What is the management of sarcoidosis?

Management of Sarcoidosis

Corticosteroids remain the first-line therapy for symptomatic sarcoidosis, with methotrexate as the preferred second-line agent and infliximab as the recommended biologic for refractory cases. 1

Treatment Decision Framework

Treatment decisions in sarcoidosis should be based on:

1. Risk of mortality or organ failure - Pulmonary and cardiac disease are the most common causes of death from sarcoidosis 1
2. Quality of life impairment - Including fatigue and other symptomatic manifestations 1
3. Organ involvement pattern - Different organs may require specific treatment approaches 1

Treatment Algorithm by Disease Phenotype

Acute Phenotype (Newly Diagnosed Symptomatic Disease)

• First-line therapy: Prednisone 20-40 mg daily for 3-6 months 1
• For hypercalcemia or skin disease: Consider adding hydroxychloroquine 1
• If disease progression or toxicity occurs: Add methotrexate as a steroid-sparing agent 1

Chronic Phenotype (Persistent Disease)

• Add methotrexate if unable to taper corticosteroids or experiencing steroid toxicity 1
• If disease progression or toxicity continues: Add anti-TNF-α therapy, preferably infliximab 1
• Goal: Taper corticosteroids to lowest effective dose while continuing biologics for 2-3 years 1

Advanced Phenotype (Refractory Disease)

• Consider repository corticotropin injection or CLEAR therapy (concomitant levofloxacin, ethambutol, azithromycin, and rifampin) for cases that fail standard therapy 1
• Goal: Taper steroids (discontinue if possible) and maintain therapy at lowest effective dose 1

Specific Organ Involvement Management

Pulmonary Sarcoidosis

• For symptomatic disease with risk of mortality/disability: Strong recommendation for glucocorticoid treatment 1
• For patients on glucocorticoids with continued disease: Add methotrexate to preserve lung function and quality of life 1
• For refractory pulmonary disease: Add infliximab 1

Cutaneous Sarcoidosis

• For cosmetically important lesions uncontrolled by local treatment: Consider oral glucocorticoids 1
• Monitor for: Progressive radiographic changes, persistent symptoms, lung function deterioration, or critical extrapulmonary involvement 1

Sarcoidosis-Associated Fatigue

• Consider non-pharmacological approaches first 1
• For troublesome fatigue: Pharmacological interventions may be necessary 1

Medication Specifics

Corticosteroids

• Initial dose: Prednisone 20-40 mg daily 1, 2
• Duration: Minimum 3-6 months, then taper over 4-8 weeks if improvement occurs 1
• Maintenance: Aim for lowest effective dose (≤10 mg daily) 1
• Caution: Monitor for steroid-induced complications including weight gain and reduced quality of life 1
• Prophylaxis: Consider PCP prophylaxis for patients receiving ≥20 mg methylprednisolone or equivalent for ≥4 weeks; calcium and vitamin D supplementation; proton pump inhibitor therapy 1

Methotrexate (Second-line)

• Indication: Steroid-sparing agent for chronic disease or when steroids are not tolerated 1
• Evidence: Most widely studied cytotoxic agent for sarcoidosis 1
• Monitoring: Regular laboratory monitoring for toxicity 1

Anti-TNF Therapy (Third-line)

• Preferred agent: Infliximab has the strongest evidence 1
• Indication: For patients who have failed corticosteroids and methotrexate 1
• Precaution: T-spot testing should be performed to exclude tuberculosis prior to starting anti-TNF therapy 1
• Duration: Continue biologics for 2-3 years in chronic disease 1

Common Pitfalls and Caveats

• Not all patients require treatment - Nearly half of sarcoidosis patients never need systemic therapy 1
• Asymptomatic disease - Little evidence that corticosteroid treatment changes the natural course in asymptomatic pulmonary sarcoidosis 1
• Relapse risk - Relapse of symptomatic disease occurs in a significant number of patients (20-80%) upon withdrawal of therapy after 1-2 years 1
• Treatment re-evaluation - Need for continued treatment should be re-evaluated every 1-2 years 1
• Ocular involvement - Limited specific evidence for treatment of ocular sarcoidosis, though adalimumab has been used for non-infectious uveitis 1
• Transplantation - May be considered for advanced pulmonary or cardiac disease, though recurrence in the allograft can occur 1

Treatment Response Assessment

• Pulmonary function tests: Monitor FVC and DLCO 1
• Radiographic changes: Follow chest imaging 1
• Symptom improvement: Track quality of life and symptom resolution 1
• Consider discontinuation of treatment if disease has been stable for 2-3 years 1

Bold text indicates the most important recommendations based on the strongest evidence.