II. Epidemiology

  1. Common over age 50 years (peaks between ages 70 to 80 years old)
    1. Incidence: 50 per 100,000
    2. Prevalence: One in 143 persons
  2. Most common in white persons of Northern European descent
    1. Rare in Black Patients
    2. Rare in Asian patients (Incidence 2 per 100,000 in Korea over age 50 years)
    3. Incidence is as high as 44 (Iceland) to 113 (Norway) per 100,000 over age 50 years
  3. Women predominate by 2:1 ratio
  4. Associated with HLA-DR4 and Cw3 haplotypes
    1. Seasonal outbreaks suggest an infectious trigger
  5. Associated with Temporal Arteritis
    1. Temporal Arteritis is occurs in 18-26% of Polymyalgia Rheumatica cases
    2. Polymyalgia Rheumatica is 2-3 times more common than Temporal Arteritis

III. Risk Factors

  1. Female gender
  2. Northern european descent
  3. Age over 50 years

IV. Symptoms

  1. Severe Muscle ache and stiffness
    1. Duration
      1. Minimum of 2 week duration for diagnosis
      2. Typically 1 month or longer at presentation
      3. Usually insidious onset
    2. Location (symmetric, bilateral involvement)
      1. Shoulders (affected in 95% of cases)
      2. Neck
      3. Pelvic girdle and hips
    3. Characteristics
      1. Ache worse at night and with movement
      2. Stiffness
    4. Timing
      1. More prominent in morning or after inactivity
      2. Morning stiffness lasts >45 minutes for diagnosis
  2. Associated systemic symptoms (30-50% of cases)
    1. Malaise
    2. Anorexia
    3. Weight loss
    4. Low grade fever
    5. Depressed mood
    6. Night Sweats

V. Signs

  1. Unremarkable physical exam
    1. Symptoms are usually well out-of-proportion to exam
    2. No true motor weakness
      1. Strength limitation in PMR should be due to pain and disuse atrophy
      2. True Muscle Weakness suggests alternative diagnosis
  2. Mild findings (variably present)
    1. Limited range of motion in affected joints
      1. Limited by proximal myalgias
      2. Difficulty raising arms overhead or rising from a seated position
    2. Shoulder or hip Bursitis
    3. Localized tenderness over Shoulders and hips
  3. Other findings which may be present (50% of cases)
    1. Asymmetric knee or wrist Arthritis
    2. Carpal Tunnel Syndrome
    3. Distal extremity edema (wrists, hands, ankles, feet)

VI. Precautions: Red Flags (suggestive of Temporal Arteritis)

  1. See Temporal Arteritis
  2. Abrupt Headache onset
  3. Jaw Claudication (or tongue Claudication)
  4. Limb Claudication
  5. Temporal artery abnormalities (prominence, beading, decreased pulse, tenderness)
  6. Visual disturbance
  7. Upper Cranial Nerve deficit

VII. Differential Diagnosis

  1. Precautions
    1. Polymyalgia Rheumatica (PMR) is a clinical diagnosis with no absolute definitive test
    2. PMR is also a diagnosis of exclusion (other disorders make PMR less likely)
  2. Musculoskeletal disorders including inflammatory Arthritis
    1. See Shoulder Pain
    2. See Rheumatologic Causes of Shoulder Pain
    3. Adhesive Capsulitis
    4. Fibromyalgia
    5. Gout
    6. Pseudogout
    7. Osteoarthritis
    8. Rheumatoid Arthritis
    9. Spondyloarthropathy
    10. Subacromial Bursitis
    11. Systemic Lupus Erythematosus
  3. Myopathy
    1. See Myopathy Causes
    2. See Polymyositis Differential Diagnosis
    3. See Medication Causes of Myositis
    4. Thyroid Myopathy (Hypothyroidism, Hyperthyroidism)
    5. Hyperparathyroidism
    6. Polymyositis
    7. Parkinsonism or other neurologic or Movement Disorder
    8. Statin-Induced Myopathy
  4. Prominent systemic symptoms (e.g. weight loss, night pain)
    1. Malignancy
      1. Multiple Myeloma
      2. Lymphoma
      3. Prostate Cancer
      4. Stomach Cancer
      5. Ovarian Cancer
      6. Renal Cell Cancer
      7. Lung Cancer and paraneoplastic syndrome
    2. Infection
      1. Subacute Bacterial Endocarditis
      2. Tuberculosis
      3. HIV Infection
      4. Hepatitis B
      5. Hepatitis C
      6. Parvovirus B19

VIII. Diagnosis: British Society for Rheumatology (BSR) and British Health Professionals in Rheumatology (BHPR) Criteria

  1. Age over 50 years old
  2. Bilateral ache in Shoulder and/or pelvic girdle
  3. Morning stiffness >45 minutes in Shoulder and/or pelvic girdle
  4. Duration of symptoms >2 weeks
  5. Acute phase reactant increase (i.e. CRP or ESR)

