Polymyalgia Rheumatica and Giant Cell Arteritis: Rapid Evidence Review

Meghan F. Raleigh; Jonathan Stoddard; Hillary J. Darrow

MEGHAN F. RALEIGH, MD, JONATHAN STODDARD, MD, AND HILLARY J. DARROW, MD

Am Fam Physician. 2022;106(4):420-426

Patient information: See related handout on polymyalgia rheumatica and giant cell arteritis.

This clinical content conforms to AAFP criteria for CME.

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.

Clinical recommendation	Evidence rating	Comments
Patients with PMR should be treated with the minimum effective dose of oral glucocorticoids with individualized duration.^14,19,22	C	Expert opinion
Early addition of methotrexate to glucocorticoid therapy should be considered in patients with PMR who are at high risk of relapse or glucocorticoid-related adverse events.^7,8,19	A	Consistent results from multiple randomized controlled trials
Intramuscular methylprednisolone every three weeks should be considered as an alternative to oral glucocorticoids in patients at high risk of adverse effects from glucocorticoids.^8,19	B	A single small, high-quality randomized controlled trial
Dose-tapering schedules for patients with PMR should be individualized based on monitoring of disease activity, laboratory markers, and adverse events.¹⁹	C	Expert opinion in the absence of clinical trials
In patients with newly diagnosed GCA, noninvasive vascular imaging (i.e., magnetic resonance imaging or computed tomography angiography) of the neck, chest, abdomen, and pelvis should be performed to evaluate for large vessel involvement.²⁰	C	Lower-quality evidence and consensus guidelines
To improve chances of successful remission, patients with newly diagnosed GCA should be treated with high-dose oral glucocorticoids and subcutaneous interleukin-6 antagonist tocilizumab (Actemra) over glucocorticoids alone.^8,20,38,39	A	Two high-quality randomized controlled trials with consistent results and consensus guidelines
Long-term monitoring is recommended for patients with GCA who are in clinical remission.²⁰	C	Evidence showing that monitoring is low risk and there is potential for harm from not monitoring; consensus guidelines

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

With no definitive diagnostic test for PMR, the diagnosis is clinical. The typical clinical presentation is shown in Table 1.^3,7–10
The differential diagnosis of PMR is summarized in Table 2.^7,9,11,12

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

Table 3 includes consensus treatment recommendations for PMR.^7,8,19–21

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Epidemiology

Polymyalgia Rheumatica

DIAGNOSIS

History and Physical Examination

Laboratory Studies

Imaging

TREATMENT

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Epidemiology

Polymyalgia Rheumatica

DIAGNOSIS

History and Physical Examination

Laboratory Studies

Imaging

TREATMENT

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