Wegener’s granulomatosis: the relationship between ocular and systemic disease. Harper SL, Letko E, Samson CM, Zafirakis P, Sangwan V, Nguyen Q, Uy H, Baltatzis S, Foster CS. J Rheumatol 2001;28:1025–1032.

Abstract

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. These authors 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. 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 1988–1998 with a diagnosis of WG. 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 follow-up period, and final outcome. Forty-seven patients found to have WG were identified. Twenty-eight were women (59.6%), and 19 were men (40.4%). The average age was 53 years (range, 18–90). Patients were divided into four groups. Group I included 27 patients (57.4%) who had systemic disease first and subsequently developed an ocular lesion. Group II included three patients (6.3%) who had ocular inflammation first and then subsequently developed systemic manifestations of WG. Group III included three patients (6.3%) who presented because of ocular symptoms but on initial evaluation 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 follow-up visit. The authors conclude that ocular inflammation can occur with or without obvious systemic manifestations of WG. It may represent the first sign of WG and may enable the knowledgeable physician to diagnose this potentially lethal disease.—Thomas J. Liesegang