From the Guidelines
Sarcoidosis patients with symptomatic disease should be treated with glucocorticoids as the first-line treatment, with the goal of improving and/or preserving forced vital capacity (FVC) and quality of life (QoL) 1.
Treatment Approach
The treatment approach for sarcoidosis depends on the severity of symptoms and organ involvement. For mild cases with minimal symptoms, a watchful waiting approach may be appropriate. However, for symptomatic patients, corticosteroids are the first-line treatment, typically starting with prednisone 20-40mg daily for 1-3 months, followed by a gradual taper over 6-12 months 1.
Second-Line Options
For patients who cannot tolerate steroids or have refractory disease, second-line options include:
- Methotrexate (10-25mg weekly)
- Hydroxychloroquine (200-400mg daily)
- Azathioprine (50-200mg daily) 1
Severe or Progressive Cases
Severe or progressive cases may require combination therapy or biologics like infliximab (3-5mg/kg IV every 4-8 weeks) 1.
Monitoring and Evaluation
Pulmonary sarcoidosis patients should undergo regular pulmonary function tests and chest imaging to monitor disease progression. Cardiac involvement requires specialized evaluation including ECG, echocardiogram, and possibly cardiac MRI. Neurosarcoidosis may necessitate more aggressive immunosuppression 1.
Disease Management
The disease results from an exaggerated immune response to unknown antigens in genetically susceptible individuals, with CD4+ T cells and macrophages playing central roles in granuloma formation. While many patients experience disease resolution within 2-5 years, about 30% develop chronic disease requiring long-term management 1.
Fatigue and Small-Fibre Neuropathy
For patients with sarcoidosis-associated fatigue, a pulmonary rehabilitation programme and/or inspiratory muscle strength training for 6–12 weeks may be beneficial in improving fatigue 1. Additionally, D-methylphenidate or armodafinil may be considered for 8 weeks to test its effect on fatigue and tolerability 1. However, no recommendations were made for the treatment of small-fibre neuropathy due to a lack of sufficient evidence 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, 4.
Treatment of Sarcoidosis
- Corticosteroids are the first-choice therapeutic in sarcoidosis, but long-term use is associated with toxicity 5.
- Methotrexate and azathioprine are commonly used as second-line agents in sarcoidosis, with significant steroid-sparing potency and a similar positive effect on lung function 5, 6, 7.
- The optimal treatment for sarcoidosis remains unclear, but corticosteroid therapy has been the mainstay of therapy for those with significantly symptomatic or progressive pulmonary disease or serious extrapulmonary disease 3, 4.
- Refractory or complex cases may require immunosuppressive therapy, and transplantation can be considered for advanced and end-stage disease depending on organ involvement 3, 4.
Management and Prognosis
- Prognosis is variable and depends on epidemiologic factors, mode of onset, initial clinical course, and specific organ involvement 3.
- An early and accurate diagnosis of sarcoidosis remains challenging, because initial presentations may vary, many patients are asymptomatic, and there is no single reliable diagnostic test 3, 4.
- 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 4.