Insights from Portuguese Nephrologists and Rheumatologists Concerning the Treatment of ANCA Vasculitis

Abstract

Introduction: Antineutrophilic cytoplasmic antibodies-associated vasculitis (AAV) encompasses granulomatosis with polyangiitis, microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis. These rare diseases are characterized by an exaggerated immune response in blood vessels, leading to inflammation, vascular injury, ischemia, and necrosis of affected tissues and organs. Despite the existence of European recommendations for treating AAV, their implementation in Portugal remains unclear. Methods: To address this gap, an online Delphi panel was conducted involving 55 Portuguese nephrologists and rheumatologists, representing the main specialties involved in the management of AAV. The primary objective was to assess the level of consensus regarding the treatment of AAV in Portugal and the application of European recommendations in daily clinical practice. The specialists evaluated 29 statements formulated by a group of Portuguese experts with extensive experience in AAV, addressing key aspects of AAV management, rated on a 4-point Likert scale. Consensus was defined as > 70% of responses either strongly agreeing or strongly disagreeing with a statement, while the majority level required > 70% agreement or disagreement. Results: After one round, no statement reached consensus globally, and eight out of the 29 statements did not achieve a qualified majority. Nonetheless, this study demonstrates a high level of agreement within each medical specialty, due to the distinct characteristics of the patient populations treated by each specialty. While the results suggest partial adherence to European recommendations, they also highlight the need to standardize clinical practices for AAV treatment in Portugal. Conclusion: This study underscores the importance of aligning national practices with established European recommendations to ensure the best outcomes for patients with AAV.