In 10% of cases, antibodies against myeloperoxidase (MPO) are positive, and rarely, patients can also present with ANCA-negative disease [3]. These antibodies trigger an inflammatory cascade involving neutrophils and the complement pathway, resulting in necrotizing tissue inflammation and granuloma formation [4]. Ocular involvement is seen in approximately 30-50% of patients with GPA and can involve any part of the eye [5,6]. Here, MPO is linked to granulomatosis with polyangiitis.