What is the treatment approach for sarcoidosis?

Treatment Approach for Sarcoidosis

Systemic glucocorticoids are the first-line treatment for symptomatic pulmonary sarcoidosis and other organ-threatening manifestations, with methotrexate as the preferred second-line agent when steroids fail or cause unacceptable side effects. 1

Treatment Decision Algorithm

Step 1: Determine Need for Treatment

• No treatment needed if:

  • Asymptomatic disease
  • No evidence of organ dysfunction
  • No significant impairment of quality of life 1

• Treatment indicated for:

  • Symptomatic pulmonary disease with dyspnea, cough, or chest pain
  • Higher risk of mortality or permanent disability
  • Significant impairment of quality of life
  • Specific organ involvement:
    • Cardiac: heart block, dysrhythmias, cardiomyopathy
    • Neurological: clinically significant neurosarcoidosis
    • Cutaneous: cosmetically important active skin lesions 1

Step 2: First-Line Treatment

• Oral glucocorticoids:
  • Initial dose: Prednisone 20-40 mg daily for 3-6 months 1
  • For pulmonary disease: 20 mg prednisone daily 1
  • For cardiac sarcoidosis: Consider higher doses (strong recommendation) 1
  • For neurosarcoidosis: Glucocorticoids are strongly recommended 1

Step 3: Monitoring and Dose Adjustment

• Evaluate response after 3-6 months
• If responding: Taper to lowest effective dose (5-10 mg daily) 1
• Monitor for:
  • Bone density
  • Blood pressure
  • Serum glucose
  • Weight gain
  • Cataracts/glaucoma 1

Step 4: Second-Line Treatment (if needed)

• Add methotrexate when:
  • Disease progression despite glucocorticoids
  • Inability to taper prednisone below 10 mg daily
  • Unacceptable steroid side effects 1
• Methotrexate dosing:
  • 10-15 mg once weekly
  • Monitor CBC, hepatic and renal function 1

Step 5: Third-Line Treatment (for refractory disease)

• Add infliximab when:
  • Continued disease despite glucocorticoids and methotrexate
  • Advanced or progressive disease 1, 2
• Infliximab approach:
  • Requires negative TB test before initiation
  • Continue for 2-3 years in responders
  • Evaluate response after completing induction (after week 6) 2

Organ-Specific Considerations

Cardiac Sarcoidosis

• Strong recommendation for glucocorticoids for patients with functional cardiac abnormalities 1, 2
• Consider early addition of steroid-sparing agents 1
• Infliximab strongly recommended for cardiac sarcoidosis failing first-line therapy 2
• Monitor for prognostic variables:
  • Left ventricular ejection fraction <40%
  • Ventricular tachycardia
  • Interventricular septal thinning 1

Neurosarcoidosis

• Strong recommendation for glucocorticoids 1
• Add methotrexate if continued disease
• Consider infliximab for refractory cases 1

Cutaneous Sarcoidosis

• Consider oral glucocorticoids for cosmetically important lesions
• Add infliximab for refractory skin disease 1

Sarcoidosis-Associated Fatigue

• Consider pulmonary rehabilitation program
• For non-disease activity related fatigue, consider D-methylphenidate or armodafinil 1

Treatment Challenges and Pitfalls

• Avoid prolonged glucocorticoid monotherapy due to cumulative toxicity 1
• Beware of inhaled glucocorticoids ineffectiveness - studies show no significant benefit 1
• Monitor for steroid-induced complications including diabetes, hypertension, weight gain, osteoporosis 1, 3
• Consider mortality risk - pulmonary sarcoidosis has approximately 7% mortality within 5 years, with higher rates for cardiac involvement 3
• Recognize when to escalate therapy - progressive disease despite treatment requires prompt escalation 1

The treatment approach should be guided by disease phenotype (acute, chronic, or advanced), with appropriate escalation of therapy when indicated to improve outcomes related to morbidity, mortality, and quality of life 1.