Tarsal-Conjunctival Disease Associated With Wegener’s Granulomatosis

Abstract

Abstract: : Purpose: Ocular involvement with Wegener’s Granulomatosis (WG) most commonly involves the cornea, sclera, and orbit. There have been few reports of a cicatricial process involving the conjunctiva and tarsus leading to entropion, trichiasis and in some cases, vision loss. Herein, we report a retrospective study on WG patients to evaluate the occurrence, clinical features, and results of medical therapy in patients with tarsal-conjunctival disease. Methods: A retrospective chart review was performed on 82 patients with WG examined at the NEI from January 1996 to June 2002. Statistical analysis performed using Bayesian methods. Results: Tarsal-conjunctival disease occurred in 13/82 (16%) patients with WG. The majority of the patients were male, predominantly white, with a median age of 47 years when diagnosed with the tarsal-conjunctival disease. The disease was characterized by the following features occurring in at least one eyelid of each patient: conjunctival hyperemia in 7 patients (54%), inactive fibrous scar tissue in 7 patients (54%), active fibrovascular proliferation in 6 patients (46%), conjunctival (with or without tarsus) necrosis in 4 patients (31%), and granulomatous lesions in 3 patients (23%). Eyelid biopsies showed granulomatous inflammation, focal necrosis and areas of occlusive vasculitis. Medical therapy was required for 6/13 (46%) patients and only 1/6 patients showed a response to an increase in topical and/or systemic immunosuppression. The association of tarsal-conjunctival disease and subglottal stenosis was found to have a very high probability of clinical significance (89%). Conclusions: Tarsal-conjunctival disease associated with WG was characterized by conjunctival hyperemia, fibrovascular proliferation, and necrosis of the palpebral conjunctiva and tarsus that can lead to entropion and trichiasis. Generally, tarsal-conjunctival disease was not responsive to immunosuppression, similar to the clinical course of other mucosal manifestations of WG, such as subglottic stenosis. Tarsal-conjunctival disease was found to have a high clinical significance of association with subglottic stenosis, a disease that led to respiratory distress requiring tracheostomies and/or dilation procedures in 6/13 (46%) of our patients.

Keywords: conjunctiva • autoimmune disease