What is the diagnosis and treatment for Sarcoidosis?

From the Guidelines

Sarcoidosis treatment should prioritize corticosteroids as the first-line therapy, with a strong recommendation for their use in patients with major involvement from pulmonary sarcoidosis believed to be at higher risk of future mortality or permanent disability from sarcoidosis, as stated in the European Respiratory Journal guidelines 1. The treatment approach for sarcoidosis depends on the severity of symptoms and the organs involved.

• For mild cases with minimal symptoms, a watchful waiting approach may be appropriate, as the condition can resolve spontaneously in 30-60% of patients.
• For those requiring treatment, corticosteroids, typically starting with prednisone 20-40mg daily for 1-3 months, followed by a gradual taper over 6-12 months, remain the first-line therapy.
• In cases where steroids are ineffective or contraindicated, immunosuppressive medications like methotrexate (10-25mg weekly), azathioprine (50-200mg daily), or hydroxychloroquine (200-400mg daily) may be used.
• For refractory cases, TNF-alpha inhibitors such as infliximab (3-5mg/kg IV at weeks 0,2, and 6, then every 8 weeks) have shown efficacy, as suggested by the Delphi consensus recommendations for a treatment algorithm in pulmonary sarcoidosis 1. The disease course varies widely, with some patients experiencing complete resolution while others develop chronic disease requiring long-term management.
• Regular monitoring of pulmonary function, cardiac status, and other affected organs is essential, as is screening for complications like hypercalcemia and ocular involvement.
• The exact cause of sarcoidosis remains unknown, but it likely results from an exaggerated immune response to environmental triggers in genetically susceptible individuals, as noted in the European Respiratory Journal guidelines 1 and the Delphi consensus recommendations 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

• Pulmonary or extrapulmonary sarcoidosis should be treated with anti-inflammatory therapy if it significantly impairs the quality of life (QoL), causes significant organ dysfunction, or threatens to cause organ damage, disability, or death 5.
• Corticosteroids are the initial treatment for active disease, with refractory cases often requiring immunosuppressive or biologic therapies 5, 6.
• If treatment is initiated for improving the QoL alone, low-dose (10 mg/day) prednisone is a good initial treatment that can be tapered and stopped over 3 months 5.
• Disease that causes significant organ dysfunction needs to be treated with medium-dose glucocorticoids (initial daily dose, 20 mg of prednisone equivalent) tapered over a minimum duration of 6 months 5.

Second-Line Therapy for Sarcoidosis

• Methotrexate and azathioprine have significant steroid-sparing potency, a similar positive effect on lung function, and comparable side effects, except for a higher infection rate in the azathioprine group 7.
• If a daily prednisone dose of >10 mg is required for >6 months to maintain remission, it is best to use a second-line drug such as methotrexate or azathioprine 5.
• Anti-tumor necrosis factor agents, such as infliximab or adalimumab, may be used to treat inflammatory disease that persists on combination treatment with glucocorticoids and a second-line agent 5.

Monitoring and Follow-Up

• Effective regular follow-up is necessary to monitor changes in the disease, including extension, progression, remissions, flare-ups, and complications 4.
• A multidisciplinary approach is recommended for best outcomes, as sarcoidosis is a systemic disease 4.
• Whether or not treatment is started, regular follow-up is necessary to assess organ involvement/impairment and to establish whether the patient is likely to benefit from treatment 4.