Wegener's granulomatosis: The relationship between ocular and systemic disease

Objective. Wegener's granulomatosis (WG) is an etiologically obscure entity with multiple systemic manifestations. Ocular involvement is present in up to 58% of patients with WG. We describe a series of patients with ocular manifestations of WG to evaluate the presence of ocular lesions in the setting of systemic WG and to determine the value of ocular inflammation in the diagnosis of WG. Methods. A computerized database was used to generate a list of patients cared for in the Ocular Immunology Service of the Massachusetts Eye and Ear Infirmary during the 10 year period 1988-98 with a diagnosis of Wegener's granulomatosis. A detailed chart review was undertaken to determine demographic characteristics, history, initial manifestation of WG, initial ocular presentation, biopsy results, laboratory testing results, treatment, total followup period, and final outcome. Results. Forty-seven patients diagnosed with WG were identified. Twenty-eight were women (59.6%), 19 were men (40.4%). The average age was 53 years (range 18-90). Patients were divided into 4 groups. Group I included 27 patients (57.4%) who had systemic disease first and who subsequently developed an ocular lesion. Group II included 3 patients (6.3%) who had ocular inflammation first and who then subsequently developed systemic manifestations of WG. Group III included 3 patients (6.3%) who presented due to ocular symptoms but, on initial evaluation by us, were found to have occult systemic manifestations consistent with WG or biopsy evidence of WG. Group IV included 14 patients (30%) with ocular lesions and no history or presence of systemic disease at their last followup visit. Conclusion. Ocular inflammation can occur with or without obvious systemic manifestations of WG. It may represent the first sign of WG that enables the knowledgeable physician to diagnose this potentially lethal disease.