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Basic Information ⬇

AUTHORS: Luca Katz, BA and Manuel F. DaSilva, MD

Definition

Polymyalgia rheumatica (PMR) is an inflammatory condition characterized by muscle pain and stiffness of the neck, shoulder, and pelvic girdle. PMR primarily affects the elderly and can occur alone or in conjunction with giant cell arteritis (GCA).1

Synonyms

PMR

Anarthritic rheumatoid syndrome

ICD-10CM CODES
M31.5Polymyalgia rheumatica with giant cell arteritis
M35.3Polymyalgia rheumatica
Epidemiology & Demographics

  • PMR almost exclusively occurs above age 50 with peak incidence between ages 70 and 79 with a female:male ratio 2:1.1
  • In the U.S., incidence is 52.5 cases per 100,000, increasing with advancing age, with a prevalence of 0.5% to 0.7%. A recent literature review showed the global epidemiology of PMR is more limited with a greater variation in prevalence and estimates. Of note, GCA is higher in populations of Northern European ancestry.1
Physical Findings & Clinical Presentation

  • Patients with PMR often have symptoms for 1 to 3 mo before a diagnosis is made.
  • Onset of symmetric muscle pain and stiffness, which is worse in the morning (similar to other inflammatory disorders) and recurs with periods of inactivity.2
  • Neck, shoulders, lower back, hips, thighs, and occasionally trunk and arms are involved. Shoulders are usually affected first. Pain distribution in PMR is illustrated in Fig. E1.

Figure E1 Typical distribution of pain in patients with polymyalgia rheumatica (PMR).

Shaded areas demonstrate typical sites of pain in individuals with PMR, including (A) shoulder girdle and neck pain distribution and (B) pelvic girdle pain distribution.

From Hochberg MC: Rheumatology, ed 7, Philadelphia, 2019, Elsevier.

  • Constitutional symptoms of fatigue, malaise, weight loss, loss of appetite, and low-grade fever may accompany pain and stiffness.
  • Physical exam may reveal limited range of motion of shoulder (most common), cervical spine, and hips. May have subdeltoid and subacromial bursitis and peripheral joint synovitis. Motor exam is normal, although can be limited by pain.
  • High-spiking fevers, night sweats, visual disturbances, headaches, or jaw claudication should raise suspicion of giant cell arteritis and be further evaluated promptly.3
Etiology

The cause is unknown, but both PMR and GCA are associated with HLA-DRB1 haplotype. With both conditions, also see elevated Th17 cells and decreased regulatory T-cells.2

Diagnosis ⬆ ⬇

Differential Diagnosis

See Box 1.

BOX 1 Differential Diagnosis of Polymyalgia Rheumatica

  • Rheumatoid arthritis
  • Rotator cuff syndrome
  • Osteoarthritis of shoulder and hip joints
  • Fibromyalgia
  • Polymyositis/dermatomyositis
  • Spondyloarthritis
  • Systemic lupus erythematosus
  • Vasculitides
  • Paraneoplastic myalgias
  • Infection-associated myalgias
  • RS3PE (remitting seronegative symmetric synovitis and pitting edema)
  • Parkinson disease
  • Hypothyroidism
  • There has been a report of a case of polymyalgia rheumatica being an adverse event after administration of a COVID-19 vaccine, but this is uncommon6
Workup

  • Initial laboratory evaluation: ESR, CRP, CBC, CPK.3
  • ESR >30 mm/h in majority of patients. CRP elevation may be more common than high ESR.
  • CBC may show a normocytic anemia and thrombocytosis.
  • CPK is normal. Antibodies (ANA, RF, CCP) are typically negative.
  • An algorithm for diagnosing polymyalgia rheumatica without giant cell arteritis (GCA) is described in Fig. E2, and Tables 1 and 2 describe various classification criteria for PMR.

TABLE 1 2012 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Polymyalgia Rheumatica∗

CriteriaPoints With UltrasonographyPoints Without Ultrasonography
  1. Morning stiffness duration >45 min
22
  1. Hip pain or limited range of movement
11
  1. Absence of rheumatoid factor or anticitrullinated protein antibody
22
  1. Absence of other joint involvement
11
  1. ≥1 shoulder with subdeltoid bursitis and/or biceps tenosynovitis and/or glenohumeral synovitis (either posterior or axillary) and ≥1 hip with synovitis and/or trochanteric bursitis
1Not applicable
  1. Both shoulders with subdeltoid bursitis, biceps tenosynovitis, or glenohumeral synovitis
1Not applicable

∗Score ≥4 without ultrasonography or ≥5 with ultrasonography is categorized as polymyalgia rheumatica.

