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 symptomatic disease, particularly those at higher risk of future mortality or permanent disability from sarcoidosis 1.

Treatment Approach

The treatment approach for sarcoidosis depends on the severity of symptoms and the organs involved. For patients with pulmonary sarcoidosis, the use of glucocorticoids is recommended to improve and/or preserve forced vital capacity (FVC) and quality of life (QoL) 2.

• The initial treatment typically involves prednisone 20-40mg daily for 1-3 months, followed by a gradual taper over 6-12 months.
• In cases where steroid-sparing agents are required, methotrexate (10-25mg weekly), hydroxychloroquine (200-400mg daily), or azathioprine (50-200mg daily) may be used.
• For pulmonary sarcoidosis, inhaled corticosteroids like fluticasone or budesonide can help manage respiratory symptoms.

Biologics and Advanced Disease

Severe or refractory cases of sarcoidosis might require biologics such as infliximab (3-5mg/kg IV at weeks 0, 2, and 6, then every 4-8 weeks) 3.

• The decision to use biologics should be based on the presence of symptomatic disease and the risk of future mortality or permanent disability.
• Regular monitoring is essential, including pulmonary function tests, chest imaging, and organ-specific assessments depending on involvement.

Disease Management

Sarcoidosis results from an exaggerated immune response to unknown antigens in genetically susceptible individuals, with T-cell activation and cytokine production driving granuloma formation 4.

• While many patients experience disease resolution within 2-5 years, some develop chronic disease requiring long-term management.
• The treatment algorithm should consider escalation of care based on disease progression, weaning of prednisone to the lowest tolerable dose, and the use of biologics in advanced disease 5, 6.

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 7.
• 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 8.
• 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 8.
• 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 8.

Treatment of Sarcoidosis

• Corticosteroids are the cornerstone of sarcoidosis treatment and allow a disease remission but only with a suspensive effect 9.
• Half of the patients recover spontaneously without any treatment, while the remaining patients require treatment either at presentation or during follow-up in the presence of a disease flare 9.
• Treatment duration should be at least 12 months, and the main indications of systemic treatment include ophthalmologic, neurologic, cardiovascular, renal, laryngeal involvements, severe pulmonary manifestations, lupus pernio, and marked hypercalcaemia 9.
• Immunosuppressive drugs, such as methotrexate and azathioprine, may be useful in patients with failure or contraindication of corticosteroids or in those who need a long-term prednisone dose higher than 10 mg daily to control the sarcoidosis 9, 10.
• Infliximab can be useful in some severe sarcoidosis patients refractory to classical treatment, except for cardiac involvement 9, 11.

Second-Line and Third-Line Treatment Options

• Methotrexate and azathioprine are the most useful immunosuppressive drugs and 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 10.
• Other second-line agents, such as leflunomide and hydroxychloroquine, may be used, but the evidence supporting their use is limited 12.
• Third-line treatment options, including tumor necrosis factor-alpha inhibitors infliximab and adalimumab, are currently reserved for patients refractory to standard therapy 11, 12.

Treatment Protocols and Principles

• Pulmonary or extrapulmonary sarcoidosis should be treated with anti-inflammatory therapy if it significantly impairs the quality of life, causes significant organ dysfunction, or threatens to cause organ damage, disability, or death 11.
• If treatment is initiated for improving the quality of life alone, low-dose (10 mg/day) prednisone is a good initial treatment that can be tapered and stopped over 3 months 11.
• 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 11.