Rheumatology Book

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Polymyalgia Rheumatica

Aka: Polymyalgia Rheumatica, PMR
  1. See Also
    1. Temporal Arteritis
  2. Epidemiology
    1. Common over age 50 years
      1. Incidence: 50 per 100,000
    2. Rare in Asian and black patients
    3. Women predominate by 2:1 ratio
    4. Associated with HLA-DR4 and Cw3 haplotypes
    5. Associated with Temporal Arteritis (15%)
  3. Symptoms
    1. Severe muscle ache and stiffness
      1. Duration: 1 month or longer at presentation
        1. Usually insidious onset
      2. Location (symmetric involvement)
        1. Shoulders (most commonly affected)
        2. Neck
        3. Pelvic girdle and hips
      3. Characteristics
        1. Ache and stiffness
      4. Timing
        1. More prominent in morning or after inactivity
    2. Associated systemic symptoms
      1. Malaise
      2. Anorexia
      3. Weight loss
      4. Low grade fever
      5. Depressed mood
      6. Night Sweats
  4. Signs
    1. Unremarkable physical exam
      1. Symptoms are usually well out-of-proportion to exam
      2. No true weakness
    2. Mild findings (variably present)
      1. Limited range of motion in affected joints
        1. Limited by proximal myalgias
      2. Shoulder or hip bursitis
      3. Localized tenderness over Shoulders and hips
    3. Other findings which may be present
      1. Asymmetric knee or wrist arthritis
      2. Carpal Tunnel Syndrome
      3. Distal extremity edema
  5. Diagnosis of Exclusion; Need Absence of
    1. Inflammatory arthritis
    2. Infection
    3. Malignancy
    4. Hypothyroidism
  6. Labs
    1. Erythrocyte Sedimentation Rate
      1. Increased > 50 mm in 1 hour (often >100 mm)
    2. Creatine Phosphokinase (CPK) Normal
      1. Differentiate from Polymyositis
    3. Nonspecific Lab findings
      1. Moderate Anemia
      2. Decreased Serum Albumin
      3. Mild hepatic dysfunction
  7. Associated Conditions: Temporal Arteritis
    1. Occurs in 15% 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
  8. Management
    1. General measures
      1. Consider concurrent Temporal Arteritis (See above)
      2. NSAIDs
    2. Prednisone (key to management)
      1. See Corticosteroid Associated Osteoporosis
      2. Efficacy: 90% response
        1. Dramatic improvement in first 48 hours
        2. If no response to steroids
          1. Reconsider diagnosis
          2. Consider Methotrexate
      3. Polymyalgia alone
        1. Dose: 15-20 mg PO qd
      4. Polymyalgia with Temporal Arteritis
        1. Dose: 40-60 mg PO qd
        2. Symptoms and signs remit within 1 month
        3. Decrease dose by 10% each week after improvement
      5. Course
        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. Indicated when symptom free for 6-12 months
          2. Do not taper until sedimentation rate normalizes
          3. 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%
      6. Prevention of Corticosteroid related Osteoporosis
        1. See Corticosteroid Associated Osteoporosis
  9. Prognosis
    1. Self limited course over years (usually 3-6 years)
  10. References
    1. Ostor (2002) Practitioner 246:756-63
    2. Selvarani (2002) N Engl J Med 347:261-71
    3. Unwin (2006) Am Fam Physician 74:1547-58
    4. Weyand (2003) Ann Intern Med 139:505-15

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 195356007, 195354005, 195355006, 195357003, 400130008, 155442004, 87511001, 414341000
English Arteritides, Giant Cell, Giant Cell Arteritides, ARTERITIS OF THE AGED, ARTERITIS, GIANT CELL, Arteritis, Giant Cell, HORTON DISEASE, GCA - Giant cell arteritis, Giant cell arteritis NOS, Giant cell arteritis syndrome, GIANT CELL ARTERITIS, GCA, POLYMYALGIA RHEUMATICA, HORTON DIS, HORTONS DIS, Arteritis, Giant Cell, Horton's, Giant Cell Arteritis, 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 NOS (disorder), Giant Cell Arteritis [Disease/Finding], horton's disease, gca, horton disease, horton's arteritis, arterities cell giant, arteritis cell giant, 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 Horton de Células Gigantes, Doença de Horton, Arterite de Células Gigantes de Horton
Spanish Arteritis de células gigantes, Giant cell arteritis, Arteritis de Celulas Gigantes, Arteritis de Células Gigantes, arteritis de células gigantes, SAI (trastorno), arteritis de células gigantes, SAI, arteritis temporal de células gigantes, enfermedad de Horton, arteritis de células gigantes (trastorno), arteritis de células gigantes, Arteritis de Horton, Enfermedad de Horton, Arteritis de Celulas Gigantes de Horton, Arteritis de Células Gigantes de Horton
Japanese ホートン病, ﾎｰﾄﾝﾄﾞｳﾐｬｸｴﾝ, ﾎｰﾄﾝﾋﾞｮｳ, ｷｮｻｲﾎﾞｳｾｲﾄﾞｳﾐｬｸｴﾝ, 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
Czech Hortonův syndrom, Hortonova arteriitida, Hortonova nemoc, Obrovskobuněčná arteriitida, obrovskobuněčná arteritida
Finnish Ohimovaltimotulehdus
Russian АРТЕРИИТ ВИСОЧНЫЙ, ARTERIIT TEMPORAL'NYI, АРТЕРИИТ ГИГАНТОКЛЕТОЧНЫЙ, ARTERIIT VISOCHNYI, GIGANTOKLETOCHNYI ARTERIIT, GRANULEMATOZNYI ARTERIIT, ARTERIIT GIGANTOKLETOCHNYI, АРТЕРИИТ ТЕМПОРАЛЬНЫЙ, ГИГАНТОКЛЕТОЧНЫЙ АРТЕРИИТ, ГРАНУЛЕМАТОЗНЫЙ АРТЕРИИТ
Swedish Jättecellsarterit
Polish Olbrzymiokomórkowe zapalenie tętnic, Zapalenie tętnic olbrzymiokomórkowe
Hungarian Óriássejtes arteritis, Horton-arteritis, Horton-betegség
Sources
Derived from the NIH UMLS (Unified Medical Language System)


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