What is the recommended treatment for sarcoidosis?

Recommended Treatment for Sarcoidosis

For patients with sarcoidosis requiring treatment, oral glucocorticoids (prednisone) at an initial dose of 20 mg once daily are the first-line therapy, with methotrexate (10-15 mg weekly) being an equally effective alternative first-line option with a different side effect profile. 1, 2, 3

Treatment Algorithm for Sarcoidosis

Step 1: Determine Need for Treatment

• Treatment is indicated for:
  • Symptomatic patients (cough, dyspnea)
  • Patients with parenchymal infiltrates
  • Abnormal pulmonary function tests
  • Bone lytic lesions
  • Significant extrapulmonary organ involvement

Step 2: First-Line Treatment Options

Option A: Prednisone

• Initial dose: 20 mg once daily 1, 2
• Duration: 3-6 months with response assessment 2
• Maintenance dose: 5-10 mg daily or every other day 2
• Monitoring:
  • Bone density
  • Blood pressure
  • Serum glucose 1
• Common side effects: diabetes, hypertension, weight gain, osteoporosis, cataracts, glaucoma, mood changes 1

Option B: Methotrexate (Alternative First-Line)

• Recent high-quality evidence shows methotrexate is non-inferior to prednisone for pulmonary sarcoidosis 3
• Dosage: 10-15 mg once weekly 1, 2
• Monitoring: CBC, hepatic and renal function tests 1
• Common side effects: nausea, leukopenia, hepatotoxicity 1
• Contraindication: significant renal failure 1

Step 3: Second-Line Treatment Options

For patients with inadequate response or intolerance to first-line therapy:

• Leflunomide: 10-20 mg once daily 1
• Azathioprine: 50-250 mg once daily 1
• Mycophenolate mofetil: 500-1500 mg twice daily 1

Step 4: Third-Line Treatment Options

For persistent disease despite previous treatments:

• Infliximab: 3-5 mg/kg initially, 2 weeks later, then every 4-6 weeks 1, 2
  • Requires TB screening
  • Contraindicated in severe CHF, prior malignancy, demyelinating neurologic disease 1
• Adalimumab: 40 mg every 1-2 weeks 1
• Rituximab: 500-1000 mg every 1-6 months 1

Special Considerations

Symptom-Specific Management

• For fatigue:

  • Pulmonary rehabilitation program for 6-12 weeks 1, 2
  • Inspiratory muscle strength training 1, 2
  • For troublesome fatigue not related to disease activity: D-methylphenidate or armodafinil for 8 weeks 1, 2

• For hypercalcemia:

  • Hydroxychloroquine: 200-400 mg once daily 1, 2

Treatment Duration and Monitoring

• Regular clinical assessment for symptom improvement
• Radiographic follow-up
• Pulmonary function testing
• Monitoring for adverse effects

Important Caveats

1. Relapse rates range from 13% to 75% depending on disease stage, organ involvement, and other factors 4
2. Approximately 10-40% of patients develop progressive pulmonary disease despite treatment 4
3. Mortality rate for sarcoidosis over 5 years is approximately 7%, with >60% of deaths due to advanced cardiopulmonary disease 4
4. Consider Pneumocystis prophylaxis in patients on high-dose immunosuppression (≥20 mg prednisone with a cytotoxic agent for >6 months) 2

Treatment Selection Considerations

The choice between prednisone and methotrexate as first-line therapy should consider:

• The recent PREDMETH study demonstrated methotrexate is non-inferior to prednisone for improving FVC at 24 weeks 3
• Prednisone has a faster onset of action but more metabolic side effects (weight gain, insomnia, increased appetite) 3
• Methotrexate has fewer systemic side effects but may cause nausea, fatigue, and liver function abnormalities 3
• Patient comorbidities (diabetes, osteoporosis, renal function)
• Patient preference regarding side effect profiles