Treatment of Sarcoidosis with Multiple Organ Involvement

Introduction to Treatment Approach

The European Respiratory Society recommends a stepped treatment approach for sarcoidosis with multiple organ involvement, starting with prednisone 20-40 mg daily for 3-6 months, followed by addition of methotrexate as a second-line agent if disease continues or steroid side effects become unacceptable, and infliximab as a third-line agent for refractory cases 1, 2, 3

Prednisone 20-40 mg daily is the preferred initial treatment for symptomatic sarcoidosis with multiple organ involvement, allowing 3-6 months to demonstrate responsiveness 1, 2
The reported rate of disease relapse upon glucocorticoid withdrawal after 2 years of initial therapy ranges from 20% to 80% 2, 5
Prolonged prednisone may be required to stabilize disease, with at least half of patients remaining on treatment 2 years later 1, 4

Add methotrexate (10-15 mg weekly) to prednisone if there is disease progression despite adequate glucocorticoid treatment or unacceptable side effects from glucocorticoids 1, 2, 3
Alternative second-line agents include azathioprine, mycophenolate mofetil, and leflunomide, although the evidence for these is not as strong 1, 4

Add infliximab for patients with continued disease despite glucocorticoids and second-line agents 1, 2, 3
Discontinuation of infliximab after 6-12 months is associated with disease relapse in more than half of cases 2, 5

For cardiac sarcoidosis, glucocorticoids with or without immunosuppressives are strongly recommended for patients with functional cardiac abnormalities 4
For neurosarcoidosis, first-line treatment is glucocorticoids, with methotrexate as second-line and infliximab as third-line 4
For pituitary sarcoidosis, high-dose corticosteroids are used in about 90% of patients, with steroid-sparing therapy subsequently used in 57% 6
For cutaneous sarcoidosis, oral glucocorticoids are suggested for cosmetically important active skin lesions not controlled by local treatment 3
Hydroxychloroquine 200-400 mg once daily with periodic ocular exams can be considered as an alternative treatment for cutaneous sarcoidosis 4

Continue therapy for at least 3-6 months if there is improvement in symptoms, pulmonary function tests, and radiographic findings 4
Re-evaluate the need for continued successful treatment every 1-2 years 2, 5