Rheumatology Book

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Polymyalgia RheumaticaAka: PMR

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  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
  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
    2. Selvarani (2002) N Engl J Med 347:261
    3. Unwin (2006) Am Fam Physician 74:1547
    4. Weyand (2003) Ann Intern Med 139:505

Giant Cell Arteritis (C0039483)

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)
ConceptsDisease or Syndrome (T047)
ICD9446.5, 446.5
MSHD013700
EnglishARTERITIS OF THE AGED, GCA, GCA - Giant cell arteritis, Giant Cell Arteritides, GIANT CELL ARTERITIS, HORTON DIS, HORTON DISEASE, Horton Giant Cell Arteritis, Horton's disease, Horton's Giant Cell Arteritis, HORTONS DIS, Hortons Disease, POLYMYALGIA RHEUMATICA, Temporal giant cell arteritis
Spanisharteritis de celulas gigantes, arteritis temporal de celulas gigantes, enfermedad de Horton
Parent ConceptsExaminer's Diagnosis (C0178413), Polyarteritis nodosa and allied conditions NOS (C0155757), Arteritis (C0003860), Skin Diseases, Vascular (C0162819), Vasculitis, Central Nervous System (C0751878), Giant Cell Arteritis (C0039483), Ambiguous concept (C1274012)
SourcesAIR, COSTAR, CSP, DXP, ICD9CM, MEDLINEPLUS, MSH, MTH, MTHICD9, NCI, OMIM, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)



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