What is the recommended treatment for sarcoidosis?

Treatment for Sarcoidosis

For patients with symptomatic pulmonary sarcoidosis at higher risk of mortality or permanent disability, systemic glucocorticoids (prednisone) are the first-line treatment, with methotrexate as the preferred second-line agent and infliximab as the third-line option when disease persists despite previous treatments. 1

Treatment Algorithm Based on Disease Severity and Organ Involvement

Asymptomatic Sarcoidosis

• No treatment is recommended for asymptomatic pulmonary sarcoidosis (even with radiographic abnormalities) 1
• Observation with regular monitoring is the appropriate approach
• There is little evidence that corticosteroid treatment in asymptomatic patients changes the natural course of the disease 1

Symptomatic Pulmonary Sarcoidosis

1. First-line: Systemic Glucocorticoids

   • Initial dose: Prednisone 20 mg once daily 1
   • Duration: 3-6 months with response assessment 1
   • Maintenance: 5-10 mg daily or every other day 1
   • Monitoring: Bone density, blood pressure, serum glucose 1

2. Second-line: Methotrexate

   • Indications: Continued disease despite glucocorticoids or unacceptable steroid side effects 1
   • Dosage: 10-15 mg once weekly 1
   • Monitoring: CBC, hepatic and renal function tests 1

3. Third-line: Infliximab

   • Indications: Continued disease despite glucocorticoids and second-line agents 1
   • Dosage: 3-5 mg/kg initially, 2 weeks later, then every 4-6 weeks 1
   • Screening: Prior TB, contraindicated in severe CHF, prior malignancy, demyelinating neurologic disease 1

Neurosarcoidosis

• Strong recommendation for glucocorticoids as first-line treatment 1
• Methotrexate as second-line agent 1
• Infliximab as third-line agent when disease persists despite previous treatments 1

Cutaneous Sarcoidosis

• For cosmetically important active skin lesions not controlled by local treatment, oral glucocorticoids are recommended 1
• For persistent skin disease despite glucocorticoids, infliximab is suggested 1

Cardiac Sarcoidosis

• Strong recommendation for glucocorticoids (with or without other immunosuppressives) for patients with functional cardiac abnormalities 1

Management of Sarcoidosis-Associated Fatigue

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

Important Considerations and Pitfalls

Steroid-Related Complications

• Prolonged use of even low-dose prednisone can cause significant toxicity 1
• Complications include diabetes, hypertension, weight gain, osteoporosis, cataracts, glaucoma 1
• Consider steroid-sparing alternatives early in treatment course 1

Monitoring

• Baseline testing: Serum creatinine, alkaline phosphatase, calcium levels, ECG 2
• Regular follow-up to assess response and monitor for adverse effects
• Vitamin D monitoring with both 25-OH and 1,25-OH levels before supplementation 2

Disease Progression

• 10-40% of patients develop progressive pulmonary disease 3
• Stage IV disease (fibrosis) has no chance of resolution and highest mortality risk 3
• Relapse rates range from 13-75% depending on disease stage, organ involvement, and other factors 3

Treatment Duration

• For responsive disease: 6-18 months 4
• For chronic disease: May require long-term treatment 4
• Assess need for glucocorticoid continuation in chronic fibrotic pulmonary sarcoidosis 1

Special Considerations

• Pneumocystis prophylaxis should be considered in patients on high-dose immunosuppression (e.g., ≥20 mg prednisone with a cytotoxic agent for >6 months) 1
• Hydroxychloroquine (200-400 mg daily) may be beneficial for hypercalcemia or skin disease 1
• Inhaled glucocorticoids have not shown significant benefits in controlled trials 1

The treatment approach should be guided by disease severity, organ involvement, and response to therapy, with careful monitoring for adverse effects of medications and disease progression.