> Table of Contents > Polymyalgia Rheumatica
Polymyalgia Rheumatica
Ronald G. Chambers Jr., MD, FAAFP
Megan Babb, DO
image BASICS
DESCRIPTION
  • A clinical syndrome characterized by pain and stiffness (a term often used interchangeably by patients) of the shoulder, hip girdles, and neck (1).
  • Primarily impacts the elderly, associated with morning stiffness and elevated markers of inflammation
  • System(s) affected: musculoskeletal, hematologic/lymphatic/immunologic
  • Synonym(s): senile rheumatic disease; polymyalgia rheumatica syndrome; pseudo-polyarthrite rhizomélique
Geriatric Considerations
  • Incidence increases with age
  • Average age of onset ˜70 years
Pediatric Considerations
Rare in patients <50 years of age; peak incidence between 70 and 80 years of age (2)
EPIDEMIOLOGY
Incidence
  • Incidence increases after 50. Incidence of PMR is 50/100,000 and incidence of giant cell arteritis (GCA) is 18/100,000 people in the United States.
  • Predominant sex: female > male (2 to 3:1) (3)
  • Most common in Caucasians, especially those of northern European ancestry
Prevalence
Prevalence in population >50 years old: 700/100,000
ETIOLOGY AND PATHOPHYSIOLOGY
  • Unknown. Symptoms appear to be related to enhanced immune system activity and periarticular inflammatory activity.
  • Pathogenesis
    • Polygenic; multiple environmental and genetic factors contribute
    • Histologic evidence of GCA and parvovirus B19 DNA in temporal artery specimen
Genetics
Associated with human leukocyte antigen determinants (HLA-DRB1*04 and DRB1*01 alleles) (4)
RISK FACTORS
  • Age >50 years
  • Presence of GCA
COMMONLY ASSOCIATED CONDITIONS
GCA (temporal arteritis) may occur in 15-30% of patients; more common in females than males with PMR.
image DIAGNOSIS
PHYSICAL EXAM
  • Decreased range of motion (ROM) of shoulders, neck, and hips
  • Muscle strength is usually normal, although it may be limited by pain and/or stiffness.
  • Muscle tenderness
  • Disuse atrophy
  • Synovitis of the small joints and tenosynovitis
  • Coexisting carpal tunnel syndrome
DIFFERENTIAL DIAGNOSIS
  • Rheumatoid arthritis
  • Palindromic rheumatism
  • Late-onset seronegative spondyloarthropathies (e.g., psoriatic arthritis, ankylosing spondylitis)
  • Systemic lupus erythematosus; Sjögren syndrome; fibromyalgia
  • Polymyositis/dermatomyositis (check creatine phosphokinase, aldolase)
  • Thyroid disease
  • Hyperparathyroidism, hypoparathyroidism
  • Hypovitaminosis D
  • Viral myalgia
  • Osteoarthritis
  • Rotator cuff syndrome; adhesive capsulitis
  • Remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome
  • Occult infection or malignancy (e.g., lymphoma, leukemia, myeloma, solid tumor)
  • Myopathy (e.g., steroid, alcohol, electrolyte depletion)
  • Depression
DIAGNOSTIC TESTS & INTERPRETATION
Consider PMR in patients >50 years of age with proximal muscle pain and stiffness.
  • Temporal artery biopsy if symptoms of GCA are present
  • ESR (Westergren) elevation >40 mm/hr
    • ESR is typically elevated, sometimes >100 mm/hr
    • ESR normal (<40 mm/hr) in 7-22% of patients
  • Elevated C-reactive protein
  • Normochromic/normocytic anemia
  • Anticyclic citrullinated peptide (anti-CCP) antibodies usually negative (in contrast to elderly-onset rheumatoid arthritis [RA])
  • Rheumatoid factor: usually negative (5-10% of patients >60 years of age will have positive rheumatoid factor without RA)
  • Mild elevations in liver function tests, especially alkaline phosphatase
  • Antibodies to ferritin peptide
  • Drugs that may alter lab results: prednisone
  • Disorders that may alter lab results: other disorders causing elevation of the sedimentation rate (e.g., infection, neoplasm, renal failure)
  • Normal EMG
  • Normal muscle histology
Initial Tests (lab, imaging)
  • ESR (usually >40 mm/hr); C-reactive protein; CBC
  • MRI is not necessary for diagnosis but may show periarticular inflammation, tenosynovitis, and bursitis.
