Alzheimer’s Society specifically for their input into new topic development, review of current topic content and advice on relevant areas of expert knowledge.
  • Guideline development groups where the topic is an implementation of a guideline.
  • The British National Formulary team.
  • The editorial team that develop MeReC Publications.
• Reviewers are provided with clear instructions about what to review, what comments are particularly helpful, how to submit comments, and declaring interests.

Patient engagement

Clarity Informatics has enlisted the support and involvement of patients and lay persons at all stages in the process of creating the content which include:

• Topic selection
• Scoping of topic
• Selection of clinical scenarios
• First draft internal review
• Second draft internal review
• External review
• Final draft and pre-publication

Our lay and patient involvement includes membership on the editorial steering group, contacting expert patient groups, organizations and individuals.

Evidence exclusion criteria

Our policy

Scoping a literature search, and reviewing the evidence for CKS is a methodical and systematic process that is carried out by the lead clinical author for each topic. Relevant evidence is gathered in order that the clinical author can make fully informed decisions and recommendations. It is important to note that some evidence may be excluded for a variety of reasons. These reasons may be applied across all CKS topics or may be specific to a given topic.

Studies identified during literature searches are reviewed to identify the most appropriate information to author a CKS topic, ensuring any recommendations are based on the best evidence. We use the principles of the GRADE and PICOT approaches to assess the quality of published research. We use the principles of AGREE II to assess the quality of published guidelines.

Standard exclusions for scoping literature:

• Animal studies
• Original research is not written in English

Possible exclusions for reviewed literature:

• Sample size too small or study underpowered
• Bias evident or promotional literature
• Population not relevant
• Intervention/treatment not relevant
• Outcomes not relevant
• Outcomes have no clear evidence of clinical effectiveness
• Setting not relevant
• Not relevant to UK
• Incorrect study type
• Review article
• Duplicate reference

Organizational, behavioural and financial barriers

Our policy

The CKS literature searches take into consideration the following concepts, which are discussed at the initial scoping of the topic.

• Feasibility
  • Studies are selected depending on whether the intervention under investigation is available in the NHS and can be practically and safely undertaken in primary care.
• Organizational and Financial Impact Analysis
• Studies are selected and evaluated on whether the intervention under investigations may have an impact on local clinical service provision or national impact on cost for the NHS. The principles of clinical budget impact analysis are adhered to, evaluated and recorded by the author. The following factors are considered when making this assessment and analysis.
  • Eligible population
  • Current interventions
  • Likely uptake of new intervention or recommendation
  • Cost of the current or new intervention mix
  • Impact on other costs
  • Condition-related costs
  • In-direct costs and service impacts
  • Time dependencies
• Cost-effectiveness or cost-benefit analysis studies are identified where available. 

We also evaluate and include evidence from NICE accredited sources which provide economic evaluations of recommendations, such as NICE guidelines. When a recommended action may not be possible because of resource constraints, this is explicitly indicated to healthcare professionals by the wording of the CKS recommendation.

Declarations of interest

Our policy

Clarity Informatics requests that all those involved in the writing and reviewing of topics, and those involved in the external review process to declare any competing interests. Signed copies are securely held by Clarity Informatics and are available on request with the permission of the individual. A copy of the declaration of interest form which participants are asked to complete annually is also available on request. A brief outline of the declarations of interest policy is described here and full details of the policy is available on the Clarity Informatics website. Declarations of interests of the authors are not routinely published, however competing interests of all those involved in the topic update or development are listed below. Competing interests include:

• Personal financial interests
• Personal family interest
• Personal non-financial interest
• Non-personal financial gain or benefit

Although particular attention is given to interests that could result in financial gains or losses for the individual, competing interests may also arise from academic competition or for political, personal, religious, and reputational reasons. An individual is not obliged to seek out knowledge of work done for, or on behalf of, the healthcare industry within the departments for which they are responsible if they would not normally expect to be informed.

Who should declare competing interests?

Any individual (or organization) involved in developing, reviewing, or commenting on clinical content, particularly the recommendations should declare competing interests. This includes the authoring team members, expert advisers, external reviewers of draft topics, individuals providing feedback on published topics, and Editorial Steering Group members. Declarations of interest are completed annually for authoring team and editorial steering group members, and are completed at the start of the topic update and development process for external stakeholders.

Competing interests declared for this topic:

None.