Cytomegalovirus-related Scleritis

Scleritis is an uncommon inflammatory disease, which might be associated with ocular complications in 60% of cases and decrease in visual acuity in 17% of patients.1 Approximately 40–50% of patients with scleritis have or will have an associated systemic disorder, while infections are responsible for up to 10% of cases.1,2 Scleritis is bilateral in 41% of the cases, and posterior in 39% of cases.2 Posterior scleritis is defined as an involvement of the sclera posterior of the insertion of the recti muscles. Severe pain is present in 55% of patients and visual disturbance is the commonest reason for seeking treatment. Serous retinal detachment, subretinal mass, choroidal retinal folds, annular choroidal detachment, optic disc and macular edema, and myositis are the typical features of posterior scleritis, although up to 20% of patients with ultrasound evidence of posterior scleritis show no abnormalities in the posterior segment of the eye.3 Specific investigations for posterior scleritis include B-scan ultrasonography, fluorescein angiography, nuclear magnetic resonance imaging, and laboratory investigations.3 More recently, OCT, a complete noninvasive technique, has been indicated as a useful tool for both diagnostic and therapeutic evaluation.3–6

Case Report

We report the case of a 46-year-old woman who presented to the emergency room after suffering from moderate pain and redness in both eyes for 2 days, with fever and headache lasting for 10 days previously treated with ceftriaxone 1 g/day. Past systemic history was positive for a post-surgical pelviperitonitis 9 years before. Best-corrected visual acuity was 10/10 in both eyes, and a bilateral conjunctival hyperemia was present. No anterior chamber or vitreous inflammation or pupillary defects were present. Intraocular pressure was normal in both eyes. Fundus examination revealed in both eyes mild disc edema and hyperemia, macular edema and choroidal folds. B-scan ultrasonography demonstrated bilateral diffuse scleral thickening. OCT showed wavy appearance of the whole retinochoroidal complex, increased choroidal thickness, choroidal and retinal folds, and accumulation of fluids underneath the neurosensory retina (Figure 1). Fluorescein angiography presented bilateral retinal staining around the optic disc; visual field displayed a blind spot enlargement. Body temperature was 37.3 °C and physical examination was normal. A treatment with prednisone 50 mg/day was started immediately, associated with the therapy previously taken (ceftriaxone). Serology obtained subsequently was positive for recent cytomegalovirus (CMV) infection (IgM 1.91 IU/mL and IgG 15.900 IU/mL), while only IgG were detected for herpes simplex virus 1, Epstein-Barr virus, and varicella zoster virus. Serology was completely negative for herpes simplex virus type 2 and HIV. Erythrocyte sedimentation rate, C-reactive protein, CD T-cell count, immunoglobulins (IgG, IgA, IgM), angiotensin-converting enzyme, and chest x-ray were normal; antinuclear antibody, antineutrophil cytoplasmic antibodies, rheumatoid factor, VDRL, TPHA, and Mantoux test were negative. Nuclear magnetic resonance of the brain showed cicatricial white-matter lesions in FLAIR and T2-weighted images, without gadolinium enhancement. Neurologic examination was normal and the lesions were defined unrelated to ocular disease. In 2 days body temperature returned to normal, and 7 days after starting therapy the patient was symptom-free, optic disc was normal, and choroidal folds were less evident. After serology resulted positive for CMV, antibiotic therapy was stopped and acyclovir 2.4 g/day for 14 days was administered while oral prednisone was gradually tapered by 5 mg every 10 days. Two weeks later ophthalmoscopy, OCT (Figure 2) and B-scan ultrasonography showed normal findings, and bilateral best-corrected visual acuity was 10/10. In the following 18 months of follow-up after therapy discontinuation, no ocular or neurologic/neuroradiologic changes were detected.

FIGURE 1. (a) Right and (b) left eye: SD-OCT horizontal scan showing remarkable retinochoroidal folds.

FIGURE 2. (a) Right and (b) left eye: SD-OCT images showing choroidal fold resolution 14 days after the therapy.

Discussion

Posterior scleritis is reported to be associated with systemic disease in 36% of cases.2 Rheumatoid arthritis is the most common associated disease, followed by HLAB27-associated uveitis with spondiloarthropathy, Wegener granulomatosis, relapsing polycondritis, arthritis and inflammatory bowel disease, systemic lupus erythematosus, and other systemic diseases. Viruses are the most frequent cause of infectious scleritis, followed by bacteria, parasites, and fungi.2 Among viruses the two most common causes of scleritis are varicella zoster and herpes simplex virus, the former concomitant with herpes zoster ophthalmicus. Herpetic-related scleritis usually presents as an anterior scleritis7 and no previous case of cytomegalovirus-associated bilateral posterior scleritis has been described in an immunocompetent patient. Nevertheless, cytomegalovirus might theoretically be the pathogen responsible for a scleritis, being detected in the sclera of an HIV patient with CMV retinitis treated with ganciclovir8 and in the sclera after intraocular inoculation in a murine model of CMV infection.9 A bilateral posterior scleritis was diagnosed in our immunocompetent patient 10 days after an acute cytomegalovirus systemic infection, thus demonstrating that even in patients with normal immunity CMV infection might be responsible for scleritis. This CMV-associated scleritis responded successfully to a combination of acyclovir and prednisone, probably because of the immune status of the patient. OCT is a reproducible and noninvasive technique that helps in the diagnosis of posterior scleritis showing a wavy appearance of the retina and choroid and the presence of folds at the retinal/RPE/choriocapillaris levels.5 Furthermore, OCT can detect the amount of fluid in the subretinal space and adequately monitor the complete resolution of inflammation,5,6 as was confirmed in our patient 2 weeks after starting therapy (Figure 2). In our patient with CMV-related scleritis we have found a bilateral posterior scleritis, normal visual acuity at presentation, and no sign of keratitis or uveitis, somehow different from herpes simplex and herpes zoster-related scleritis, which are more frequently unilateral, mostly associated with keratitis or uveitis, and with a significant decrease in visual acuity at presentation.7

Declaration of interest

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.