American Board of Internal Medicine (ABIM) Certification Practice Exam

In patients with organ-threatening ANCA-associated vasculitis, what is the recommended initial treatment?

1. High-dose steroids alone

2. Glucocorticoids with rituximab or cyclophosphamide

3. Only rituximab

4. Maintenance immunotherapy

The correct answer is: Glucocorticoids with rituximab or cyclophosphamide

In patients with organ-threatening ANCA-associated vasculitis, the recommended initial treatment involves the use of glucocorticoids in conjunction with rituximab or cyclophosphamide. This combination is designed to rapidly control the severe inflammation and prevent further damage to vital organs, which is critical in managing this condition effectively. Glucocorticoids serve to suppress the inflammatory response significantly, while rituximab and cyclophosphamide are both powerful immunosuppressive agents that help to target the underlying autoimmune process driving the vasculitis. The use of both treatment modalities together helps to ensure a more robust and rapid therapeutic response, reducing the risk of complications and protecting affected organs from further injury. While high-dose steroids may provide some degree of symptom relief, they alone are insufficient for controlling the underlying disease in cases where there is organ threat. Monotherapy with rituximab is also not recommended as an initial treatment in severe cases because it typically requires a more comprehensive approach to adequately address the rapidly progressing vasculitis. Maintenance immunotherapy is generally considered after achieving adequate disease control, rather than as an initial treatment strategy in acute presentations.