Contributors: R. Jean Campbell and Diva R. Salomão
Wegener granulomatosis (Wegener disease) is a disease characterized by a triad of necrotizing granulomas of the upper and lower respiratory tract, necrotizing and crescentic glomerulonephritis and small vessel necrotizing vasculitis [vasculitis - necrotizing]. Orbital involvement alone is seen in 46% of patients; 54% of patients have the classical triad. The female to male ratio is 10:7.This contrasts with the generalized form of the disease which is more common in the male. Age of occurrence ranges from the second to the eighth decade. The disease is an immunopathological disease characterized by the presence of autoantibodies against extra neutrophil components of neutrophils and monocytes. In the classical form of Wegener disease, the ophthalmic manifestations affect 28-50% of patients and may be the presenting form of the disease. Secondary involvement of the orbit to disease in the paranasal sinus is more common than primary orbital disease. Proptosis is painful and associated with limited extraocular muscle movement. A limited form of the disease may occur and this may be confined to the orbit. Visual loss, when it occurs, is due to compression of the optic nerve or to vasculitis. Rarely, the nasolacrimal duct is obstructed. Vasculitis of the anterior ciliary arteries and posterior ciliary arteries leads to conjunctivitis, corneal ulceration, episcleritis, necrotizing scleritis [scleritis - necrotizing] and may be complicated by choroidal detachment [detachment - choroid] and retinal detachment.