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HIV patient presents with painful unilateral visual loss
Yellow choroidal lesions suggestive of infiltrates, peripheral choroidal detachments and significant macular edema were seen in the left eye.
A 47-year-old Hispanic man presented with a 4-day history of painful unilateral visual loss. He reported “everything seems to appear darker” and suffered from a “boring pain” in the left eye. Ocular history was significant for an episode of central serous chorioretinopathy 9 years earlier in the left eye that resolved with observation.
 Shazia Ahmed |  My Hanh T. Nguyen |
The patient had a diagnosis of HIV for 16 years and was on highly active antiretroviral therapy (HAART) for 10 years. His most recent CD4 count was 280 cells/µL, and he had an undetectable viral load. The patient had no known history of an AIDS-defining opportunistic infection. Medical history was significant for hypertension, diabetes, arrhythmia, depression and Reiter’s syndrome. Medications included lamivudine/zidovudine, ritonavir, atazanavir, doxazosin, propranolol, metformin, pravastatin, testim, citalopram and eszopiclone. Drug allergies included bactrim and keflex. Review of systems was significant for an irregular heartbeat, urinary difficulties, arthritis and depression. No neurological or dermatologic abnormalities were noted.
Examination
The patient’s best corrected visual acuity was 20/20 in the right eye and 20/200 in the left eye. Pupils were reactive to light without afferent pupillary defects. Motility was full; however, “soreness” was noted on movements of the left eye. IOP measured 14 mm Hg bilaterally by applanation tonometry.
Slit lamp examination of the anterior segment was unremarkable in both eyes. There were no inflammatory cells in either the anterior chamber or vitreous cavity. Fundus examination of the right eye demonstrated scattered drusen and chronic retinal pigment epithelium pigmentary changes (Figure 1a). Examination of the left eye demonstrated multiple yellow choroidal lesions suggestive of infiltrates, peripheral choroidal detachments and significant macular edema (Figures 1b and 1c). Fluorescein angiography of the right eye demonstrated several punctate areas of mild hyperfluorescence in the late frames (Figure 2a). Fluorescein angiography of the left eye revealed large areas of hyperfluorescence correlating with the locations of the choroidal lesions with pooling and leakage in the later frames (Figures 2b to 2d). Central foveal thickness was normal in the right eye and 965 µm in the left eye measured by optical coherence tomography (Figure 3). B-scan ultrasonography of the left eye showed thickening of the posterior choroid and anterior choroidal detachments (Figures 4a and 4b).
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| Color fundus photograph of the right eye showed drusen and retinal pigment epithelium pigmentary changes. | Color fundus photographs of the left eye revealed multiple yellow perivascular infiltrative choroidal lesions (1b) and peripheral anterior choroidal detachments (1c). | Fluorescein angiography of the right eye demonstrated several punctate areas of mild hyperfluorescence in the late frames. | |
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| Multiple pinpoint areas of hyperfluorescence with pooling and leakage in the later frames of the angiogram of the left eye | OCT of the left eye demonstrated diffuse subretinal fluid. | ||
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| B-scan ultrasonography showed thickening of the posterior choroid (4a) and peripheral choroidal detachments (4b). Images: Yoon SJ, Duker JS | |
What is your diagnosis?
HIV patient
The medical history of the patient is notable for HIV and Reiter’s syndrome. The possibility of opportunistic infections and immune-mediated inflammatory reactions is important in the differential diagnosis.
The history of central serous chorioretinopathy (CSCR) in this patient suggests the possibility of an atypical recurrence. Medication-induced side effects are also important to consider, with the possibility of a steroid-induced exacerbation of CSCR, an adverse reaction to a sulfa-based drug or an unknown side effect to HAART. An atypical presentation of immune recovery uveitis could also be considered, despite the negative history of cytomegalovirus retinitis or severely depressed CD4 counts.
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Fluorescein angiography findings of multiple pinpoint areas of hyperfluorescence suggest Vogt-Koyanagi-Harada syndrome and sympathetic ophthalmoplegia as important possibilities as well. Thickening of choroid and peripheral choroidal detachments on ultrasonography suggests consideration for posterior scleritis.
A workup for systemic infection and inflammation was performed, including CBC, ANCA, ANA, FTA-ABS, ACE, HLA-B27, RF, ESR, Lyme titers, urinalysis and chest X-ray. The patient was HLA-B27 positive, and the remaining systemic workup was within normal limits.
Diagnosis
A definitive diagnosis at this point was not determined. The lack of anterior or vitreous inflammation, predominantly unilateral presentation, and the lack of associated trauma, dermatologic involvement or neurological involvement made a noninfectious panuveitis, such as sympathetic ophthalmoplegia or Vogt-Koyanagi-Harada syndrome, less likely. The history of Reiter’s syndrome and HLA-B27 seropositivity made posterior scleritis a strong consideration. The treatment of choice was systemic immunosuppression. However, it was noted that the possibility of exacerbation of an opportunistic infection or worsening of CSCR could occur with systemic corticosteroids. Discussion was made with the patient concerning these possibilities.
The patient was started on high-dose oral prednisone (80 mg daily) with close clinical observation. The patient had rapid resolution of pain within days, and visual acuity improved to 20/60 at 2 weeks follow-up with substantial decrease in subretinal fluid (Figure 5a). Resolution of subretinal fluid and choroidal detachments occurred after 1 month of treatment (Figures 5b and 5c). A 3-month steroid taper was initiated with subsequent return of the patient’s visual acuity to 20/20 in the affected eye.
Discussion
Posterior scleritis is a rare and under-recognized disease in ophthalmology due to its variable clinical presentation and posterior location. It occurs in all ages; however, patients older than 50 years have an increased risk of visual loss and association with systemic disease. Visual loss occurs in one-third of patients, and clinical features include periocular pain, pain with eye movement, decreased vision and symptoms associated with anterior scleritis. Signs of posterior scleritis are variable and include associated anterior scleritis, serous retinal detachment, swollen optic disc, elevated IOP and choroidal effusions.
This patient presented with various manifestations of posterior scleritis supported by clinical findings, ultrasonography and the rapid response to corticosteroid therapy. The prior diagnosis of CSCR and potential for opportunistic infections related to HIV were important to consider in this case, especially regarding the therapeutic decision to use an immunosuppressant medication to treat this entity.
The patient’s history of Reiter’s syndrome, HIV and CSCR made this case unique in many ways. The association between Reiter’s syndrome and anterior scleritis has been reported in up to 3% of patients; however, the association with posterior scleritis has not been described. Only one case report has described an association between CSCR and HIV, and no known association between HIV and posterior scleritis has yet been determined.
For more information:
- Steven J. Yoon, MD, and Jay S. Duker, MD, can be reached at New England Eye Center, Tufts University School of Medicine, 750 Washington St., Box 450, Boston, MA 02111; 617-636-4219; fax: 617-636-4866; Web site: www.neec.com.
- Edited by Shazia Ahmed, MD, and My Hanh T. Nguyen, MD. Drs. Ahmed and Nguyen can be reached at New England Eye Center, Tufts University School of Medicine, 750 Washington St., Box 450, Boston, MA 02111; 617-636-4219; fax: 617-636-4866; Web site: www.neec.com. Drs. Ahmed and Nguyen have no direct financial interest in the products mentioned in this article, nor are they paid consultants for any companies mentioned.
References:
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- McCluskey PJ, Watson PG, et al. Posterior scleritis: clinical features, systemic associations, and outcome in a large series of patients. Ophthalmology. 1999;106(12):2380-2386.
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