From the Guidelines
Sarcoidosis treatment should prioritize corticosteroids as the first-line therapy, with methotrexate or TNF-alpha inhibitors considered for patients who cannot tolerate steroids or require steroid-sparing agents, as recommended by the European Respiratory Journal in 2021 1.
Treatment Approach
The treatment approach for sarcoidosis depends on symptom severity and organ involvement. For mild cases with minimal symptoms, a watchful waiting approach may be appropriate as 30-60% of cases resolve spontaneously within 2-5 years.
- When treatment is necessary, corticosteroids are the first-line therapy, typically starting with prednisone 20-40mg daily for 1-3 months, followed by a gradual taper over 6-12 months.
- For patients who cannot tolerate steroids or require steroid-sparing agents, methotrexate (10-25mg weekly) or TNF-alpha inhibitors like infliximab may be used.
Monitoring and Multidisciplinary Approach
- Pulmonary function tests and chest imaging should be monitored regularly to assess disease progression.
- Calcium levels should also be monitored as sarcoidosis can cause hypercalcemia due to increased vitamin D production by activated macrophages in granulomas.
- Patients should be evaluated by specialists based on organ involvement, such as pulmonologists for lung disease or ophthalmologists for ocular manifestations, as sarcoidosis management often requires a multidisciplinary approach tailored to the specific manifestations and severity in each individual.
Recent Guidelines
The European Respiratory Journal published guidelines in 2021, which recommend the use of glucocorticoids for patients with pulmonary sarcoidosis who are at higher risk of future mortality or permanent disability from sarcoidosis 1. The guidelines also suggest the addition of methotrexate or infliximab for patients who have continued disease or unacceptable side-effects from glucocorticoids.
- A Delphi consensus study published in the European Respiratory Review in 2020 also recommended a treatment algorithm for pulmonary sarcoidosis, which includes the use of glucocorticoids, methotrexate, and biologic therapies such as infliximab 1.
From the FDA Drug Label
Symptomatic sarcoidosis
- Sarcoidosis is listed as one of the conditions for which prednisone is indicated.
- The FDA drug label indicates that prednisone is used for symptomatic sarcoidosis 2.
- This suggests that prednisone can be used to manage symptoms of sarcoidosis.
From the Research
Definition and Diagnosis of Sarcoidosis
- Sarcoidosis is a systemic disease of unknown etiology characterized by the presence of noncaseating granulomas in any organ, most commonly the lungs and intrathoracic lymph nodes 3.
- A diagnosis of sarcoidosis should be suspected in any young or middle-aged adult presenting with unexplained cough, shortness of breath, or constitutional symptoms, especially among blacks or Scandinavians 3.
- Diagnosis relies on three criteria: (1) a compatible clinical and radiologic presentation, (2) pathologic evidence of noncaseating granulomas, and (3) exclusion of other diseases with similar findings, such as infections or malignancy 3.
Treatment of Sarcoidosis
- Corticosteroids remain the initial treatment of choice for most patients with sarcoidosis 4, 3, 5.
- Second-line therapy includes cytotoxic agents such as methotrexate and azathioprine, which have been shown to have significant steroid-sparing potency and a positive effect on lung function 6, 4, 7.
- Immunosuppressives such as leflunomide and mycophenolate have also been reported as effective in sarcoidosis 4.
- Biologics and other agents are third-line therapy, with monoclonal antibodies directed against tumor necrosis factor having been shown to be particularly effective for advanced disease 4.
Management and Prognosis
- Prognosis is variable and depends on epidemiologic factors, mode of onset, initial clinical course, and specific organ involvement 3.
- Refractory or complex cases may require immunosuppressive therapy, and end-stage disease may ultimately require lung or heart transplantation for eligible patients 3, 5.
- Ongoing dyspnea and dry cough in a young to middle-aged adult should increase the suspicion for sarcoidosis, and symptoms can present at any age and affect any organ system 5.