Therapy of ocular complications in ANCA+ associated vasculitis
S Macarie, Răzvan-Marian Mihăilă, Daniela Mariana Macarie, Dan Alexandru Țoc
Abstract
ANCA+ associated vasculitis (AAV) is a group of rare diseases with potentially vision-threatening complications. Ocular and orbital complications of these diseases are caused by vasculitis of the small vessels of the eye or by granulomatous mass formation. ANCA (anti-neutrophil cytoplasmic antibodies) represent a key component of pathophysiological pathways as well as a diagnostic marker. Various manifestations are reported in literature, scleritis and episcleritis being the most common, followed by pseudotumor orbitae. In vision-threatening orbital or ocular disease, aggressive systemic treatment with a combination of high-dose glucocorticoids and either cyclophosphamide or rituximab is needed. Certain cases require locoregional surgical management to preserve ocular integrity or vision. Ocular involvement of AAV remains a challenge in clinical practice, requiring multi-specialty cooperation in order to ensure the best possible visual outcome.