Therapy of ocular complications in ANCA+ associated vasculitis

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. Abbreviations: AAV = ANCA+ associated vasculitis, ANCA = anti-neutrophil cytoplasmic antibodies, GPA = granulomatosis with polyangiitis, EGPA = eosinophilic granulomatosis with polyangiitis, MPA = microscopic polyangiitis

Clinical profile and management of granulomatosis with polyangiitis–associated scleritis from a tertiary care hospital in South India

“…First, remission is usually induced by combining high-dose corticosteroids with cyclophosphamide or rituximab. [ 33 ] Intravenous pulse cyclophosphamide is preferred over oral daily cyclophosphamide because of lower rates of adverse events. [ 34 ] Absence of remission should be managed by switching to rituximab from cyclophosphamide or vice versa.…”

“…[ 34 ] Absence of remission should be managed by switching to rituximab from cyclophosphamide or vice versa. [ 33 ] Once remission is achieved, maintenance therapy can be continued with immunomodulators like cyclophosphamide, MMF, or methotrexate, along with continued corticosteroid tapering. [ 33 ] In cases unresponsive to conventional therapy, there are anecdotal reports on using tumor necrosis factor-a inhibitors like etanercept and infliximab to achieve remission.…”

“…[ 33 ] Once remission is achieved, maintenance therapy can be continued with immunomodulators like cyclophosphamide, MMF, or methotrexate, along with continued corticosteroid tapering. [ 33 ] In cases unresponsive to conventional therapy, there are anecdotal reports on using tumor necrosis factor-a inhibitors like etanercept and infliximab to achieve remission. [ 35 36 37 38 39 ] Similar protocols were followed in treating our patients in this study.…”

Current concepts in granulomatosis poly angiitis and the eye

“…In cases of progressive vision loss and persistent pain, surgical management with orbitotomy with orbital decompression or orbital debulking surgery may be considered. Exenteration may be required in cases of marked proptosis, blindness, and severe pain [57 ▪▪ ].…”