What is the recommended treatment for sarcoidosis?

Treatment Approach for Sarcoidosis

Glucocorticoids are the first-line treatment for symptomatic sarcoidosis, with prednisone 20-40 mg daily for 3-6 months recommended as initial therapy, followed by a gradual taper over 6-18 months based on clinical response. 1, 2

Initial Assessment and Treatment Decision

The decision to treat sarcoidosis depends on:

1. Organ involvement and severity:

   • Pulmonary involvement with abnormal PFTs and symptoms
   • Cardiac involvement (heart block, dysrhythmias, cardiomyopathy)
   • Neurological involvement
   • Significant cutaneous disease
   • Hypercalcemia

2. Disease activity markers:

   • Symptoms (cough, dyspnea, fatigue)
   • Radiographic findings
   • Pulmonary function tests
   • Organ-specific assessments

Treatment Algorithm

First-Line Treatment

• Prednisone 20-40 mg daily for 3-6 months 1, 2
• For pulmonary sarcoidosis with symptoms and abnormal PFTs
• For cardiac sarcoidosis (strong recommendation despite low evidence quality) 3
• Taper over 6-18 months if improvement in symptoms, PFTs, and imaging 2

Second-Line Treatment (Steroid-Sparing Agents)

Add when:

• Unable to taper prednisone to ≤10 mg daily
• Significant steroid side effects
• Disease progression despite steroids

Options include:

• Methotrexate: 10-15 mg weekly (monitor for nausea, leukopenia, hepatotoxicity) 1, 4
• Azathioprine: 50-250 mg daily 1, 4
• Leflunomide: 10-20 mg daily 1
• Mycophenolate mofetil: 500-1500 mg twice daily 1, 4
• Hydroxychloroquine: 200-400 mg daily (particularly for cutaneous sarcoidosis and hypercalcemia) 3, 1

Third-Line Treatment (Biologics)

For refractory disease despite second-line therapy:

• Infliximab: 3-5 mg/kg initially, then 2 weeks later, followed by every 4-6 weeks 3, 1, 4
• Adalimumab: 40 mg every 1-2 weeks 3, 1, 4

Organ-Specific Approaches

Pulmonary Sarcoidosis

• Most common (90% of cases) 5
• Treatment indicated for symptomatic disease with abnormal PFTs
• Higher risk of mortality with Stage IV disease (fibrocystic changes), >20% fibrosis on HRCT, or pulmonary hypertension 2

Cardiac Sarcoidosis

• Present in 25% of patients but clinically significant in only 5% 5
• Can be suddenly fatal - screening recommended for all patients 5
• Strong recommendation for glucocorticoids with/without immunosuppressives 3

Cutaneous Sarcoidosis

Stepwise approach:

1. Topical glucocorticoids
2. Systemic prednisone/prednisolone
3. Hydroxychloroquine/chloroquine
4. Methotrexate
5. Infliximab
6. Adalimumab 3

Neurosarcoidosis

• Typically requires combination therapy
• Often treated with infliximab or adalimumab plus second-line agents 3

Sarcoidosis-Associated Fatigue

• Pulmonary rehabilitation program and/or inspiratory muscle strength training for 6-12 weeks 3, 1
• Consider D-methylphenidate or armodafinil for 8 weeks if fatigue persists 3, 1

Monitoring and Follow-up

• Regular assessment of:
  • Symptoms
  • Pulmonary function tests
  • Chest imaging
  • Organ-specific parameters
• Monitor for treatment-related adverse effects:
  • Complete blood count
  • Hepatic and renal function
  • Bone density
  • Blood pressure
  • Serum glucose 1

Pitfalls and Caveats

1. Relapse rates range from 13-75% depending on disease stage, organ involvement, and other factors 2

2. Cardiac involvement may be asymptomatic but potentially fatal - screening is essential 5

3. Ocular involvement requires screening as it may be asymptomatic but can cause permanent vision impairment 5

4. Consider Pneumocystis prophylaxis in patients on high-dose immunosuppression (≥20 mg prednisone with cytotoxic agent for >6 months) 1

5. Treatment decisions should be multidisciplinary, especially for complex cases with multi-organ involvement 4