IMMUNE RECONSTITUTION INFLAMMATORY SYNDROME CAUSING PROGRESSIVE OPTIC NERVE EDEMA IN CRYPTOCOCCAL MENINGITIS
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Published on: August 21, 2017
Astrid C Werner1, Laurel N Vuong2, Thomas R Hedges2
1Department of Ophthalmology, Tufts New England Eye Center, Boston, Massachusetts.
Purpose:
To report an human immunodeficiency virus-positive patient undergoing therapy for cryptococcal meningitis who developed progressive optic disk edema that was steroid responsive.
Methods:
Observational case report.
Results:
One month after commencing antifungal treatment for cryptococcal meningitis, the patient developed bilateral, progressive, recurrent optic disk edema with subretinal fluid that coincided with initiation of highly active antiretroviral therapy and recovery of CD4 cell counts. Lumbar puncture revealed normal opening pressure, and cerebrospinal fluid showed no recurrence of cryptococcal infection. There was no evidence of uveitis. The patient rapidly improved with a 5-day course of high-dose intravenous methylprednisolone.
Conclusion:
Recurrent optic disk edema with loss of vision after treatment of cryptococcal meningitis in the setting of normal intracranial pressure may represent a unique manifestation of immune reconstitution inflammatory syndrome localized to the optic nerve without uveitis. This is consistent with the temporal relationship between starting highly active antiretroviral therapy, CD4 count recovery, and the development of progressive disk edema in the study patient. Isolated optic nerve inflammation as a manifestation of immune reconstitution inflammatory syndrome has not been widely reported.
Insights
A human immunodeficiency virus-positive patient with cryptococcal meningitis developed optic disk edema. This condition, potentially a rare form of immune reconstitution inflammatory syndrome, resolved with steroid treatment.
Area of Science:
- Ophthalmology
- Infectious Diseases
- Neurology
Background:
- Cryptococcal meningitis is a serious opportunistic infection in human immunodeficiency virus (HIV)-positive individuals.
- Immune reconstitution inflammatory syndrome (IRIS) can occur during antiretroviral therapy, leading to paradoxical worsening of infections or new inflammatory conditions.
- Optic nerve involvement in IRIS, particularly without uveitis, is uncommon.

