How is sarcoidosis treated?
Most patients with sarcoidosis do not require therapy. In patients with critical organ involvement, multiorgan disease, or symptoms that significantly affect quality of life, corticosteroids remain the first-line treatment. The exact dosing strategy that balances the desired effect and side effects of corticosteroids is less clear. Oral corticosteroids (up to 1 mg/kg per day) are used as first-line treatment for symptomatic and progressive stage II and III lung disease, malignant hypercalcemia, and severe ocular, neurologic, cardiac, and skin involvement. The dose is typically tapered to 10 mg/day by 6 months and maintained on 10 mg/day for an additional 6 months before tapering further. If the patient continues to have progressive disease, or cannot taper the prednisone to <10 mg daily, additional immunosuppressive therapy is indicated. Patients with severe cardiac or neurologic involvement may benefit from additional immunosuppressive medication when corticosteroids are started initially. Methotrexate at doses of 15 to 20 mg weekly is the most commonly used steroid-sparing agent for both pulmonary and extrapulmonary involvement. Azathioprine (2 mg/kg per day), leflunomide (10–20 mg/day) and mycophenolate mofetil (1000–15000 mg twice a day) have also been reported as effective. Failure to respond to at least one immunosuppressive/disease-modifying antirheumatic drug plus prednisone is an indication for treatment with anti-TNF-α agents, which can be effective against infiltrating noncaseating granuloma. Not all anti-TNF-α agents are equally effective in sarcoidosis. Infliximab (5 mg/kg every 4–6 weeks) or adalimumab (40 mg subcutaneous weekly) is recommended. Etanercept appears to have limited benefit. Chloroquine/hydroxychloroquine has been used for cutaneous or musculoskeletal involvement. Cyclophosphamide (oral and intravenous) has shown benefit in patients with cardiac or neurosarcoidosis. In case reports, rituximab has been used successfully for refractory disease particularly when granulomatous mass lesions are present. Implantable pacemakers are inserted in patients with cardiac involvement and arrhythmias or heart block. Solid organ transplantation can be life-saving in patients who have failed all medical therapies.
Patients must be monitored for side effects and prophylactic measures used to prevent toxicities. Patients on high-dose corticosteroids should receive prophylaxis against Pneumocystis jirovecii . Osteopenia and osteoporosis due to dysregulated calcium metabolism and medications used in therapy can occur in up to two-thirds of patients (see Question 17). Vaccinations should be kept up to date. Depression is common (46%–66%) and should be screened for and treated. Fatigue is common (50%–70%), can be disabling, and may respond to armodafinil or dexmethylphenidate. Hypothyroidism, hypoxemia, depression, and sleep apnea are potential and correctable causes of fatigue.
