American Board of Internal Medicine (ABIM) Certification Practice Exam

What is the role of azathioprine in the treatment of SSc?

1. First-line therapy for severe cases

2. Maintenance therapy following cyclophosphamide

3. Only used in combination with glucocorticoids

4. Recommended as initial therapy

The correct answer is: Maintenance therapy following cyclophosphamide

Azathioprine is often utilized as a maintenance therapy following cyclophosphamide in the treatment of systemic sclerosis (SSc). Cyclophosphamide is generally used as an aggressive first-line treatment for severe manifestations of SSc, particularly for interstitial lung disease. Once the more acute phase of treatment is completed and the patient shows improvement, azathioprine can be introduced to maintain disease control, allow for steroid-sparing effects, and minimize the risk of relapse. Azathioprine has immunosuppressive properties that help manage autoimmune manifestations and prevent further progression of the disease. Its role as a maintenance therapy is valued because it has a favorable long-term safety profile and can help manage disease activity more effectively than other agents in the maintenance setting. Other options do not accurately represent the established use of azathioprine in the treatment regimen of SSc. For instance, it is not recognized as first-line therapy for severe cases, nor is it exclusively used in combination with glucocorticoids. Additionally, while it may be considered as part of initial therapy in specific scenarios, it is primarily indicated for maintenance once a more intensive immunosuppressive regimen has been initiated.