IX. Labs

  1. Acute phase reactant increased (obtain both C-RP and ESR)
    1. C-Reactive Protein (C-RP)
      1. Better Test Sensitivity for Polymyalgia Rheumatica than ESR (elevated in >90% of PMR cases)
      2. ESR however is preferred for predicting relapse
    2. Erythrocyte Sedimentation Rate (ESR)
      1. Normal upper limit ESR is typically age/2 for men and (age+10)/2 for women
      2. ESR >40 mm/h in >91% of Polymyalgia Rheumatica
        1. False Negative ESR in 6-20% of patients with Polymyalgia Rheumatica
        2. C-RP is typically positive in these False Negative cases
      3. ESR >50 mm/h in most cases (mean 65 mm/h)
      4. ESR 83 mm/h is average for Giant Cell Arteritis
        1. ESR >100 mm is associated with higher likelihood of Giant Cell Arteritis (or underlying malignancy)
  2. Nonspecific Lab findings in Polymylagia Rheumatica (PMR)
    1. Moderate Anemia
    2. Decreased Serum Albumin
    3. Mildly elevaled Alkaline Phosphatase
  3. Labs to evaluate differential diagnosis
    1. Complete Blood Count with Platelet Count
      1. Normochromic Anemia and Thrombocytosis may be present in PMR
    2. Thyroid Stimulating Hormone (TSH)
    3. Urinalysis
    4. Comprehensive metabolic panel
      1. Includes Electrolytes, Renal Function tests and Liver Function Tests
      2. Mildly elevaled Alkaline Phosphatase and decreased Serum Albumin may be present in PMR
    5. Creatine Phosphokinase (CPK)
      1. Normal in PMR, in contrast to its elevation in Polymyositis or Rhabdomyolysis
    6. Rheumatologic Serology and other advanced testing as indicated (normal in PMR)
      1. Rheumatoid Factor
      2. Antinuclear Antibody
      3. Antineutrophil Cytoplasmic Antibody (ANCA)
      4. Anti-Citrullinated Peptide Antibody
      5. Serum Protein Electrophoresis or Urine Protein electrophoresis

X. Imaging

  1. Chest XRay
    1. Consider if paraneoplastic syndrome associated with Lung Cancer is suspected
  2. Shoulder Ultrasound
    1. Subdeltoid Bursitis is present in 79% of PMR
    2. Other associated findings include Biceps Tenosynovitis or glenohumeral synovitis
    3. Falsetti (2011) Scand J Rheumatol 40(1): 57-63 [PubMed]

XI. Associated Conditions: Temporal Arteritis

  1. Occurs in 18-26% of PMR patients
  2. Risk of blindness
  3. Consider Temporal Arteritis in all PMR patients
  4. Factors suggesting concurrent Temporal Arteritis
    1. Age over 70 years
    2. New onset Headache
    3. Jaw Claudication
    4. Raised liver enzymes
    5. Abnormal temporal arteries on exam
  5. References
    1. Rodriguez-Valverde (1997) Am J Med 102:331-6 [PubMed]

