What are the guidelines for diagnosing and managing sarcoidosis (a systemic granulomatous disease)?

From the Guidelines

For patients with sarcoidosis, glucocorticoid treatment is recommended as the first-line treatment to improve and/or preserve forced vital capacity (FVC) and quality of life (QoL) in those with major involvement from pulmonary sarcoidosis believed to be at higher risk of future mortality or permanent disability from sarcoidosis. The treatment approach for sarcoidosis varies depending on the organ involved and the severity of the disease.

• For pulmonary sarcoidosis, glucocorticoid treatment is recommended as the first-line treatment 1.
• For patients with symptomatic pulmonary sarcoidosis who have been treated with glucocorticoids and have continued disease or unacceptable side-effects, methotrexate or infliximab may be added to improve and/or preserve FVC and QoL 1.
• For cutaneous sarcoidosis, oral glucocorticoids may be considered to reduce skin lesions in patients with cosmetically important active skin lesions which cannot be controlled by local treatment 1.
• For cardiac sarcoidosis, glucocorticoids with or without other immunosuppressives are recommended for patients with evidence of functional cardiac abnormalities 1.
• For neurosarcoidosis, glucocorticoids are recommended as the first-line treatment, and methotrexate or infliximab may be added for patients with continued disease 1.
• For sarcoidosis-associated fatigue, a pulmonary rehabilitation programme and/or inspiratory muscle strength training, or D-methylphenidate or armodafinil may be used to improve fatigue 1. The goal of treatment is to suppress the granulomatous inflammation characteristic of sarcoidosis and improve QoL. Regular monitoring is necessary to assess organ involvement and medication side effects. Treatment duration varies based on disease activity, and some patients may require lifelong therapy while others may achieve remission.

From the FDA Drug Label

Symptomatic sarcoidosis The drug prednisone is indicated for symptomatic sarcoidosis 2.

• The guidelines for sarcoidosis include the use of prednisone as a treatment option.
• Prednisone is used to manage symptomatic sarcoidosis.

From the Research

Treatment Guidelines for Sarcoidosis

• The treatment of sarcoidosis is dependent on nonspecific suppression of the immune response, with corticosteroids being the initial treatment of choice for most patients 3.
• Corticosteroids are usually effective for all forms of sarcoidosis and work quickly, but alternative medications often need to be considered due to potential toxicities 4.
• The main indications for systemic treatment include ophthalmologic, neurologic, cardiovascular, renal, laryngeal involvements, severe pulmonary manifestations, lupus pernio, and marked hypercalcaemia 5.
• Initial therapy is usually prednisone or a similar glucocorticoid, with a dose of 0.5 to 1 mg/kg daily for 6 to 12 weeks to obtain a complete remission 5.

Alternative Treatment Options

• Immunosuppressive drugs such as methotrexate, azathioprine, leflunomide, and mycophenolate have been reported as effective in sarcoidosis 6, 7.
• Biologic agents, especially monoclonal anti-tumor necrosis factor (anti-TNF) antibodies, have proved effective in patients who have failed other treatments 6, 7.
• Infliximab has been the most studied drug in this class and has been shown to be particularly effective for advanced disease 6, 7.
• Hydroxychloroquine and chloroquine are indicated in extensive skin lesions or as corticosteroids sparing agents 5.

Treatment Duration and Monitoring

• Treatment duration should be of at least 12 months, with a gradual dose reduction every 6 to 12 weeks 5.
• After treatment completion, a 36-month duration monitoring is warranted to confirm recovery 5.
• The assessment of response to disease includes functional studies such as the forced vital capacity, and radiologic imaging such as chest x-ray 6.