creation date: 2025-12-21 23:14
tags: Pathologies
Polymyalgia Rheumatica
Background
Definitions
Polymyalgia rheumatica (PMR) is a rheumatic disorder characterized by neck, shoulder, and hip pain and is associated with white adults over age 50.
PMR is the second most common rheumatic disease after rheumatoid arthritis.
Etiology and Pathophysiology
The etiology of PMR is not well understood. There seems to be associations with:
- Genetic predisposition (HLA class II alleles)
- Concurrence with giant cell arteritis
- Epstein-Barr virus
- Diverticulitis
- Vaccine adjuvants
PMR is an immune-mediated disorder and is associated with:
- Elevated inflammatory markers (ESR/CRP)
- Decreased number of circulatory B cells
- Decreased regulatory T cells and T helper cells
- Increased Th17 cells
Clinical Presentation
Signs & Symptoms
PMR is characterized by symmetrical aching (in order of % patients) of the:
- Shoulders
- Neck
- Hip girdle
- Torso
The pain is described as:
- Abrupt onset “overnight one day”
- Worse in the morning and improves with activity
- Functionally limiting
Additionally, non-specific systemic symptoms/signs may be present which includes:
- Malaise
- Fatigue
- Depression
- Anorexia
- Weight loss
- Low-grade fever
It should be noted that fever is rare without comorbid GCA so presence of such should prompt GCA workup.
History & Physical Exam
History should confirm stiffness and aching is not longstanding as that is not suggestive of PMR.
A musculoskeletal exam should be done, including determining the range of motion of the joints.
History and physical should focus on ruling out alternative diagnoses (especially GCA), assessing comorbidities which may impact treatment (eg. diabetes, hypertension, osteoporosis).
Risk factors
- Age >50
- White
Diagnosis
Criteria
There is no established diagnostic criteria and PMR is a clinical diagnosis. Generally, a diagnosis can be confidently made if all of the following are met:
- Age 50 years or older at onset
- Bilateral shoulder and/or pelvic girdle aching lasting longer than 45 minutes persisting for ≥2 weeks
- Stiffness involves ≥2 of the following three areas: neck or torso, shoulders or proximal regions of arm, hip or proximal aspects of thighs
- Elevated acute phase reactants (eg. ESR and/or CRP)
- Rapid resolution of symptoms with low-dose glucocorticoids
Work-up
Laboratory studies
- CBC - normocytic anemia may be present but nonspecific
- ESR/CRP - elevated
- Autoantibodies - negative with PMR (consider RA if positive)
Additional workup may be indicated if a particular alternative diagnosis is suspected.
GCA workup
Owing to the high rates of co-occurrence, assess for GCA at initial diagnosis and routinely screen at subsequent visits.
Differential
Several disorders have similar manifestations as PMR.
Inflammatory conditions
- Rheumatoid arthritis (late-onset) - common re-diagnosis after 1 year follow-up
- Spondyloarthropathy
- RS3PE syndrome (Remitting Seronegative Symmetrical Synovitis with Pitting Edema)
- Inflammatory myopathy
- Non-GCA vasculitis
- Crowned dens syndrome
- Infection (fever)
Noninflammatory conditions
- Fibromyalgia (typically younger patient)
- Multifocal local musculoskeletal disease
- Bone disease (eg. hyperparathyroidism, multiple myeloma)
- Malignancy
- Hypothyroidism
- Parkinson disease
- Depression
- Drug-induced myalgias or myositis
Red Flags / Complications
Prognosis is not significantly different from general population.
PMR is associated with increased risk of cardiovascular diseases and inflammatory arthritis.
Management
Initial Treatment
The goal of management is the relief of symptom. The primary treatment modality is low-dose systemic glucocorticoids. There is no evidence suggesting prednisone or prednisolone is superior over one another.
Initial dose
Prednisone 15 mg/day PO is recommended first dose
- Lower dose of 7.5-10 mg/day may be sufficient for mild symptoms
- Higher dose of 20 mg/day may be needed for severe pain and constitutional symptoms
Generally, this dose is used for 2-4 weeks following the resolution of symptoms.
Monitoring response
Follow-up should be made after 1-2 weeks of therapy to assess for prompt symptomatic response and if adjustment of dosing is required.
A formal visit should be made at 4-8 weeks to confirm resolution of symptoms and taper planning. Persistent symptoms at this point should indicate workup for alternative diagnoses.
Tapering
Dose is reduced as tolerated to the minimum amount needed to maintain suppression of symptoms.
- Over 10 mg/day, reduce by 2.5 mg/day decrements q2-4 weeks
- Under 10 mg/day, reduce by 1 mg/day decrements q4 weeks
Management of Relapse and Recurrent Disease
Relapses are common during tapering, especially in the first 1-2 years of treatment and when prednisone doses are under 10 mg/day.
If relapse occurs during tapering, the glucocorticoid dose is increased to the lowest dose that achieves symptomatic improvement.
If recurrence occurs following discontinuation of glucocorticoids, prednisone should be resumed at the original dose that managed symptoms.
In cases of comorbidities, development of glucocorticoid-related side effects, or relapsing disease, glucocorticoid-sparing agents may be considered. This is not recommended routinely.