Am Fam Physician. 2022;106(4):420-426
Patient information: See related handout on polymyalgia rheumatica and giant cell arteritis.
Author disclosure: No relevant financial relationships.
Polymyalgia rheumatica and giant cell arteritis are inflammatory conditions that occur predominantly in people 50 years and older, with peak incidence at 70 to 75 years of age. Polymyalgia rheumatica is more common and typically presents with constitutional symptoms, proximal muscle pain, and elevated inflammatory markers. Diagnosis of polymyalgia rheumatica is clinical, consisting of at least two weeks of proximal muscle pain, constitutional symptoms, and elevated erythrocyte sedimentation rate or C-reactive protein. Treatment of polymyalgia rheumatica includes moderate-dose glucocorticoids with a prolonged taper. Giant cell arteritis, also known as temporal arteritis, usually presents with new-onset headache, visual disturbances or changes, constitutional symptoms, scalp tenderness, and temporal artery symptoms. Inflammatory markers are markedly elevated. Temporal arterial biopsy should be used for diagnosis. However, color duplex ultrasonography, magnetic resonance imaging, and fluorodeoxyglucose positron emission tomography may be helpful when biopsy is negative or unavailable. All patients with suspected giant cell arteritis should receive empiric high-dose glucocorticoids because the condition may lead to blindness if untreated. Tocilizumab is approved by the U.S. Food and Drug Administration for giant cell arteritis and should be considered in addition to glucocorticoids for initial therapy. Polymyalgia rheumatica and giant cell arteritis respond quickly to appropriate dosing of glucocorticoids but typically require prolonged treatment and have high rates of relapse; therefore, monitoring for glucocorticoid-related adverse effects and symptoms of relapse is necessary. Methotrexate may be considered as an adjunct to glucocorticoids in patients with polymyalgia rheumatica or giant cell arteritis who are at high risk of relapse.
Polymyalgia rheumatica (PMR) and giant cell arteritis (GCA) are related inflammatory conditions, which typically affect people 50 years and older. This article provides a summary and review of the best available patient-oriented evidence for each condition.
Epidemiology
Incidences of PMR and GCA vary among people 50 years and older depending on geographic location. The incidence per 100,000 people 50 years and older ranges from 2 (Korea) to 113 (Norway) for PMR and 1 (Turkey) to 44 (Iceland) for GCA.1
Approximately 20% of patients with PMR have GCA, and about 50% of patients with GCA have PMR.2,3
Risk factors for PMR and GCA include female sex, Northern European ancestry, and age.4–6
The age of onset for both conditions is typically 50 years and older, with peak incidence at 70 to 75 years of age.2,3
Polymyalgia Rheumatica
DIAGNOSIS
| Patient demographics |
| Age 50 years and older |
| Symptoms |
| Acute onset of bilateral pain in the shoulder or pelvic girdle |
| Associated morning stiffness lasting longer than 45 minutes |
| Constitutional symptoms such as fever, fatigue, night sweats, or weight loss (present in up to 50% of patients) |
| Physical examination |
| Decreased active and passive range of motion in the affected joints |
| Laboratory findings |
| Evidence of acute phase reaction (e.g., elevated inflammatory markers) |
| Ultrasound findings |
| Subdeltoid bursitis, biceps tenosynovitis, or glenohumeral synovitis |
| Duration of symptoms |
| Rapid onset of symptoms lasting longer than two weeks |
| Response to treatment |
| Rapid response to appropriate glucocorticoid therapy (typically within two to four weeks) |
| Polymyalgia rheumatica | Giant cell arteritis |
|---|---|
| Musculoskeletal | |
| Adhesive capsulitis Calcium pyrophosphate deposition disease Gout Osteoarthritis Rotator cuff pathology Spondyloarthropathy Subacromial bursitis | Dental pain/conditions Temporomandibular disorders |
| Autoimmune | |
| Dermatomyositis Polymyositis Rheumatoid arthritis Systemic lupus erythematosus Vasculitis | Optic neuritis Polyarteritis nodosa Small/medium vessel vasculitis |
| Neurologic | |
| Parkinson disease | Cerebrovascular accident/retinal artery occlusion Migraine Trigeminal neuralgia |
| Infectious | |
| Endocarditis Hepatitis B or C HIV Parvovirus B19 Tuberculosis | Acute otitis media/externa Endocarditis Herpes zoster Meningitis/encephalitis Sinusitis |
| Malignant | |
| Lung cancer paraneoplastic syndrome Lymphoma Multiple myeloma Prostate cancer | Central mass effect Lymphoma Multiple myeloma |
| Endocrine | |
| Hyperparathyroidism Thyroid disorders | Hypothyroidism |
| Psychiatric | |
| Depression Fibromyalgia | Depression |
| Iatrogenic | |
| Statin-induced myopathy | Drug-induced vasculitis |
History and Physical Examination
The patient history reveals predominantly symmetrical shoulder and thigh stiffness, which may cause difficulties in rising from a seated position and raising arms overhead.3
Up to 50% of patients report constitutional symptoms such as fever, fatigue, night sweats, or weight loss.3
Physical examination may show restricted active range of motion and weakness or tenderness in the hip and shoulder muscles.
Laboratory Studies
An erythrocyte sedimentation rate (ESR) of more than 30 to 40 mm per hour or elevated C-reactive protein (CRP) levels are present in approximately 90% of patients with PMR.13,14
Complete blood count often shows associated normochromic anemia and thrombocytosis.4 Liver function tests may show mildly elevated alkaline phosphatase.15
Muscle enzyme levels, including creatinine kinase, are normal in patients with PMR.16
Positive antinuclear antibodies, antineutrophil cytoplasmic antibodies, rheumatoid factor, and anti-citrullinated peptide antibodies are not found in patients with PMR and indicate an alternative diagnosis if present.7
Imaging
Ultrasonography of the shoulder shows subdeltoid bursitis in up to 79% of patients with PMR and may be considered as an adjunct diagnostic study.17,18
If clinically indicated, chest radiography may be considered because lung cancer paraneoplastic syndrome can mimic PMR.14 An exhaustive evaluation for underlying occult malignancy or infection is not recommended.4
Plain radiography of the involved joints may show concomitant degenerative joint disease but does not add diagnostic value.7
TREATMENT
Subscribe
From $145- Immediate, unlimited access to all AFP content
- More than 130 CME credits/year
- AAFP app access
- Print delivery available
Issue Access
$59.95- Immediate, unlimited access to this issue's content
- CME credits
- AAFP app access
- Print delivery available
Article Only
$25.95- Immediate, unlimited access to just this article
- CME credits
- AAFP app access
- Print delivery available