  • US may show bursitis, tendinitis, and synovitis.
  • MRI, PET, and temporal artery US may all play a role in diagnosis of PMR.
Diagnostic Procedures/Other
A temporal artery biopsy is indicated in patients with symptoms suggestive of GCA. Treat empirically pending biopsy results.
Test Interpretation
Scoring algorithm: morning stiffness >45 minutes (2 points), hip pain/limited ROM (1 point), absence of rheumatoid factor and anti-citrullinated protein antibody (ACPA) (2 points), and absence of peripheral joint pain (1 point). A score of >4 has 68% sensitivity and 78% specificity which increase with a positive temporal artery US.
image TREATMENT
GENERAL MEASURES
  • Address risk of steroid-induced osteoporosis.
    • Obtain dual energy x-ray absorptiometry and check 25-OH vitamin D levels if necessary.
    • Consider antiresorptive therapies (bisphosphonates) based on recommendations for treatment of corticosteroid-induced osteoporosis.
  • Encourage adequate calcium (1,500 mg/day) and vitamin D (800 to 1,000 U/day) supplementation.
  • Physical therapy for ROM exercises, if needed
MEDICATION
First Line
  • Prednisone: 10 to 20 mg/day PO initially; expect a dramatic (diagnostic) response within days. 15 mg/day is an effective dose in most patients.
    • Increase to 20 mg/day if no immediate response.
    • If no response to 10 to 20 mg/day within a week, reconsider diagnosis.
  • Divided-dose steroids (BID or TID) may be helpful (especially if symptoms recur in the afternoon).
  • Consider using delayed-release prednisone at bedtime (may be more efficient in treating morning stiffness)
  • Begin slow taper by 2.5 mg decrements every 2 to 4 weeks to a dose of 7.5 to 10 mg/day. Below this dose, taper by 1 mg/month to prevent relapse.
  • Increase prednisone for recurrence of symptoms (relapse common).
  • Corticosteroid treatment often lasts at least 1 to 2 years.
  • May stop steroids at 6 to 12 months if patient is symptom-free and there is a normal ESR
  • Contraindications
    • Use steroids with caution in patients with chronic heart failure, diabetes mellitus, immunocompromised conditions, and with systemic fungal or bacterial infection.
    • Treat infections concurrently.
  • Precautions
    • Long-term steroid use (>2 years) is associated with sodium and water retention, exacerbation of chronic heart failure, hypokalemia, increased susceptibility to infection, osteoporosis, fractures, hypertension, cataracts, glaucoma, avascular necrosis, depression, and weight gain
    • Patients may develop temporal arteritis while on low-dose corticosteroid treatment for PMR. This requires an increase in dose to 40 to 60 mg.
    • Alternate-day steroids are not effective.
P.827

Second Line
  • NSAIDs usually are not adequate for pain relief.
  • Methotrexate has a modest effect in reducing relapse rate and lowering the cumulative dose of steroid therapy.
  • There is conflicting evidence for antitumor necrosis factor agents (anti-TNF) (infliximab, etanercept) regarding steroid-sparing effects.
  • Anti-interleukin (anti-IL) 6 therapy is under investigation for future use (5).
  • Corticosteroid injections may reduce pain and stiffness and allow for increased levels of activity.
image ONGOING CARE
FOLLOW-UP RECOMMENDATIONS
Patient Monitoring
  • Evaluate patients monthly initially and during medication taper; every 3 months otherwise.
  • Follow ESR as steroids are tapered; both ESR and CRP should decline as symptoms improve.
  • Follow-up for symptoms of GCA (e.g., headache, visual loss, and diplopia) and report immediately.
  • Monitor side effects of corticosteroid therapy such as osteoporosis, hypertension, and hyperglycemia.
  • Do not treat elevated ESR (do not increase the steroid dose to normalize the ESR If patient is asymptomatic).
DIET
  • Regular diet
  • Aim for adequate calcium and vitamin D.
PATIENT EDUCATION
  • Review adverse effects of corticosteroids.
  • Discuss the symptoms of GCA and instruct the patient to report them immediately should any occur.
  • Contact physician if symptoms recur during steroid taper.
  • Instruct patients to not discontinue steroids abruptly.
  • Counsel patients on calcium and vitamin D requirements.
