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Giant cell arteritis (GCA) is a medium-to-large vessel vasculitis that usually presents with headache, loss of vision, jaw claudication, and scalp tenderness. We report a case of biopsy-consistent GCA presenting as an infectious bacterial keratitis. To our knowledge, this is the first such case reported in the English-language ophthalmic literature.
A 76-year-old white woman presented with progressive loss of vision and acute onset of several weeks’ duration of pain and redness in her left eye (OS) with associated headache and neck pain (Fig. 1A). On examination, the best-corrected visual acuity was 20/400 in the right eye (OD) and no light perception in the left eye (OS). She had a left relative afferent pupillary defect. Motility examination was full in both eyes (OU). The left eye had conjunctival injection and a purulent corneal ulcer with discharge that was mixed with blood. The left pupil was fixed and dilated with neovascularization of the iris (rubeosis iridis) (NVI) and an intraocular pressure of 50 mm Hg. The right eye had a dense cataract, intraocular pressure of 20 mm Hg OD, and an unremarkable posterior pole examination. No scalp tenderness or temporal artery nodularity was found.
A corneal scraping OS for gram stain and cultures was performed, and the patient was started on fortified broad-spectrum topical and systemic antibiotic therapy. A diagnosis of neovascular glaucoma and possible ocular ischemic syndrome was made. B-scan ocular ultrasonography showed no endophthalmitis or vitreous opacity OS. Erythrocyte sedimentation rate and C-reactive protein were elevated at 55 mm/hour and 0.64 mg/dL, respectively. A computed tomography scan of the head and orbits was unremarkable. Three days after admission, the corneal cultures grew Streptococcus pneumoniae and coagulase-negative Staphylococcus. Magnetic resonance imaging (MRI) of the brain and orbits showed abnormal T2 signal intensity of the left optic nerve (Fig. 1B). Magnetic resonance angiography of the head was unremarkable. A temporal artery biopsy showed no active giant cells but had segmental absence of the internal elastic lamina, focal calcification, and CD68-positive histiocytes at the level of the muscularis and elastic lamina junction consistent with features seen in healing/treated temporal arteritis (Fig. 2), and the patient was started on corticosteroids. She later underwent evisceration with ocular implant OS because of severe pain and no light perception vision. Histopathology showed a partially infarcted and necrotic iris with neovascularization and chronic atrophic changes of the optic nerve with Schnabel's cavernous degeneration. Ulcerative acute bacterial (gram-positive cocci) keratitis was also confirmed (Fig. 3). Three months later, the patient underwent successful cataract extraction and intraocular lens placement OD with best-corrected visual acuity of 20/70 −2, intraocular pressure of 12, cup-to-disc ratio of 0.4, 2+ arteriovenous nicking, and epiretinal membrane +1 OD. Of note, no disc edema or pallor was present.
This patient presented with an infectious bacterial keratitis superimposed on an orbital/ocular ischemic syndrome secondary to biopsy-consistent GCA. The patient had severe orbital inflammation and secondary ischemia with enhancement on MRI. Papathanassiou et al
presented a case of peripheral ulcerative keratitis while on treatment for a GCA. Two other cases found a recurrent central corneal ulcer and marginal corneal ulceration (limbal guttering) as the presenting signs of GCA.
A number of possible ischemic or inflammatory mechanisms could produce an infectious or inflammatory keratitis or corneal ulceration in the setting of GCA. The most likely explanation is that GCA can lead to selective ischemia in the anterior segment (iris and cornea) and produce an ocular ischemic syndrome that can lead to a secondary microbial keratitis from superinfection in a compromised cornea. Systemic inflammatory dysregulation of immunologic surveillance function in GCA could also be a factor. The cornea is avascular and is thought to possess some degree of immune privilege, and this likely explains the paucity of prior reports of keratopathy in GCA.
In some of the previously published keratitis cases in GCA, prior treatment with corticosteroids was likely an additional iatrogenic risk factor for secondary infectious complications of immunosuppression.
The patient had no history of glaucoma but was found to have NVI OS on examination. NVI, a complication of ischemic intraocular tissues, was confirmed in the eviscerated eye. In this case, the segmental iris ischemia and subsequent NVI were likely secondary to hypoperfusion by one of the long posterior ciliary arteries. The NVI might have evolved into neovascular glaucoma and optic nerve atrophy. The high intraocular pressures and the probable inflammatory vascular occlusions of the short ciliary vessels and orbital vessels nourishing the optic nerve caused the accumulation of hyaluronidase-sensitive acid mucopolysaccharides (Schnabel's cavernous degeneration) in the atrophic nerve.
This probable course of events may also explain the MRI enhancement of the optic nerve and peri-optic nerve tissues. Cataract extraction 3 months later OD revealed no signs of glaucoma.
Most clinicians are already aware of the protean manifestations of GCA and that acute, painful, severe visual loss in elderly patients with elevated acute phase reactants (e.g., erythrocyte sedimentation rate and C-reactive protein) should be considered to be GCA until proven otherwise. Our case highlights, however, that microbial keratitis can be a sign of orbital or incomplete ocular ischemic syndrome and can be the presenting manifestation of GCA. To our knowledge, this is the first such case to be reported in the English-language ophthalmic literature.
Footnotes and Disclosure
The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.
A rare case of peripheral ulcerative keratitis in temporal arteritis.