What is the treatment for sarcoidosis?

Treatment for Sarcoidosis

Glucocorticoids are the first-line treatment for symptomatic sarcoidosis with risk of mortality or permanent disability, with methotrexate recommended as the preferred second-line agent when glucocorticoids are ineffective or cause unacceptable side effects. 1

Treatment Algorithm Based on Disease Severity

Pulmonary Sarcoidosis

• For symptomatic pulmonary sarcoidosis with high risk of mortality or permanent disability:

  • Initial therapy: Prednisone 20 mg once daily for 2-6 weeks 1, 2
  • Maintenance: Taper to 5-10 mg daily or every other day 1, 2
  • Duration: Minimum 1 year of treatment unless no improvement after 3 months 3
  • Monitor: Bone density, blood pressure, serum glucose 1

• For patients with continued disease or unacceptable side effects from glucocorticoids:

  • Add methotrexate 10-15 mg once weekly to improve/preserve FVC and quality of life 1
  • Alternative second-line agents: azathioprine (50-250 mg daily), leflunomide (10-20 mg daily), or mycophenolate mofetil (500-1500 mg twice daily) 1, 4

• For patients who fail glucocorticoids and second-line agents:

  • Consider infliximab (3-5 mg/kg initially, 2 weeks later, then every 4-6 weeks) 1

• For patients with low risk of mortality/disability and minimal symptoms:

  • Observation for 2+ years is warranted before initiating treatment 3

Cardiac Sarcoidosis

• For patients with functional cardiac abnormalities (heart block, dysrhythmias, cardiomyopathy):
  • Strongly recommended to use glucocorticoids with or without other immunosuppressives 1
  • Consider early initiation of steroid-sparing medications due to significant morbidity of long-term glucocorticoid use 1

Neurosarcoidosis

• For clinically significant neurosarcoidosis:
  • First-line: Glucocorticoids 1
  • Second-line: Add methotrexate if disease continues despite glucocorticoids 1
  • Third-line: Add infliximab if disease continues despite glucocorticoids and second-line agents 1

Cutaneous Sarcoidosis

• For patients with continued cosmetically important active skin disease despite glucocorticoids/immunosuppressives:
  • Consider adding infliximab 1
  • Alternative: Hydroxychloroquine 200-400 mg once daily with periodic ocular exams 1

Sarcoidosis-Associated Fatigue

• First-line: Pulmonary rehabilitation program and/or inspiratory muscle strength training for 6-12 weeks 1, 5
• Second-line: Consider D-methylphenidate or armodafinil for 8 weeks to test effect on fatigue and tolerability 1, 5

Important Considerations

Monitoring Treatment Response

• Assess improvement in symptoms, pulmonary function tests, and radiographic findings 1, 2
• For cardiac sarcoidosis: Monitor left ventricular ejection fraction and brain natriuretic peptide 1
• For fatigue: Evaluate functional improvement with 6-minute walk test 5

Duration of Treatment

• Response to treatment for 3-6 months provides rationale for continuing therapy 1
• At least half of patients started on glucocorticoids remain on treatment 2 years later 1
• Continued low-dose prednisone (10-15 mg daily) helps prevent relapses 3
• Periodic attempts at tapering are justified, but repeated relapses may indicate need for life-long treatment 3

Treatment Pitfalls

• Inhaled glucocorticoids added to oral glucocorticoids do not provide significant benefits 1
• No data exist concerning mortality balance between benefits from long-term treatment and risks due to treatment-induced comorbidities 1
• Patients incorrectly labeled as "corticosteroid failures" may be subjected to other potentially toxic drugs unnecessarily 6
• Evidence for all fatigue interventions is of low quality with small sample sizes 5

Risk Stratification

• Treatment decisions should prioritize patients at risk for mortality or permanent disability 1
• For cardiac sarcoidosis, risk factors include age >50 years, left ventricular ejection fraction <40%, ventricular tachycardia, and cardiac inflammation on imaging 1
• For pulmonary sarcoidosis, risk factors include pulmonary hypertension, reduced lung function, and pulmonary fibrosis 1, 2

By following this evidence-based approach, treatment can be tailored to the specific organ involvement and disease severity, minimizing unnecessary medication exposure while preventing progression to irreversible organ damage.