  • Resources for patients
    • Arthritis Foundation: http://www.arthritis.org/
    • American College of Rheumatology: http://www.rheumatology.org/I-Am-A/Patient-Caregiver/Diseases-Conditions/Polymyalgia-Rheumatica
PROGNOSIS
  • Most patients require at least 2 years of corticosteroid treatment.
  • Prognosis is very good with proper treatment.
  • Relapse is common (in 25-50% of patients), particularly if steroids are tapered too quickly.
  • Higher age at diagnosis, female sex, high baseline ESR, increased plasma viscosity, increased levels of soluble IL-6 receptor, or high initial steroid dose have been associated with a prolonged disease course and greater number of disease flares.
REFERENCES
1. Mackie SL, Hughes R, Walsh M, et al. “An impediment to living life”: why and how should we measure stiffness in polymyalgia rheumatica? PLoS One. 2015;10(5):e0126758.
2. Salvarani C, Gabriel SE, O'Fallon WM, et al. Epidemiology of polymyalgia rheumatica in Olmsted County, Minnesota, 1970-1991. Arthritis Rheum. 1995;38(3):369-373.
3. Liozon E, Ouattara B, Rhaiem K, et al. Familial aggregation in giant cell arteritis and polymyalgia rheumatica: a comprehensive literature review including 4 new families. Clin Exp Rheumatol. 2009;27(1 Suppl 52):S89-S94.
4. Weyand CM, Hunder NN, Hicok KC, et al. HLA-DRB1 alleles in polymyalgia rheumatica, giant cell arteritis, and rheumatoid arthritis. Arthritis Rheum. 1994;37(4):514-520.
5. Seitz M. Polymyalgia rheumatica: what is the current status? Z Rheumatol. 2015;74(6):507-510.
Additional Reading
&NA;
  • Aikawa NE, Pereira RM, Lage L, et al. Anti-TNF therapy for polymyalgia rheumatica: report of 99 cases and review of the literature. Clin Rheumatol. 2012;31(3):575-579.
  • Buttgereit F, Gibofsky A. Delayed-release prednisone-a new approach to an old therapy. Expert Opin Pharmacother. 2013;14(8):1097-1106.
  • Camellino D, Cimmino MA. Imaging of polymyalgia rheumatica: indications on its pathogenesis, diagnosis and prognosis. Rheumatology (Oxford). 2012;51(1):77-86.
  • Dasgupta B, Borg FA, Hassan N, et al. BSR and BHPR guidelines for the management of polymyalgia rheumatica. Rheumatology (Oxford). 2010;49(1):186-190.
  • Dasgupta B, Cimmino MA, Maradit-Kremers H, et al. 2012 provisional classification criteria for polymyalgia rheumatica: a European League Against Rheumatism/American College of Rheumatology collaborative initiative. Ann Rheum Dis. 2012;71(4):484-492.
  • Hernández-Rodríguez J, Cid MC, López-Soto A, et al. Treatment of polymyalgia rheumatica: a systematic review. Arch Intern Med. 2009;169(20):1839-1850.
  • Kreiner F, Galbo H. Effect of etanercept in polymyalgia rheumatica: a randomized controlled trial. Arthritis Res Ther. 2010;12(5):R176.
  • Michet CJ, Matteson EL. Polymyalgia rheumatica. BMJ. 2008;336(7647):765-769.
  • Régent A, Ly KH, Blet A, et al. Contribution of antiferritin antibodies to diagnosis of giant cell arteritis. Ann Rheum Dis. 2013;72(7):1269-1270.
  • Spies CM, Burmester GR, Buttgereit F. Methotrexate treatment in large vessel vasculitis and polymyalgia rheumatica. Clin Exp Rheumatol. 2010;28 (5 Suppl 61):S172-S177.
See Also
&NA;
Arteritis, Temporal; Osteoarthritis; Arthritis, Rheumatoid (RA); Depression; Fibromyalgia; Polymyositis/Dermatomyositis
Codes
&NA;
ICD10
  • M35.3 Polymyalgia rheumatica
  • M31.5 Giant cell arteritis with polymyalgia rheumatica
Clinical Pearls
&NA;
  • Consider PMR in patients >50 years of age presenting with proximal limb (hip, neck, shoulder) pain and stiffness.
  • A normal ESR does not exclude PMR.
  • If there is not a dramatic and rapid response to steroids, reconsider the diagnosis.
  • Adjust steroid dosing according to patient symptoms, not the ESR.