Ocular manifestations of systemic disease: antineutrophil cytoplasmic antibody-associated vasculitis

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) may be localized or systemic, and can involve the eyes. This review presents the major disease features and treatments of these forms of systemic vasculitis.

It is increasingly recognized that ocular disease in AAV can occur in the absence of systemic disease, and that in limited eye disease the ANCA is frequently undetected. Ocular disease in AAV can take many forms, from orbital pseudotumor to scleritis, keratitis, and retinitis. Management of eye disease in ANCA-associated vasculitis includes local anti-inflammatory and moisturizing therapies, local and systemic glucocorticosteroid therapies. Systemic therapies are highly effective for the management of organ involvement of AAV including eye disease, and include the chemotherapies cyclophosphamide, azathioprine, mycophenolate mofetil, and methotrexate. The results of recent, randomized, clinical trials have demonstrated the efficacy of a new tool in the therapeutic armamentarium for AAV, the use of B-cell-depleting therapy with rituximab.

Ocular disease in AAV can occur in isolation or more commonly as a manifestation of systemic disease. With recent advances in treatment, the organ and overall life expectancy of patients suffering from AAV has markedly improved.