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 u p 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 sett ing 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 ca red for in the Ocular Immunology Service of the Massachusetts Eye and Ear I nfirmary during the 10 year period 1988-98 with a diagnosis of Wegener's gr anulomatosis. A detailed chart review was undertaken to determine demograph ic characteristics, history, initial manifestation of WG, initial ocular pr esentation, biopsy results, laboratory testing results, treatment, total fo llowup period, and final outcome. Results, Forty-seven patients diagnosed with WG were identified. Twenty-eig ht were women (59.6%) 19 were men (40.4%). The average age was 53 years (ra nge 18-90). Patients were divided into 4 groups. Group I included 27 patien ts (57.4%) who had systemic disease first and who subsequently developed an ocular lesion. Group II included 3 patients (6.3%) who had ocular inflamma tion first and who then subsequently developed systemic manifestations of W G. Group III included 3 patients (6.3%) who presented due to ocular symptom s but, on initial evaluation by us, were found to have occult systemic mani festations consistent with WG or biopsy evidence of WG. Group IV included 1 4 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 We. It may represent the first sign of WG that enables th e knowledgeable physician to diagnose this potentially lethal disease.