XII. Management

  1. General measures
    1. Consider concurrent Temporal Arteritis (See above)
    2. NSAIDs (Exercise caution due to Gastritis risk of Corticosteroids)
    3. Exercise program to maintain Muscle mass and mobility
    4. Fall Prevention
  2. Prednisone (key to management)
    1. See Corticosteroid Associated Osteoporosis
    2. Efficacy: 90% response
      1. Dramatic improvement within first week (especially in the first 48 hours)
      2. Acute phase reactants (C-RP and ESR) normalize within first 4 weeks
      3. If no response to steroids
        1. Reconsider differential diagnosis
        2. Consider Methotrexate (see below)
    3. Polymyalgia alone
      1. Dose: 15-20 mg (up to 25 mg) orally daily
        1. Prednisone 15 mg is sufficient for most patients and no added benefits to higher dose
        2. Prednisone 10 mg is associated with increased relapse rate
      2. Example Prednisone taper course
        1. Prednisone 15 mg daily for 3 weeks, then
        2. Prednisone 12.5 mg daily for 3 weeks, then
        3. Prednisone 10 mg daily for 4-6 weeks, then
        4. Prednisone taper by 1 mg/day every 4-8 weeks over 1-2 years
      3. Alternative: Example Methylprednisolone IM course
        1. Indicated in those at risk of Corticosteroid adverse effects
        2. Methylprednisolone (Depo Medrol) 120 mg IM every 3-4 weeks
        3. Taper by 20 mg/dose every 2-3 months
      4. Relapse management
        1. Re-evaluate diagnosis
        2. Increase Prednisone dose 10 to 20% over baseline dose prior to relapse
        3. Once symptoms stabilize, restart slow Corticosteroid taper over 4 to 8 weeks
    4. Polymyalgia with Temporal Arteritis
      1. Dose: 40-60 mg orally daily
      2. Symptoms and signs remit within 1 month
      3. Decrease dose by 10% each week after improvement
    5. Course (mean total length of treatment 1.8 years)
      1. Initial: Maintain starting dose for 1 month
      2. First steroid taper (depends on clinical response)
        1. Taper by 2.5 mg per month down to 10 mg/day then
        2. Taper 1 mg per 4-6 weeks down to 5 to 7.5 mg/day
      3. Final steroid taper
        1. Patient is considered in remission once stable on Prednisone 10 mg dose or less
        2. Indicated when symptom free for 6-12 months
        3. Do not taper until sedimentation rate normalizes
        4. Taper by 1 mg every 6-8 weeks until done
      4. Anticipate 2-6 year course of steroids
        1. Relapse common in first 18 months of steroid use
        2. Patients off steroids at 2 years: 25%
      5. Monitoring
        1. Follow C-RP and anticipate decreased levels after initiating therapy
    6. Prevention of Corticosteroid related Osteoporosis
      1. See Corticosteroid Associated Osteoporosis
      2. Vitamin D Supplementation
      3. Calcium Supplementation
      4. Consider DEXA Scan while starting Corticosteroids
      5. Consider bisphosphonate on starting Prednisone
        1. Strongly consider for high risk of Fracture (over 65 years old or prior Fracture)
        2. Also consider if DEXA ScanT-Score -1.5 or less
  3. Adjunctive medications (added to Corticosteroids)
    1. Methotrexate 7.5 to 10 mg orally once weekly
      1. Associated with lower steroid doses and lower relapse rates
      2. Consider in patients at risk of relapse, or in whom lower Corticosteroid dose would be optimal (e.g. diabetes, Osteoporosis)
      3. Caporali (2004) Ann Intern Med 141(7):493-500 +PMID: 15466766 [PubMed]
    2. Biologic agents (e.g. Etanercept, Infliximab) have not been found to be beneficial in PMR
      1. Kreiner (2010) Arthritis Res Ther 12(5):R176 [PubMed]
      2. Salvarani (2007) Ann Intern Med 146(9): 631-9 [PubMed]

XIII. Management: Follow-up

  1. Rheumatology referral indications (most cases referred in U.S.)
    1. Age <60 years old at onset
    2. Chronic presentation >2 years
    3. Other Rheumatologic Disease
    4. Poor response to Corticosteroids
    5. Significant systemic symptoms (e.g. weight loss, neurologic symptoms)
    6. Significant increases in acute phase reactants (e.g. ESR >100 mm/h)
    7. Absent key PMR features
      1. Minimal morning stifffness
      2. No Shoulder involvement
  2. Clinic Visits
    1. Timing
      1. One week after starting steroids, then
      2. Three weeks after starting steroids, and then
      3. Every 3 months
    2. Labs (each visit)
      1. Complete Blood Count
      2. Erythrocyte Sedimentation Rate (ESR)
      3. C-Reactive Protein (C-RP)
      4. Basic chemistry panel (Electrolytes, Renal Function tests, and Serum Glucose)
    3. Evaluation
      1. Relapse symptoms
        1. Proximal Muscle pain (Shoulder or pelvic girdle)
        2. Morning stiffness
        3. Fatigue
      2. Giant Cell Arteritis symptoms
        1. Temporal Headache
        2. Jaw Claudication (or tongue Claudication)
        3. Vision changes
      3. Adverse effects to treatment (Corticosteroid adverse effects)
        1. Gastritis or peptic ulcer
        2. Bone density
        3. Hyperglycemia
    4. Approach
      1. Individualize Corticosteroid dosing and taper based on symptoms, signs, lab markers and adverse effects
      2. Continue to reevaluate differential diagnosis in refractory cases and in recurrent relapse
      3. Consider Bone Mineral Density screening (DEXA Scan)
        1. See Steroid-Related Bone Mineral Density Loss

XIV. Prognosis

  1. PMR associated synovitis is non-erosive and should not cause longterm tissue damage after PMR resolution
  2. Self limited course over years (usually 3-6 years)
  3. Polymyalgia Rheumatica (PMR) Relapse or Temporal Arteritis Risk Factors (relapse occurs in up to 50% of patients)
    1. Female Gender
    2. High baseline Erythrocyte Sedimentation Rate (ESR)
    3. Initial Prednisone dosing <10 mg/day or >20 mg/day
    4. Faster than typical Corticosteroid taper

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Related Studies

Ontology: Giant Cell Arteritis (C0039483)

Definition (MEDLINEPLUS)

Giant cell arteritis is a disorder that causes inflammation of arteries of the scalp, neck, and arms. The inflammation narrows the arteries, which keeps blood from flowing well. Giant cell arteritis often occurs with another disorder called polymyalgia rheumatica. Both disorders are more common in women than in men. They almost always affect people over the age of 50.

