Ocular Manifestations of Granulomatosis with Polyangiitis: A Review of the Literature

Abstract

Granulomatosis with polyangiitis (GPA) is a rare systemic autoimmune disease of unknown etiology that is characterized by granulomatous inflammation, tissue necrosis, and vasculitis in small- and medium-sized vessels. Ocular and orbital manifestations are common in almost half of patients with GPA, affecting every structure of the eye, from the eyelid and orbit to the retina, the choroid, and the optic nerve, with a wide range of severity. Since imaging findings are not always specific for the diagnosis of GPA, biopsy is useful to confirm the diagnosis. Regarding treatment, a localized pharmaceutical and surgical approach may be helpful to achieve remission, while immunosuppressive therapy, corticosteroids, and cyclosporine are also useful. In any case, multidisciplinary intervention is required to reduce the rates of relapse and morbidity in patients with GPA.

Granulomatosis with polyangiitis (GPA) in a 76-year old woman presenting with pulmonary nodule and accelerating acute kidney injury

“…Ophthalmic involvement by GPA is less common but typically presents as scleritis/episcleritis, which presents with eye redness and pain, conjunctivitis and corneal ulceration. More rare ophthalmic presentation of GPA includes Optic neuropathy, uveitis, retinal vasculitis with visual disturbances, diplopia, foreign body sensation in the eye and proptosis have also been reported [10]. Oral ulcers or gingivitis (strawberry gingivitis) have been seen in a patient with GPA [11].…”

Atypical painless vision loss in a patient with granulomatosis with polyangiitis

“…Granulomatosis with polyangiitis (GPA) is a necrotizing granulomatous vasculitis affecting the small- and medium-sized vessels in the orbit, sinus, lungs, and kidneys [ 1 ], [ 2 ]. It is a rare disease with the annual incidence being 2.4–11 cases per million, equally affecting both genders of individuals in their 40s–70s [ 1 ], [ 3 ], [ 4 ], [ 5 ], [ 6 ]. Ocular findings are seen in 15–60% of cases and can be some of the earliest findings [ 1 ], [ 2 ], [ 3 ], [ 5 ], [ 7 ].…”

“…It is a rare disease with the annual incidence being 2.4–11 cases per million, equally affecting both genders of individuals in their 40s–70s [ 1 ], [ 3 ], [ 4 ], [ 5 ], [ 6 ]. Ocular findings are seen in 15–60% of cases and can be some of the earliest findings [ 1 ], [ 2 ], [ 3 ], [ 5 ], [ 7 ]. Nearly 50% of patients can develop painful scleritis, as well as other ocular signs and symptoms including uveitis, keratitis, and orbital inflammation resulting in proptosis, diplopia, and epiphora.…”

“…Nearly 50% of patients can develop painful scleritis, as well as other ocular signs and symptoms including uveitis, keratitis, and orbital inflammation resulting in proptosis, diplopia, and epiphora. If orbital findings are present, 20–50% of patients can develop severe vision loss [ 1 ], [ 3 ], [ 8 ], [ 9 ]. Painless vision loss without orbital findings has rarely been reported [ 1 ], [ 10 ].…”

“…If orbital findings are present, 20–50% of patients can develop severe vision loss [ 1 ], [ 3 ], [ 8 ], [ 9 ]. Painless vision loss without orbital findings has rarely been reported [ 1 ], [ 10 ]. High clinical suspicion and collaboration with multiple sub-specialties is required to reduce mortality and morbidity.…”