The majority of patients will have spontaneous remission and a generally benign clinical course. Treatment is reserved for patients with worsening pulmonary function tests or worsening pulmonary symptoms (cough, shortness of breath, chest pain or hemoptysis) and patients with extrapulmonary sarcoidosis including arthritis, neuropathy, cardiac and renal sarcoid. It is also initiated when the patient has intractable fatigue, weakness or fevers.
According to the American Thoracic Society, the first line of treatment is corticosteroids. Oral prednisone is given in a slow taper for a total of 6-12 months. The dose of prednisone recommended is 0.5-1mg/kg daily for the first 4-6 weeks. A reassessment of symptoms is done at that time and thereafter every 4-6 weeks. If the patient is improved, the dose is decreased by 5-10 mg until a daily maintenance dose of 10-15 mg is reached. This is given for the remainder of the 6-12 months to prevent relapse of disease.
These therapies are reserved for patients that cannot tolerate or have not responded to corticosteroids.
Methotrexate is a drug with both immunosuppressive and antiinflammatory properties and has been demonstrated in studies to suppress the disease in 40-60% of patients. Oral methotrexate is started at a dose of 7.5mg weekly and is gradually increased by 2.5mg every 2 weeks until a dose of 10-15mg is achieved. Therapy should be continued for at least 4-6 months to access relief of symptoms. Blood tests to monitor white blood cell count and liver function should be taken monthly.
Anti-TNF agents work to block Tumor Necrosis Factor Alpha, which is thought to accelerate the inflammatory process in sarcoidosis. They have been studied in patients with sarcoidosis non-responsive to corticosteroids. There are several formulations available but the only one shown in a small study to be effective is Infliximab (Remicade). Infliximab is a chimeric human-mouse antihuman antibody that specifically blocks TNF-alpha. It is given through intravenous infusion at a dose of 5mg/kg initially then at 2, 4, 6-8 and 12 weeks with doses given every 2 months thereafter. Infliximab is to be used in conjunction with small doses of methotrexate or prednisone to prevent the formation of antibodies to Infliximab. Duration of treatment is not known and may be indefinite.
Lung transplantation is considered for end-stage pulmonary fibrosis from sarcoidosis. Patients with extensive extrapulmonary sarcoidosis are generally excluded from this treatment option. Although evidence of sarcoidosis is seen in the transplanted lung, clinically significant disease is not.
|Terrence C.Demos, M.D. Patrick J. Fahey, M.D.||
Last Updated: March 14, 1996
Created: March 1, 1996