What is the initial treatment for sarcoidosis?

Initial Treatment for Sarcoidosis

Oral prednisone at a dose of 20-40 mg daily for 3-6 months is the first-line therapy for symptomatic sarcoidosis. 1

Treatment Decision Algorithm

Step 1: Determine if treatment is needed

• No treatment needed if:

  • Asymptomatic pulmonary sarcoidosis (little evidence that corticosteroids change natural course) 1
  • Possibility of spontaneous remission 1

• Treatment indicated if:

  • Symptomatic disease
  • Risk of mortality or organ failure
  • Significant impairment in quality of life 1

Step 2: First-line treatment approach

• Initial therapy: Prednisone 20-40 mg daily for 3-6 months 1
  • Recent evidence shows that low-dose (20 mg) is as effective as high-dose (40 mg) with similar adverse effect profiles 2
  • Goal: Improve symptoms, preserve organ function, prevent progression to fibrotic disease 1
  • Monitor C-Reactive Protein (CRP) every 1-3 months during initial treatment 1
  • Allow 3-6 months to demonstrate responsiveness 1

Step 3: Response assessment and dose adjustment

• If responding:

  • Taper prednisone to lowest effective dose (5-10 mg daily) 1
  • Continue maintenance therapy for 1-2 years before considering withdrawal 1
  • Be aware that relapse rates upon withdrawal range from 20-80% 1, 3

• If inadequate response or steroid intolerance:

  • Add or switch to methotrexate (10-15 mg once weekly) as a steroid-sparing agent 1
  • Recent evidence suggests methotrexate may be noninferior to prednisone as first-line treatment, with a different side effect profile 4

Treatment Considerations by Disease Phenotype

• Acute phenotype: Start with prednisone 20 mg daily, evaluate after 3-6 months 1
• Chronic phenotype: Add methotrexate if disease progression or steroid toxicity occurs 1
• Advanced phenotype: Consider escalation to infliximab if inadequate response to methotrexate 1

Second and Third-Line Options

• Second-line agents:

  • Methotrexate (10-15 mg once weekly) - most widely studied 1, 5
  • Alternatives: leflunomide, azathioprine, mycophenolate mofetil 1

• Third-line therapy:

  • Infliximab (3-5 mg/kg initially, then at 2 weeks, then every 4-6 weeks) 1
  • Requires TB screening before initiation 1
  • Adalimumab is an alternative anti-TNF agent 1

Monitoring and Adverse Effects

• Monitor for steroid toxicity:

  • Blood pressure, blood glucose, weight, bone density 1
  • Chest X-ray, pulmonary function tests, regular symptom assessment 1
  • Be aware that prolonged use of even low doses can lead to significant toxicity 1

• Common prednisone side effects:

  • Weight gain, insomnia, increased appetite 4

• Common methotrexate side effects:

  • Nausea, fatigue, abnormal liver function tests 4

Important Caveats

• Mortality rate for sarcoidosis over a 5-year follow-up is approximately 7% 3
• 10-40% of patients develop progressive pulmonary disease 3
• Scadding stages for pulmonary sarcoidosis correlate with outcomes:
  • Stages I and II: 30-80% chance of radiographic remission
  • Stage III: Only 10-40% chance of resolution
  • Stage IV: No chance of resolution 3
• Consider steroid-sparing alternatives when:
  • High risk for steroid toxicity exists
  • Systemic/extrapulmonary involvement is present
  • Long-duration therapy is anticipated
  • Inadequate response to steroid therapy occurs 1