What is the initial treatment for sarcoidosis?

From the Guidelines

The initial treatment for sarcoidosis often involves oral corticosteroids as the first-line therapy, typically prednisone starting at 20-40 mg daily for 3-6 months, as recommended by the Delphi consensus recommendations 1. For symptomatic patients or those with significant organ involvement, the dosage and duration of corticosteroids depend on disease severity and response to treatment.

• Topical corticosteroids may be used for localized skin or eye involvement.
• For patients who cannot tolerate or respond inadequately to corticosteroids, second-line options include methotrexate (10-25 mg weekly) or other immunosuppressants, as suggested by the European Respiratory Society clinical practice guidelines 1. Treatment decisions should be guided by which organs are affected, symptom severity, and disease progression.
• Regular monitoring of pulmonary function, blood work, and affected organ systems is essential during treatment. The goal of treatment is to lower the morbidity and mortality risk or to improve quality of life (QoL), as stated in the European Respiratory Journal 1. Corticosteroids work by suppressing the granulomatous inflammation characteristic of sarcoidosis, reducing the immune response that causes tissue damage. However, prolonged use of corticosteroids is associated with significant toxicity, and glucocorticoid-sparing alternatives are available, as noted in the Delphi consensus recommendations 1. In patients with pulmonary sarcoidosis, the introduction of glucocorticoid treatment is recommended to improve and/or preserve forced vital capacity (FVC) and QoL, as suggested by the European Respiratory Society clinical practice guidelines 1. For patients with symptomatic pulmonary sarcoidosis who have been treated with glucocorticoids and have continued disease or unacceptable side-effects, the addition of methotrexate is suggested to improve and/or preserve FVC and QoL, as recommended by the European Respiratory Society clinical practice guidelines 1.

From the FDA Drug Label

Symptomatic sarcoidosis The initial treatment for sarcoidosis is prednisone (PO), as it is indicated for symptomatic sarcoidosis 2.

• The drug label does not provide further information on the specific dosage or treatment duration.
• Prednisone is used as an initial treatment to manage symptoms of sarcoidosis.

From the Research

Initial Treatment for Sarcoidosis

The initial treatment for sarcoidosis typically involves the use of glucocorticosteroids, which are considered the "drugs of choice" for this condition 3.

• The recommended initial dose of prednisolone is 30-60 mg/day or its equivalent 3.
• Daily therapy with oral steroids is the most widely applied method of administration 3.
• Alternate day therapy can be used during the maintenance phase 3.
• Inhaled steroids can also be tried during the maintenance phase for treatment of pulmonary sarcoidosis 3.

Alternative Treatment Options

Other drugs that may be effective in sarcoidosis and have a steroid-sparing capacity include:

• Methotrexate 3, 4
• Azathioprine 3, 4
• Chlorambucil 3
• Cyclophosphamide 3
• Chloroquine can be used for chronic skin lesions 3
• Potassium para-aminobenzoate may soften fibrotic lesions and keloids 3

Treatment Duration and Monitoring

The duration of treatment varies with the clinical situation, ranging from 6 to 18 months to lifetime 3.

• Continuing signs of disease activity and functional impairment require continuing treatment 3.
• Symptomatic patients with stage II-III pulmonary sarcoidosis, and many extrapulmonary manifestations of the disease, must be adequately treated 3.
• Symptom-free patients with deteriorating lung function and/or biochemical signs of disease activity also require treatment 3.

Cardiac Sarcoidosis Treatment

For cardiac sarcoidosis, first-line therapy is usually with prednisone, based on clinician experience, expert opinion, and small observational cohorts 5.

• A low-dose prednisone/methotrexate combination may have non-inferior efficacy to standard-dose prednisone and result in significantly better quality of life 5.

Corticosteroid Therapy Phases

Corticosteroid therapy for pulmonary sarcoidosis involves six phases:

1. Initial high doses to control inflammation
2. Tapering to a maintenance dose
3. Continuing to receive the maintenance dose
4. Tapering off corticosteroid therapy
5. Observation for relapse
6. Treatment if relapse occurs 6

Comparison of Treatment Options

A randomized trial (SARCORT trial) compared high-dose (40 mg) versus low-dose (20 mg) prednisolone for treating sarcoidosis and found that high-dose prednisolone was not superior to a lower dose in improving outcomes or health-related quality of life 7.