TABLE 2 Classification Criteria for Polymyalgia Rheumatica

Chuang Criteria
  1. Patients aged 50 yr or older
  2. Bilateral aching and stiffness persisting for 1 mo or more involving two of the following areas: Neck or torso, shoulders or proximal regions of the arms, and hips or proximal aspects of the thighs
  3. ESR >40 mm/h
  4. Exclusion of other diagnoses except GCA

The presence of all these criteria defines diagnosis of PMR.
Healey Criteria (22)
  1. Persistent pain (for at least 1 mo) involving two of the following areas: Neck, shoulders, and pelvic girdle
  2. Morning stiffness lasting >1 h
  3. Rapid response to prednisone (≤20 mg/day)
  4. Absence of other diseases capable of causing the musculoskeletal symptoms
  5. Age >50 yr
  6. ESR >40 mm/h
The diagnosis of PMR is made if all the above criteria are satisfied.
Bird Criteria (23)∗
  1. Bilateral shoulder pain and/or stiffness
  2. Onset of illness within 2 wk
  3. Initial ESR >40 mm/h
  4. Morning stiffness >1 h
  5. Age >65 yr
  6. Depression or loss of weight
  7. Bilateral upper arm tenderness

ESR, Erythrocyte sedimentation rate; GCA, giant cell arteritis.

∗A diagnosis of probable polymyalgia rheumatica (PMR) is made if any three or more of these criteria are fulfilled. The presence of any three or more criteria yields a sensitivity of 92% and a specificity of 80%.

From Hochberg MC: Rheumatology, ed 7, Philadelphia, 2019, Elsevier.

Figure E2 Algorithm for diagnosing polymyalgia rheumatica without giant cell arteritis.

!!flowchart!!

From Firestein GS et al: Firestein & Kelley’s textbook of rheumatology, ed 11, Philadelphia, 2021, Elsevier.

  • Ultrasonography, MRI, and PET may identify bursitis or tenosynovitis, (features of the 2012 PMR classification of criteria), which increase sensitivity and specificity.4

Treatment ⬆ ⬇

Pearls & Considerations ⬆ ⬇

Patients with PMR should be monitored closely for the development of GCA. Patients who have incomplete response to prednisone or have an evolving pattern of pain and swelling should be reevaluated for the possibility of a different diagnosis such as rheumatoid arthritis.

Related Content

Polymyalgia Rheumatica (PMR) (Patient Information)

Giant Cell Arteritis (Related Key Topic)

Vasculitis, Systemic (Related Key Topic)

Related Content ⬆

    1. Sharma A. : Incidence and prevalence of giant cell arteritis and polymyalgia rheumatica: a systematic literature reviewSemin Arthritis Rheum. ;50(5):1040-1048, 2020.
    2. Carmona F.D. : A large-scale genetic analysis reveals a strong contribution of the HLA class II region to giant cell arteritis susceptibilityAm J Hum Genet. ;96(4):565-580, 2015.
    3. Caylor T., Perkins A. : Recognition and management of polymyalgia rheumatica and giant cell arteritisAm Fam Physician. ;88(10):676-684, 2013.
    4. Dasgupta B. : 2012 provisional classification criteria for polymyalgia rheumatica: a European League Against Rheumatism/American College of Rheumatology collaborative initiativeAnn Rheum Dis. ;71(4):484-492, 2012.
    5. Akiyama M. : Tocilizumab in isolated polymyalgia rheumatica: a systematic literature reviewSemin Arthritis Rheum. ;50(3):521-525, 2020.
    6. Devauchelle-Pensec V : Effect of tocilizumab on disease activity in patients with active polymyalgia rheumatica receiving glucocorticoid therapy: a randomized clinical trialJAMA. ;328(11):1053-1062, 2022.
    7. Manzo C. : Polymyalgia rheumatica as uncommon adverse event following immunization with COVID-19 vaccine: a case report and review of literatureAging Med. ;4(3):234-238, 2021.