What is the diagnosis and treatment for Sarcoidosis?

From the Guidelines

Sarcoidosis patients with symptomatic disease should be treated with corticosteroids as the first-line treatment, typically starting with prednisone 20-40mg daily for 1-3 months, followed by a gradual taper over 6-12 months, as recommended by the European Respiratory Society clinical practice guidelines 1. The decision to treat sarcoidosis depends on two major factors: risk for death or organ failure and impairment of quality of life (QoL) 1. Pulmonary and cardiac disease are the most common reasons for death from sarcoidosis, and irreversible organ damage to the brain, eyes, or kidneys can also cause significant morbidity 1. Some key points to consider in the treatment of sarcoidosis include:

• The use of anti-inflammatory therapy for less severe but impaired patients may prevent progression to irreversible disease 1
• Sarcoidosis-associated fatigue (SAF) and small-fibre neuropathy (SFN)-associated symptoms are encountered in a significant number of sarcoidosis patients and treatment is a high priority for these patients 1
• The treatment algorithm for symptomatic or organ-threatening sarcoidosis derived from the Delphi consensus recommendations suggests the use of corticosteroids, antimetabolites, and biologic agents such as infliximab or adalimumab in a step-wise approach 1
• Methotrexate is the most widely studied drug for sarcoidosis and is recommended as a second-line agent for patients who cannot tolerate steroids or have refractory disease 1
• Biologic agents such as infliximab are reserved for severe, treatment-resistant cases and have multiple clinical trials supporting their use in various manifestations of sarcoidosis 1. Regular pulmonary function tests and chest imaging are essential to monitor disease progression in pulmonary sarcoidosis patients 1. The exact cause of sarcoidosis remains unknown, but it likely results from an exaggerated immune response to an unidentified antigen in genetically susceptible individuals 1. The disease often resolves spontaneously within 2-5 years, but some patients develop chronic disease requiring long-term management 1. In terms of specific treatment regimens, the European Respiratory Society clinical practice guidelines recommend the use of corticosteroids as the first-line treatment, with immunosuppressive agents and biologic agents used in a step-wise approach for patients who cannot tolerate steroids or have refractory disease 1. Overall, the treatment of sarcoidosis should be individualized based on symptom severity and organ involvement, with a focus on preventing progression to irreversible disease and improving quality of life.

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 are the initial treatment for active disease, with refractory cases often requiring immunosuppressive or biologic therapies 3, 4.
• Methotrexate is generally the preferred agent for treatment of chronic sarcoidosis when corticosteroids have inadequate efficacy and/or severe adverse effects 5.
• Azathioprine has been shown to have significant steroid-sparing potency and a similar positive effect on lung function as methotrexate, but with a higher infection rate 6.
• Treatment patterns in the United States vary, with 42.5% of patients receiving treatment within 1 year of diagnosis, and 40% of treated patients receiving nonsteroidal immunosuppressives 7.

Prognosis and Complications

• Prognosis is variable and depends on epidemiologic factors, mode of onset, initial clinical course, and specific organ involvement 3.
• Despite aggressive treatment, some patients may develop life-threatening pulmonary, cardiac, or neurologic complications from severe, progressive disease 3.
• End-stage disease may ultimately require lung or heart transplantation for eligible patients 3, 4.