Dr. Tanaz Kermani, founder of the Vasculitis Program at UCLA, dives into the complexities of ANCA-associated vasculitis. She discusses when to choose rituximab over cyclophosphamide and the potential of avacopan as an adjunct therapy. The conversation covers critical decision-making in steroid tapering and the viability of plasma exchange. Kermani also highlights the significance of accurate diagnosis through biopsies, emphasizing the role of pathologists in managing this rare autoimmune disorder.
The choice between rituximab and cyclophosphamide for induction treatment in ANCA-associated vasculitis must consider each patient's specific condition and renal involvement.
Avacopan is emerging as an effective adjunct therapy that may improve renal recovery and facilitate quicker glucocorticoid tapering in select patients.
Deep dives
Challenges in Diagnosing Vasculitis
Confirming a diagnosis of ANCA-associated vasculitis often presents challenges, particularly when biopsies are difficult or declined by patients. While biopsies can provide definitive evidence, certain procedures, like nerve or lung biopsies, may be more complex and require collaboration with specialists. Additionally, there is a concern about interpreting serologies, as positive tests can arise from conditions other than vasculitis, such as infections or other autoimmune disorders. The importance of accurate pathology is underscored, as a misinterpretation of biopsy results can lead to incorrect diagnoses and treatment plans.
Selecting Induction Treatments
When managing critically ill patients with ANCA-associated vasculitis, selecting the appropriate induction treatment, such as rituximab or cyclophosphamide, is crucial. Rituximab is often preferred due to its effectiveness, but in severe cases or when rapid action is needed, cyclophosphamide may be chosen based on specialist input. The discussion emphasizes the importance of evaluating each patient's specific condition, particularly with regard to renal involvement. Complications, like the potential for concurrent glomerulonephritis, play a significant role in making these treatment decisions.
Advancements with Avacopan
Avacopan has emerged as a significant adjunct therapy in treating ANCA-associated vasculitis, particularly for patients with renal insufficiency. Studies suggest that it may facilitate better renal recovery while allowing for a quicker tapering of glucocorticoids, addressing concerns related to steroid-induced side effects. Although it is not recommended for all newly diagnosed patients, it can be beneficial for those struggling to taper glucocorticoids due to comorbidities. The ongoing exploration of avacopan's role, especially concerning relapses and long-term treatment strategies, highlights its potential in enhancing patient outcomes.
When should we choose rituximab over cyclophosphamide? What role can avacopan play as an adjunct therapy? When is plasma exchange a viable option? How can we effectively navigate steroid tapering? These are just a few of the critical questions we’ll explore with our next guest, Dr. Tanaz Kermani, founder and director of the Vasculitis Program at UCLA, a dedicated clinician and active researcher. Join us as we delve into these topics and address the challenges of diagnosing and managing patients with vasculitis.
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