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Polymyalgia rheumatica

Background

Overview

Definition
PMR is a chronic, systemic inflammatory disorder associated with synovitis, tenosynovitis, and bursitis of proximal large joints.
1
Pathophysiology
The exact cause of PMR remains unknown. Genetic polymorphisms in intercellular adhesion molecules and interleukins have been associated with an increased risk. Epidemiological studies have suggested that infectious triggers (Mycoplasma pneumoniae, parvovirus B19, Chlamydia pneumoniae) may have a role in disease pathogenesis.
2
Epidemiology
The incidence of PMR in the US is 63.9 cases per 100,000 person-years.
3
Disease course
PMR is associated with predominant inflammation of periarticular structures (bursitis, tenosynovitis), although synovitis has also been reported. Hallmark clinical features include pain and stiffness involving the neck or torso, shoulders or proximal regions of the arms, and hips or proximal aspects of the thighs. Although myalgias are prominent symptoms in patients with PMR, muscle inflammation is not typically seen.
2
Prognosis and risk of recurrence
Symptoms of PMR typically respond rapidly to low-dose prednisone or prednisolone (≤ 20 mg). Patients with PMR have reduced QoL at time of diagnosis, which improves with treatment. Overall survival is similar to that of the general population.
2

Guidelines

Key sources

The following summarized guidelines for the evaluation and management of polymyalgia rheumatica are prepared by our editorial team based on guidelines from the American College of Rheumatology (ACR 2023), the European League Against Rheumatism (EULAR 2023), the Italian Society of Rheumatology (ISR 2020), and the European League Against Rheumatism (EULAR/ACR 2015). ...
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Screening and diagnosis

Differential diagnosis: as per ISR 2020 guidelines, adopt a safe and specific approach to exclude relevant mimicking or associated conditions, such as non-inflammatory, inflammatory (giant cell arteritis or rheumatoid arthritis), drug-induced, endocrine, infectious and neoplastic, to ascertain the PMR case definition in the presence of sufficient clinical findings.
B
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Classification and risk stratification

Risk factors: as per ISR 2020 guidelines, insufficient evidence to define the role of risk factors for relapse/prolonged therapy in patients with PMR.
I

Diagnostic investigations

Clinical evaluation: as per ACR/EULAR 2015 guidelines, assess for and exclude relevant mimicking conditions (non-inflammatory, inflammatory, drug-induced, endocrine, infective, and neoplastic) as part of the initial evaluation of patients with PMR.

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  • Laboratory tests

  • Diagnostic imaging

  • Evaluation for comorbidities

  • Screening for chronic infections

Medical management

General principles
As per ISR 2020 guidelines:
Treat patients with PMR aiming to provide the best care based on a shared decision by the patient and treating physician.
B
Provide an individualized management plan in patients with PMR.
B

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  • Corticosteroids (indications)

  • Corticosteroids (dosing and duration)

  • Corticosteroids (tapering)

  • Methotrexate

  • NSAIDs

  • Biological agents

Nonpharmacologic interventions

Exercise programs: as per ISR 2020 guidelines, consider offering individualized exercise programs in patients with PMR aiming to maintain muscular mass and function and reduce risk of falls, especially in older patients on long-term corticosteroids as well as in frail patients.
C

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  • Herbal products

Patient education

Patient education: as per ISR 2020 guidelines, provide patients with PMR access to education focusing on the impact and treatment of the disease (including disease and disease predictors) and advice on individually-tailored exercise programs.
B

Preventative measures

Routine immunizations: as per ACR 2023 guidelines, consider offering high-dose or adjuvanted influenza vaccination, rather than regular-dose influenza vaccination, in ≥ 65 years old patients with rheumatic or musculoskeletal diseases and in 18-65 years old patients with rheumatic or musculoskeletal diseases on immunosuppressive medications.
C
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  • Prophylaxis for P. jirovecii pneumonia

Follow-up and surveillance

Indications for referral: as per ISR 2020 guidelines, consider referring patients with PMR for specialist consultation, particularly in the following situations:
atypical presentation such as peripheral inflammatory arthritis, systemic symptoms, low inflammatory markers, age of < 60 years
experience of or high risk of therapy-related side effects
refractory to corticosteroid therapy
relapses/prolonged therapy.
C

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  • Follow-up