Ophthalmic Manifestations in Granulomatosis with Polyangiitis: A Narrative Review

Abstract

Granulomatosis with polyangiitis (GPA), a type of vasculitis, is characterized by necrotizing inflammation of small-medium blood vessels and is associated with anti-neutrophil cytoplasmic antibodies (ANCA). While GPA commonly affects the upper respiratory tract, lungs, and kidneys, ocular manifestations can also occur as a part of systemic GPA or isolated GPA involving eyes, the diagnosis of which can be challenging. Ocular involvement in GPA is variable and can present as scleritis, keratitis, conjunctivitis, uveitis, or orbital involvement. This review article briefly explores the etiology of GPA with a focus on the ophthalmic manifestations of GPA, including their clinical features, diagnosis, differentials, and treatment options. Understanding the ocular manifestations can help in early diagnosis, preventing irreversible eye damage and vision loss. Conclusion: The small blood vessels in almost any organ or tissue can be involved in GPA, the involvement of the eye being one such organ which can present with non-specific signs and symptoms in both generalized and isolated forms. Knowledge of the ocular manifestations of GPA can help catch the disease in its initial stage and prevent irreversible eye damage. Despite being recognized in over half of patients with GPA, ophthalmic signs go misdiagnosed in most patients. The most efficacious strategy for preventing disease mortality and morbidity is having a high level of clinical suspicion, early diagnosis, and commencing immunosuppressive medication early in the disease course.