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Polymyalgia Rheumatica (PMR)

BASICS

  • Definition: Inflammatory syndrome with pain and morning stiffness of shoulder, hip girdles, and neck in adults >50 years.
  • Features: Morning stiffness, elevated inflammatory markers, rapid onset of symptoms.
  • Associated: ~15–30% have concurrent giant cell arteritis (GCA).
  • Systems: Musculoskeletal, hematologic/lymphatic/immunologic
  • Synonyms: Senile rheumatic disease, PMR syndrome, pseudo-polyarthrite rhizomélique

EPIDEMIOLOGY

  • Incidence: Increases after age 50; 50/100,000 for PMR, 18/100,000 for GCA (US data)
  • Sex: Female > Male (2–3:1)
  • Ethnicity: Most common in Caucasians, especially Northern Europeans/Scandinavians
  • Peak: Ages 70–80 years
  • Prevalence: 700/100,000 in those >50 years old
  • Rare in patients <50 years

ETIOLOGY & PATHOPHYSIOLOGY

  • Unknown; involves immune dysregulation and periarticular inflammation.
  • Polygenic: HLA-DRB104 and DRB101 alleles implicated.
  • Environmental: Parvovirus B19 DNA found in temporal artery specimens (GCA association).

RISK FACTORS

  • Age >50 years (key risk)
  • Presence of GCA

COMMONLY ASSOCIATED CONDITIONS

  • Giant Cell Arteritis (GCA): 15–30% of PMR patients; higher in women

DIAGNOSIS

History

  • Symptoms: Proximal limb pain/stiffness (neck, shoulders, hips)
  • Onset: Rapid
  • Functional: Difficulty rising from chair, combing hair, dressing
  • Other: Night pain, severe morning stiffness, can become bilateral, fatigue, weight loss, low-grade fever, depression, anorexia
  • Carpal tunnel: 10–15% of cases
  • Duration: Usually >2 weeks

Physical Exam

  • Decreased ROM in shoulders/neck/hips
  • Normal muscle strength (limited by pain/stiffness)
  • Muscle tenderness, disuse atrophy
  • Synovitis/tenosynovitis (not feet/ankles)
  • Possible carpal tunnel syndrome

Differential Diagnosis

  • Rheumatoid arthritis (elderly-onset)
  • Palindromic rheumatism
  • Late-onset seronegative spondyloarthropathies
  • SLE, Sjögren syndrome, fibromyalgia, depression
  • Polymyositis/dermatomyositis (check CK/aldolase)
  • Thyroid disease, vitamin D deficiency
  • Osteoarthritis, rotator cuff syndrome, adhesive capsulitis
  • RS3PE syndrome, infection, malignancy, myopathy

Diagnostic Criteria (ACR/EULAR 2012)

  • Patients ≥50 years, bilateral shoulder aching, abnormal CRP or ESR
  • ≥4 points (without US) or ≥5 points (with US):
  • Morning stiffness >45 min (2 pts)
  • Hip pain or restricted ROM (1 pt)
  • Absence of RF or ACPA (2 pts)
  • Absence of other joint involvement (1 pt)
  • (US) Subdeltoid bursitis, biceps tenosynovitis, or glenohumeral synovitis (shoulder/hip findings)
  • Lab: ESR >40 mm/hr (but can be normal), ↑ CRP, normocytic anemia, RF/anti-CCP usually negative, normal CK, mild ↑ LFTs, negative ANA

Initial & Other Tests

  • ESR, CRP, CBC
  • MRI/US (for periarticular inflammation/bursitis, if needed)
  • Temporal artery biopsy if GCA symptoms
  • Exclude infection, malignancy, thyroid, vitamin D deficiency

TREATMENT

General Measures

  • Address risk of steroid-induced osteoporosis: DEXA, vitamin D (800–1000 IU/day), calcium (1500 mg/day), consider bisphosphonates
  • Physical therapy for ROM as needed

First-line Medication

  • Prednisone: 10–20 mg/day PO (most respond to 15 mg/day)
  • Expect dramatic response in days; if not, reconsider diagnosis
  • If needed, increase to 20 mg/day
  • Divided doses (BID/TID) may help at start
  • Taper by 2.5 mg every 2–4 weeks to 7.5–10 mg/day, then by 1 mg/month to prevent relapse
  • Relapse: Increase dose temporarily
  • May stop after 6–12 months if symptom-free and normal ESR/CRP

  • Precautions: Use steroids cautiously in CHF, DM, active infections; monitor for side effects

Second-line & Adjunct

  • Methotrexate: May reduce relapse and steroid exposure (limited data)
  • Tocilizumab: IL-6 inhibitor (case reports, not routine)
  • NSAIDs: Not adequate for pain relief
  • Steroid injections: For shoulder pain

Monitor For:

  • Steroid side effects: osteoporosis, hypertension, hyperglycemia, infections, cataracts/glaucoma, weight gain
  • GCA development: requires higher prednisone (40–60 mg) if suspected

ONGOING CARE

  • Follow-up: Monthly initially/with taper, then every 3 months
  • Monitor: ESR/CRP (follow symptoms, not just labs), watch for GCA symptoms (headache, vision changes), osteoporosis risk

  • Do NOT: Increase steroids solely to normalize ESR if patient is asymptomatic


DIET

  • Regular diet; ensure adequate calcium and vitamin D

PATIENT EDUCATION

  • Review corticosteroid side effects and the importance of not stopping abruptly
  • Educate on GCA symptoms (seek immediate care for headache, vision loss, diplopia)
  • Arthritis Foundation & ACR resources

PROGNOSIS

  • Most require ≥2 years of corticosteroids
  • Relapse common with rapid taper (25–50%)
  • Prognosis is good with treatment
  • Older age, female sex, high baseline ESR, high initial steroid dose = prolonged course/increased flares

COMPLICATIONS

  • Steroid-induced complications
  • GCA development (even if PMR controlled)

ICD-10

  • M35.3 Polymyalgia rheumatica
  • M31.5 Giant cell arteritis with PMR

CLINICAL PEARLS

  • Consider PMR in patients >50 years with new-onset hip, neck, or shoulder pain/stiffness.
  • Normal ESR does not exclude PMR.
  • Rapid, dramatic steroid response is characteristic.
  • Adjust steroids based on symptoms, not just ESR/CRP.