What are the symptoms and treatment options for polymyalgia rheumatica (PMR) and temporal arteritis (giant cell arteritis, GCA)?

Symptoms and Treatment for Polymyalgia Rheumatica and Temporal Arteritis

Glucocorticoids are the cornerstone of treatment for both polymyalgia rheumatica (PMR) and giant cell arteritis (GCA), with the primary goal being to achieve remission, prevent tissue ischemia, and minimize treatment-related adverse effects. 1

Symptoms

Polymyalgia Rheumatica (PMR)

• Characteristic symptoms:
  • New-onset bilateral shoulder pain
  • Subdeltoid bursitis (present in 69% of patients on ultrasound) 2
  • Morning stiffness in shoulders and hips
  • Muscle and joint pain
  • Functional impairment
  • Constitutional symptoms (fever, malaise, weight loss)
  • Elevated inflammatory markers (ESR, CRP) in >90% of cases 2

Giant Cell Arteritis (GCA)

• Cranial symptoms:
  • New-onset headaches
  • Temporal artery tenderness or decreased pulsation
  • Jaw claudication
  • Visual disturbances including vision loss (MEDICAL EMERGENCY)
  • Constitutional symptoms (fever, malaise, weight loss)
  • Elevated inflammatory markers (ESR, CRP) in >90% of cases 2
• Large vessel involvement:
  • Limb claudication
  • Vascular bruits
  • Asymmetric blood pressure readings
  • Aortic aneurysm (late complication)

Diagnosis

• PMR: Clinical features plus elevated inflammatory markers; ultrasound showing bilateral subdeltoid bursitis improves diagnostic accuracy 2
• GCA: Temporal artery biopsy remains the gold standard; however, ultrasound or MRI showing inflammation-induced wall thickening may be sufficient in patients with typical clinical features 2

Treatment

Polymyalgia Rheumatica

1. Initial therapy:

   • Prednisone 12.5-25 mg/day 2
   • Some patients may respond to 15-20 mg/day, but lower doses (10 mg/day) result in relapse in 65% of patients 3

2. Tapering schedule:

   • Reduce to 7.5-10 mg by 8 weeks 4
   • Aim for maintenance dose of 7.5 mg after 6-9 months 4
   • Gradual tapering thereafter

Giant Cell Arteritis

1. Initial therapy:

   • URGENT treatment is essential to prevent irreversible blindness 5
   • Prednisone 40-60 mg/day 2
   • Higher doses (60-80 mg) may be needed for persistent visual symptoms 4

2. Tapering schedule:

   • Reduce to about 20 mg by 8 weeks 4
   • Aim for maintenance dose of 7.5 mg after 6-9 months 4
   • Gradual tapering thereafter

3. Adjunctive therapy for GCA with critical vessel involvement:

   • Add aspirin for patients with critical or flow-limiting involvement of vertebral or carotid arteries 1

Management of Relapses

1. For PMR relapse:

   • Increase glucocorticoid dose 1

2. For GCA relapse with cranial ischemia symptoms:

   • Add a non-glucocorticoid immunosuppressive agent AND increase glucocorticoid dose 1
   • Tocilizumab is preferred over methotrexate due to superior glucocorticoid-sparing effect 1

3. For GCA relapse while on moderate-to-high dose glucocorticoids:

   • Add a non-glucocorticoid immunosuppressive drug 1

Glucocorticoid-Sparing Agents

1. Methotrexate:

   • Can reduce cumulative glucocorticoid dosage by 20-44%
   • Can reduce relapses by 36-54% in both PMR and GCA 2

2. Tocilizumab:

   • Shown to increase remission rates 2-4 fold in GCA 2
   • More effective glucocorticoid-sparing agent than methotrexate 1

Treatment Duration and Monitoring

• Duration:

  • Treatment withdrawal possible within 2 years for some patients
  • Many require 4+ years of therapy 4
  • Some patients relapse after withdrawal (all within 21 months in one study) 6

• Monitoring:

  • Regular clinical assessment for disease activity
  • Monitor ESR and CRP, though increases in inflammatory markers alone do not warrant treatment escalation without clinical symptoms 1
  • Monitor for glucocorticoid-related adverse effects (osteoporosis, diabetes, etc.) 5

Important Caveats

1. Visual symptoms in GCA require IMMEDIATE treatment to prevent irreversible blindness 5

2. PMR can evolve into GCA during treatment, requiring dose adjustment (observed in 6 patients in one study) 3

3. No rigid treatment schedule should be used; therapy must be adjusted based on clinical response 6

4. Treatment target should be remission, defined as absence of clinical symptoms and systemic inflammation 1

5. Surgical intervention for GCA with worsening limb/organ ischemia should only be considered after escalating immunosuppressive therapy 1