Ophthalmologic manifestations of the granulomatosis with polyangiitis (Wegener)

Abstract

PurposeWegener's granulomatosis (WG) is a severe focal granulomatous inflammation with variable ophthalmic manifestationsMethodsPatients with a confirmed WG, seen in a single tertiary center between 2008 and 2014 were included retrospectively. WG was proven based on biological or histological analysisResultsFive patients (3W/2M) aged 26–55 years, were included. Clinical features included one scleritis with dacryoadenitis, one bilateral episcleritis complicated with uveo‐scleritis, one bilateral Mooren's ulcer and two orbital inflammatory pseudotumor. Time between the onset of symptoms and the diagnosis was 100 months (range: 1–276 months). All patients had anti‐cytoplasm antibodies to polynuclear neutrophils and underwent biopsies. Histological features included vasculitis inflammatory necrotizing granulomas. Extraocular features were pulmonary lesions (100%), rhinosinusal involvment (80%), renal disease (40%), neurological injury (40%). All patients were initially treated with high‐dose corticosteroids, wich were progressively decreased and received maintenance immunosupressive drugs. Immunosuppressive treatment was initiated on average 64 months after the onset of signs (range: 1–276 months)ConclusionsWG‘s ophthalmologic symptoms are variable. Scleritis remains the most common manifestation, followed by orbital inflammation. Atypical manifestations such as Mooren's ulcer or uveitis can delay the diagnosis. A multidisciplinary approach is mandatory to control this life threatening condition. WG should be considered in all cases of chronic orbital inflammation, scleritis or keratitis to ensure prompt diagnosis and improve the systemic prognosis.