Wegener's granulomatosis: A diagnostic challenge

Background: Wegener's granulomatosis (WG) is a systemic necrotizing granulomatous vasculitis of unknown etiology. Primarily the small arteries and the arterioles are affected. The main sign is vasculitis with ulcerating granulomas in the respiratory tract and glomerulonephritis. Ocular manifestations are found in 60% of patients, involving the eye and the orbit. We are presenting a case in which the systemic disease occurred as late as 18 months after the onset of various ophthalmologic symptoms. Method and patient.: In January 1997 a 46-year-old patient was first admitted to our department for anterior scleritis. The complaints had already started about 7 months before his admission. Both eyes were painful and often red. After his first hospitalization the patient was constantly under outpatient treatment and was again hospitalized twice at our department. Due to the chronically recurrent course, complications such as episcleritis, anterior scleritis, ulcers of the cornea in the limbal area, posterior scleritis with secondary exudative retinal detachment with vision loss occurred repeatedly in the months following his hospitalization. The origin was unclear since at that time no signs of any systemic disease were observed. As necrotizing vasculitis was suspected from the beginning, among others a systemic steroid therapy was also introduced at that time. Results: WG was diagnosed as late as 18 months after the onset of ocular symptoms. Namely, the patient contracted vasculitis of the skin, pericarditis and glomerulonephritis. Subsequently a rheumatologist supplemented the systemic steroid therapy with immunsuppressives. Three years later bilateral phacoemulsification with IOL implantation was carried out due to a cortisone cataract. Under the supervision of treatment by the rheumatologist, complete remission of the disease was achieved by the end of 2004. Visual acuity in both eyes was 1.0 with correction. Conclusions: The initial symptoms of WG can be various and atypical. In ophthalmology the eye and the orbit are involved. Ocular findings can be the only or the first sign of WG, but they can also become manifest together with the systemic disease. Our patient exhibited the ocular symptoms as early as 18 months prior to the onset of the systemic disease.