Granulomatosis with polyangiitis - External and Internal Eye
The disease typically presents with upper airway disease and nonspecific complaints such as malaise, arthralgias, fever, and weight loss. Respiratory tract symptoms include sinusitis, otitis media, rhinorrhea, cough, sputum production, chest pain, dyspnea, and hemoptysis.
The disease presents variably on a continuum from limited involvement (which may remain limited for variable periods of time) to a more generalized form with involvement of the upper and lower respiratory tract, kidney, skin, and other organs.
Ocular involvement occurs in 30%-50% of patients with GPA and can occur without systemic manifestations. There can be orbital, retinal, nasolacrimal, and anterior segment involvement. The most common findings are scleritis and orbital disease. Ocular manifestations of GPA result from granulomatous sinusitis infiltrating the orbit, causing nasolacrimal duct obstruction and proptosis, putting increased pressure on the intraocular muscles and optic nerve. Many patients complain of decreased vision. Vision may also be lost, as proptosis can lead to exposure keratopathy and corneal ulceration. There may also be a focal vasculitis, such as conjunctivitis, episcleritis, scleritis, uveitis, or granulomatous vasculitis of the retina and optic nerve.
Untreated, the generalized form is fatal in less than 2 years; however, long-term remissions are now possible in more than 90% of patients with the advent of cytotoxic therapy. Complications of GPA include pericarditis, coronary arteritis, myocardial infarction, pancarditis, granulomatous valvulitis, mononeuritis multiplex, and polyneuritis.
This summary will focus on ocular manifestations of the disease.
M31.30 – Wegener's granulomatosis without renal involvement
M31.31 – Wegener's granulomatosis with renal involvement
195353004 – Wegener's granulomatosis
- Vasculitis of other types (polyarteritis nodosa, Behçet, rheumatoid vasculitis, microscopic polyangiitis)
- Sarcoidosis (and other granulomatous diseases)
- Graves ophthalmopathy
- Other causes of scleritis (rheumatoid arthritis, inflammatory bowel disease [eg, ulcerative colitis, Crohn], systemic lupus erythematosus, and relapsing polychondritis)
Last Updated: 07/26/2017