Early symptoms of giant cell arteritis resemble the flu: fatigue, loss of appetite, and fever. Other symptoms include headaches, pain and tenderness over the temples, double vision or visual loss, dizziness, and problems with coordination and balance. You may also have pain in your jaw and tongue.

Your doctor will make the diagnosis based on your medical history, symptoms, and physical examination. There is no single test to diagnose giant cell arteritis, but you may have tests that measure inflammation.

Treatment is usually with corticosteroids. Early treatment is important; otherwise there is a risk of permanent vision loss or stroke. However, when properly treated, giant cell arteritis rarely comes back.

NIH: National Institute of Arthritis and Musculoskeletal and Skin Diseases

Definition (MSH) A systemic autoimmune disorder that typically affects medium and large ARTERIES, usually leading to occlusive granulomatous vasculitis with transmural infiltrate containing multinucleated GIANT CELLS. The TEMPORAL ARTERY is commonly involved. This disorder appears primarily in people over the age of 50. Symptoms include FEVER; FATIGUE; HEADACHE; visual impairment; pain in the jaw and tongue; and aggravation of pain by cold temperatures. (From Adams et al., Principles of Neurology, 6th ed)
Concepts Disease or Syndrome (T047)
MSH D013700
ICD9 446.5
SnomedCT 155442004, 195354005, 195357003, 195355006, 195356007, 87511001, 400130008, 414341000
English Arteritides, Giant Cell, Giant Cell Arteritides, Arteritis, Giant Cell, GCA - Giant cell arteritis, Giant cell arteritis NOS, GIANT CELL ARTERITIS, GCA, POLYMYALGIA RHEUMATICA, HORTON DIS, HORTONS DIS, Arteritis, Giant Cell, Horton's, Giant Cell Arteritis, Horton's arteritis, Arteritis, Giant Cell, Horton, Giant Cell Arteritis, Horton, Horton Giant Cell Arteritis, Horton's Giant Cell Arteritis, Horton Disease, Horton's Disease, Hortons Disease, Giant Cell Arteritis [Disease/Finding], horton's disease, gca, horton disease, horton's arteritis, arteritis cell giant, giant cell arteritis, giant cell arteritis (diagnosis), Giant cell arteritis NOS (disorder), Horton's disease, giant cell; arteritis, arteritis; giant cell, Giant cell arteritis, Giant cell arteritis (disorder), Arteritis;giant cell
Dutch giant cell arteritis, ziekte van Horton, Horton arteritis, arteriitis; reuscel, reuscel; arteriitis
French Artérite temporale, Artérite à cellules géantes, Maladie d'Horton, Artérite giganto-cellulaire de Horton, Artérite gigantocellulaire de Horton, Maladie de Horton
German Horton-Arteritiis, Horton-Krankheit, Riesenzellen-Arteriitis, Senile Riesenzellarteriitis, Horton-Magath-Brown-Syndrom, Riesenzellarteriitis, Temporalarteriitis, Horton-Neuralgie, Horton-Riesenzellarteriitis
Italian Arterite di Horton, Arterite a cellule giganti, Malattia di Horton, Arterite a cellule giganti di Horton
Portuguese Arterite de células gigantes, Arterite de Horton, Arterite de Células Gigantes, Arterite de Células Gigantes de Horton, Arterite de Horton de Células Gigantes, Doença de Horton
Spanish Arteritis de células gigantes, Arteritis de Células Gigantes, enfermedad de Horton, arteritis temporal de células gigantes, arteritis de células gigantes, SAI (trastorno), arteritis de células gigantes, SAI, Arteritis de Células Gigantes de Horton, arteritis de células gigantes (trastorno), arteritis de células gigantes, Arteritis de Horton, Enfermedad de Horton
Japanese ホートン病, ホートンドウミャクエン, ホートンビョウ, キョサイボウセイドウミャクエン, 動脈炎-側頭, ホートン動脈炎, 動脈炎-巨細胞性, 巨細胞性動脈炎, 側頭動脈炎
Finnish Ohimovaltimotulehdus
Swedish Jättecellsarterit
Czech Hortonova arteriitida, Obrovskobuněčná arteriitida, obrovskobuněčná arteritida, Hortonova obrovskobuněčná arteriitida, Hortonova choroba, Hortonova nemoc, gigantocelulární arteritida
Polish Olbrzymiokomórkowe zapalenie tętnic, Zapalenie tętnic olbrzymiokomórkowe
Hungarian Óriássejtes arteritis, Horton-arteritis, Horton-betegség
Norwegian Hortons arteritt, Temporalarteritt, Kjempecellearteritt, Arteritis temporalis