Bilateral corneal perforation and iris prolapse as a complication non-peripheral ulcerative keratitis in a patient with fulminant granulomatosis with polyangiitis: a case report

Granulomatosis with polyangiitis (GPA) (formerly known as Wegener´s granulomatosis) is a systemic necrotizing vasculitis belonging to a heterogeneous group of systemic anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis which affects small and medium-sized blood vessels [1, 2]. The annual incidence of GPA has been estimated to be 8 to 10 million, has a peak age of onset of 64 to 75 years [3], and the frequency of presentation in female and male individuals is similar [4]. Constitutional signs like fever, asthenia, and weight loss are frequent (50%) but non-specific. Ear, nose, and throat signs (crusting rhinorrhea, sinusitis, chronic otitis, or damage of the facial cartilage) are present in 70 to 100% of cases at diagnosis [5]. Lung involvement, characterized by alveolar hemorrhage or parenchymatous nodules, affects 50 to 90% of patients [4]. The focal segmental necrotizing glomerulonephritis is the most frequent renal involvement in 40 to 100% of cases [6]. Involvement of the nervous system, the central nervous system, the pachymeninges, the heart, the pericardium, and the gastrointestinal system are less frequent, observed in a range of 5 to 40% of cases [1]. Ocular involvement is frequent, typically in the form of necrotizing nodular episcleritis, peripheral corneal ulcerations, and retinal vasculitis. The involvement of the eye socket is rare and can be characterized by a granulomatous retro-orbital pseudotumor or as dacryoadenitis. Inflammatory exophthalmia can also be a sinus inflammation manifestation [1]. We report a case of a male patient with GPA diagnosis with severe and fulminant progression of the disease and atypical ocular manifestations.