ADVERTISEMENT

Ocular manifestations of small-vessel vasculitis

November 1, 2012|Cleveland Clinic Journal of Medicine
James A. Garrity, MD
Author and Disclosure Information

James A. Garrity, MD
Whitney and Betty MacMillan Professor, Department of Ophthalmology, Mayo Clinic, Rochester, MN

Correspondence: James A. Garrity, MD, Department of Ophthalmology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905; Garrity.james@mayo.edu

Dr. Garrity reported that he has no financial interests or relationships that pose a potential conflict of interest with this article.

This article was developed from an audio transcript of Dr. Garrity’s presentation at the “New Directions in Small-Vessel Vasculitis: ANCA, Target Organs, Treatment, and Beyond” symposium held at Cleveland Clinic on May 4, 2011. The transcript was formatted and edited by Cleveland Clinic Journal of Medicine staff for clarity and conciseness, and was then reviewed, revised, and approved by Dr. Garrity.

ABSTRACTOphthalmic manifestations of vasculitis can be orbital, ocular (affecting the globe), or intraocular. Orbital inflammation manifests as sudden onset of pain, erythema, and proptosis, and can be sight-threatening. In the globe, red eye is typical in both episcleritis and scleritis. Episcleritis is usually otherwise asymptomatic with blanching upon instillation of topical phenylephrine, whereas scleritis is painful and does not blanch. Infectious and rheumatic diseases are present in nearly 50% of patients with scleritis. The symptoms of keratitis are similar to those of scleritis; superficial keratitis is benign but peripheral ulcerative keratitis can be sight-threatening. Anterior uveitis is the most frequent ocular manifestation of Behçet disease. Approximately 30% of patients with granulomatosis with polyangiitis (Wegener’s granulomatosis) have ocular involvement, with orbital disease being most common. With ophthalmic manifestations of vasculitis, tissue biopsy of any site that is amenable to biopsy is recommended. Biopsy must be interpreted within the context of treatment.

Intraocular inflammation

Figure 2. Ischemic optic neuropathy. Appearance of ischemic optic neuropathy (left panel) versus normal fellow eye (right panel).
Vascular involvement. Vasculitic involve ment of blood vessels within and around the eye can produce several different clinical pictures. Within the eye are two separate intraocular circulations, the retinal vessels and the choroid circulation. Retinal vessel involvement can affect the retinal arteries with thrombotic occlusions, the veins with phlebitis, or both. Retinal artery occlusions take the form of a branch or central retinal artery occlusion that infarcts corresponding portions of the retina, leading to loss of vision. The optic disc is also subject to vascular interruption of the posterior ciliary arteries, which constitute a separate branch off the ophthalmic artery. This condition produces an ischemic optic neuropathy and results in loss of vision (Figure 2).

There is no specific treatment for the eye other than treating the underlying condition. Vascular occlusions can sometimes give rise to neovascularization and patients should be followed for this possibility. As with a central nervous system ischemic event, recovery can be variable.

Uveitis. The term “uvea,” derived from the Greek word for grape, describes the shape of the iris, ciliary body, and choroid. Uveitis is a generic term for intraocular inflammation affecting any or all of these structures.

Iritis, or anterior uveitis, is a frequent accompaniment of keratitis or scleritis. Primarily uveitic involvement with retinal vessel vasculitis involving both arteries and veins is uncommon in general but typical of Behçet disease, especially if a hypopyon uveitis is present.

Anterior uveitis can be treated with topical corticosteroids and cycloplegic drugs, but middle and posterior uveitis almost always requires systemic therapy. Most recently, use of anti–tumor necrosis factor-α drugs has been effective in treating Behçet uveitis.8 The visual prognosis with Behçet disease remains guarded.

GRANULOMATOSIS WITH POLYANGIITIS: EYE INVOLVEMENT IS COMMON

In terms of specific small-vessel vasculitic diseases that affect the eye, granulomatosis with polyangiitis (GPA [Wegener’s granulomatosis]) is the quintessential condition. In data obtained from the Wegener Granulomatosis Support Group,9 eye involvement was noted at presentation in 211 of 701 patients (30%), and during the course of their disease an additional 147 patients developed eye involvement. From the time of initial presentation through the course of follow-up, 359 of the 701 patients (51%) eventually had some type of eye involvement.

In a series of patients seen at the Mayo Clinic,10 orbital inflammatory disease and scleritis were the two most frequent manifestations of eye involvement with GPA. Orbital involvement typically presents with pain, erythema, swelling, and proptosis. Varying degrees of ptosis, diplopia, or visual loss may also be present. Imaging may show an infiltrate that is usually adjacent to the maxillary or ethmoid sinus. This same process can affect the superior temporal orbital quadrant, an area apart from any sinus, and involve the lacrimal gland.

BIOPSY IS ADVISED

Biopsy, either incisional, at times to include debulking, or excisional if possible, is recommended to establish a diagnosis or aid in the selection of therapy. Orbital disease has been observed to progress in patients who are receiving maintenance therapy with methotrexate and have no evidence of systemic disease activity. Acute and chronic inflammation with evidence of active vasculitis is usually seen histologically. Personal observations suggest that intraorbital corticosteroid injection followed by rituximab has been effective therapy for this limited subset of patients. Diagnostic biopsies often must be interpreted in light of partial treatment, making histopathologic diagnosis challenging at times. Biopsy is important for exclusion of lymphoproliferative disease or fungal infection.

CONCLUSION

Underlying vasculitis might play a role in patients with nonspecific ocular presentations. It is essential that the ophthalmologist collaborate with a specialist in vasculitis (and vice versa) for evaluation and subsequent therapy, which often involves some form of immunosuppression.

ADVERTISEMENT
ADVERTISEMENT
ADVERTISEMENT