Challenging Manifestations of ANCA-Associated Vasculitis Treated with Avacopan: Two Case Reports

AGLAIA CHALKIA^1*Anastasia Politi¹Isavella Tryfonos¹Christos Koutsianas²Harikleia Gakiopoulou²Dimitrios Vassilopoulos²Dimitrios Petras¹

• ¹Hippokration General Hospital, Athens, Greece
• ²Ethniko kai Kapodistriako Panepistemio Athenon, Athens, Greece

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a small-vessel vasculitis frequently affecting the kidneys and lungs. Avacopan, a C5a receptor inhibitor, has demonstrated efficacy as a glucocorticoid-sparing therapy, but data in rare or severe manifestations such as interstitial lung disease (ILD), cardiac involvement, or epididymo-orchitis are limited. We report two patients with newly diagnosed AAV and severe kidney involvement requiring haemodialysis. Case 1, a 65-year-old man with MPO-AAV, presented with ILD with a Usual Interstitial Pneumonia (UIP) radiological pattern, cardiac dysfunction, ENT involvement, and peripheral neuropathy. Case 2, a 68-year-old man with PR3-AAV, presented with kidney disease and epididymo-orchitis. Both patients received corticosteroids, rituximab, cyclophosphamide, plasma exchange, and avacopan. Dialysis independence was achieved within the first month in both cases. In Case 1, left ventricular function normalized, and ILD showed radiological improvement over six months. In Case 2, epididymo-orchitis resolved completely within one month. Both patients achieved clinical remission and minimal glucocorticoid exposure. These cases provide insights supporting the safety and efficacy of avacopan in severe, multisystemic AAV, including rare manifestations, and highlight its potential to promote organ recovery and reduce glucocorticoid-related toxicity in high-risk presentations.