What is the first line treatment for sarcoidosis?

First-Line Treatment for Sarcoidosis

Oral glucocorticoids (prednisone) are the first-line treatment for symptomatic sarcoidosis, typically starting at a dose of 20-40 mg daily unless contraindicated. 1, 2

When to Initiate Treatment

Not all patients with sarcoidosis require treatment. Treatment decisions should be based on:

• Presence of symptoms (cough, dyspnea, chest pain) 2, 3
• Parenchymal infiltrates on imaging 2
• Abnormal pulmonary function tests 2, 3
• Risk of mortality or permanent disability 1
• Significant impairment of quality of life 1

Nearly half of sarcoidosis patients never require systemic treatment, as the disease may resolve spontaneously 1.

Initial Treatment Protocol

Dosing

• Standard initial dose: 20-40 mg prednisone daily 1, 2, 3
• Duration of initial dose: 2 weeks to 2 months 2
• Dose reductions may be needed for patients with:
  • Diabetes 1
  • Psychosis 1
  • Osteoporosis 1

Treatment Duration and Monitoring

• Allow 3-6 months to assess therapeutic response 1
• Follow-up interval of 3-6 months after steroid initiation 1
• If improvement occurs, taper prednisone over 6-18 months 2, 3
• Goal: reduce to lowest dose that provides satisfactory symptom relief and disease control 1

Adjusting Treatment Based on Response

For Improvement

• Gradually decrease steroid dose to the lowest effective dose 1
• Continue tapering with goal of eventual discontinuation if possible 1

For Stable Disease

• Decrease steroid dose to find the lowest effective dose 1

For Worsening Disease

• Add adjunctive therapy 1
• Re-evaluate the diagnosis and treatment plan 1

When to Consider Second-Line Agents

Consider adding steroid-sparing agents when:

• High risk for steroid toxicity exists 1
• Long-duration therapy is anticipated 1
• Inadequate response to steroid therapy occurs 1
• Systemic/extrapulmonary involvement is present 1
• Patient requires prolonged prednisone ≥10 mg/day 2, 3
• Adverse effects from glucocorticoids develop 2, 3

Second-Line Agents

• Methotrexate is the preferred second-line agent 1, 4
• Other options include azathioprine and mycophenolate mofetil 4

Special Considerations

Inhaled Corticosteroids

• May be appropriate for symptomatic relief of:
  • Cough 1
  • Asthma-like symptoms 1
• Should be discontinued if ineffective or if toxicities develop 1
• Not effective as monotherapy for pulmonary sarcoidosis 1

Mild Disease

• No consensus exists on using steroid-sparing therapies for mild disease 1
• For patients not at risk for morbidity/mortality and without significant quality of life impairment, no glucocorticoid treatment may be preferred 1

Common Pitfalls and Caveats

• Prolonged use of even low-dose prednisone can lead to significant toxicity including weight gain and reduced quality of life 1
• Lack of response over 3-6 months suggests need for alternative treatment strategy 1
• Patients may be inappropriately labeled as "corticosteroid failures" and subjected to other potentially toxic drugs 5
• At least half of patients started on glucocorticoids may still be on treatment 2 years later 1
• Relapse rates range from 13% to 75% depending on disease stage, number of organs involved, and other factors 2

Disease Progression and Mortality Risk

• Approximately 10-40% of patients with sarcoidosis develop progressive pulmonary disease 2, 3
• Overall mortality rate for sarcoidosis is approximately 7% within a 5-year follow-up period 2, 3
• More than 60% of deaths from sarcoidosis are due to advanced pulmonary disease 2, 3