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Case Reports and Small Case Series
September 1998

Progressive Outer Retinal Necrosis in a Patient With Rheumatoid Arthritis

Arch Ophthalmol. 1998;116(9):1249. doi:

Progressive outer retinal necrosis (PORN) is a distinct variant of herpetic necrotizing retinopathy caused by the varicella zoster virus that has been seen almost exclusively in patients with end-stage acquired immunodeficiency syndrome. Progressive outer retinal necrosis is characterized by initial geographic opacification of the deep peripheral retina without prominent vasculitis or vitritis followed by rapid progression to involve the entire retina.1 The prognosis is dismal; the visual acuity in most eyes becomes no light perception despite the institution of antiviral therapy, although there are several case reports of successful treatment if initiated early.

We describe a patient with rheumatoid arthritis who had a clinical presentation and course consistent with PORN.

Report of a Case

A 62-year-old woman was seen with a 3-day history of progressive vision loss first in the left eye and then in the right eye. Review of systems revealed a 2-week history of fatigue and weakness/paresthesias in her left leg. Her medical history included a 13-year history of rheumatoid arthritis, which 3 years prior to presentation developed into a systemic vasculitis. She had been quiescent since chlorambucil and prednisone therapy was initiated. She had a remote history of cutaneous zoster in an unknown dermatome. Ocular history included successful cataract surgery in both eyes.

Visual acuity on presentation was 20/40 OD and 20/50 OS with a severely restricted peripheral visual field in both eyes (10°-15°centrally). Pupils reacted sluggishly but had no afferent defect. Intraocular pressure, motility, and external examination results were normal. Slitlamp examination revealed pseudophakia with cells (1+ to 2+) in the anterior chamber and no vitritis. Indirect ophthalmoscopy revealed midperipheral intraretinal hemorrhages, bilateral disc edema with hemorrhages (greater in the left eye than in the right eye), and a discrete area of retinal opacity of the deep retinal layers in the temporal periphery without active vasculitis in the left eye.

Despite the administration of high-dose intravenous acyclovir sodium (12.4 mg/kg 3 times a day) instituted on hospital day 2, the retinal opacity progressed to involve the entire retina during the next 2 days (Figure 1). By 3 days after admission to the hospital, she had no light perception OU that persisted 2 years later, when marked gliosis of the retina was noted. Magnetic resonance imaging was normal. Results of an extensive laboratory evaluation for new rheumatologic and infectious conditions (including human immunodeficiency virus) were negative with the exception of an elevated protein level of 1250 g/L (125 g/dL) in the cerebrospinal fluid. The lower extremity weakness was diagnosed as an unrelated transverse myelitis. A CD4+ count was not obtained, but the patient was lymphopenic (absolute lymphocyte count, −164 cells per microliter [reference range, 150.0-300.0×109/L]).

Fundus photograph of the left eye on day 2 of admission shows a far-advanced patchy opacification of the deep retina without significant vasculitis typical of progressive outer retinal necrosis.

Fundus photograph of the left eye on day 2 of admission shows a far-advanced patchy opacification of the deep retina without significant vasculitis typical of progressive outer retinal necrosis.


Although proof of varicella zoster infection was not obtained, the course and appearance of this disease is characteristic of PORN. The presence of optic nerve swelling preceding the peripheral retinal changes is unusual, but this clinical picture has been reported for varicella in patients with acquired immunodeficiency syndrome.2 Unfortunately, the diagnosis was not made until hospital day 2, when it was too late to salvage any vision despite antiviral treatment.

To our knowledge, this is the second case of PORN that has been described outside of patients with acquired immunodeficiency syndrome; a 15-year-old bone marrow transplant patient receiving multiple immunosuppressive medicines for active graft-vs-host disease was reported with a retinitis consistent with PORN.3 Our case is a further reminder that PORN must be kept in the differential diagnosis for a rapidly progressive retinitis in all immunosuppressed patients, regardless of etiology.

This investigation was supported in part by an unrestricted grant from Research to Prevent Blindness Inc, New York, NY; the Wisconsin Lions Foundation, Rosholt; and the National Eye Institute, National Institutes of Health, Bethesda, Md, to the Department of Ophthalmology and Visual Sciences at the University of Wisconsin–Madison.

Corresponding author: Frank L. Myers, MD, 2870 University Ave, Suite 206, Madison, WI 53705-3611.

Engstrom  RE  JrHolland  GNMargolis  TP  et al.  The progressive outer retinal necrosis syndrome: a variant of necrotizing herpetic retinopathy in patients with AIDS. Ophthalmology. 1994;1011488- 1502Article
Friedlander  SMRahhal  FMEricson  L  et al.  Optic neuropathy preceding acute retinal necrosis in acquired immunodeficiency syndrome. Arch Ophthalmol. 1996;1141481- 1485Article
Lewis  JMNagae  YTano  Y Progressive outer retinal necrosis after bone marrow transplantation. Am J Ophthalmol. 1996;122892- 895