Granulomatosis with polyangiitis (GPA) (previously Wegener’s granulomatosis) is part of the spectrum of systemic necrotizing vasculiites. It is a rare systemic inflammatory disease with necrotizing granulomatous vasculitis of the small-to-medium sized vessels. The disease usually manifests in adults but rare cases have been reported in the pediatric population [1]. GPA can affect any organ, but most commonly affects the sinuses and lungs (respiratory tract), kidneys, and the eye [2]. The granulomatosis is characterized by necrosis and thrombosis of the vessels.

Ophthalmologic disease is the manifesting feature of GPA in 8–16 % of patients but develops in an estimated 50–60 % of patients [4]. Orbital disease (30 %), episcleritis, scleritis, and conjunctivitis are the most common ophthalmological manifestations of GPA. Uveal involvement and granulomatous sclerouveitis are less common presentations. Uveitis (including anterior and posterior involvement) accounts for less than 10 %, and retinal involvement accounts for less than 5 % of ocular manifestations of GPA [5].

Anterior, posterior, and panuveitis have all been described in isolation or is associated with scleritis in GPA. The majority of uveitis in GPA is an anterior uveitis (70 % of uveitis cases) and more commonly occurs synchronously with anterior scleritis [6]. Anterior uveitis can be acute, granulomatous, or chronic with relapsing phases and may induce cystoid macular edema [7]. Clinical examination may range from mild ocular injection to significant inflammation with mutton fat keratic precipitates and substantial synechiae.