American Board of Internal Medicine (ABIM) Certification Practice Exam

What is the recommended treatment for primary angiitis of the central nervous system?

1. Cyclophosphamide and low-dose glucocorticoids

2. High-dose glucocorticoids and methotrexate

3. Cyclophosphamide and high-dose glucocorticoids

4. Glucocorticoids alone

The correct answer is: Cyclophosphamide and high-dose glucocorticoids

The recommended treatment for primary angiitis of the central nervous system often includes a combination of high-dose glucocorticoids and cyclophosphamide. This approach is aimed at effectively suppressing the inflammatory process associated with this rare condition, which impacts the blood vessels of the brain and spinal cord. The use of cyclophosphamide is particularly significant due to its potent immunosuppressive properties. It is commonly used in situations where significant immunological activity leads to severe manifestations, making it beneficial in treating the aggressive nature of primary angiitis of the central nervous system. High-dose glucocorticoids such as prednisone are employed initially to manage inflammation and effectively control symptoms. While glucocorticoids alone might be considered in other inflammatory or autoimmune conditions, the severity and morbidity associated with primary angiitis necessitate a more aggressive strategy. Therefore, the combination therapy addresses the need for both immediate suppression of inflammation and longer-term control of disease activity, optimizing patient outcomes. In contrast, treatments involving only low-dose glucocorticoids or alternative immunosuppressants like methotrexate are generally not advised, as they are insufficient in controlling the aggressive inflammatory process seen in this condition.