Hydroxychloroquine retinopathy after short-term therapy.

PURPOSE: To report an unusual case of hydroxychloroquine toxicity after short-term therapy. METHODS: Observational case report. RESULTS: A 56-year-old woman presented to the Ophthalmology Clinic at Walter Reed Army Medical Center (WRAMC) with a 6-month history of gradually decreasing vision in both eyes. The patient had been taking hydroxychloroquine for the preceding 48 months for the treatment of rheumatoid arthritis. Examination of the posterior segment revealed bilateral "bull's eye" macular lesions. Fundus autofluorescence revealed hyperfluorescence of well-defined bull's eye lesions in both eyes. Optical coherence tomography revealed corresponding parafoveal atrophy with a loss of the retinal inner segment/outer segment junction. Humphrey visual field 10-2 white showed significant central and paracentral defects with a generalized depression. The patient was on a standard dose of 400 mg daily, which was above her ideal dose. The patient had no history of kidney or liver dysfunction. There were no known risk factors but there were several possible confounding factors. The patient was started on high-dose nabumetone, a nonsteroidal antiinflammatory drug, at the same time she was started on hydroxychloroquine. She also reported taking occasional ibuprofen. CONCLUSION: Retinal toxicity from chloroquine has been recognized for decades with later reports showing retinopathy from long-term hydroxychloroquine (Plaquenil) use for the treatment of antiinflammatory diseases. Hydroxychloroquine is now widely used and retinal toxicity is relatively uncommon. However, it can cause serious vision loss and is usually irreversible. The risk of hydroxychloroquine toxicity rises to nearly 1% with a total cumulative dose of 1,000 g, which is ∼5 years to 7 years of normal use. Toxicity is rare under this dose. For this reason, the American Academy of Ophthalmology has revised its recommendations such that annual screenings begin 5 years after therapy with hydroxychloroquine has begun unless there are known risk factors. This case report confirms the need for a baseline examination and annual ophthalmologic screening for patients taking hydroxychloroquine at a dose higher than the recommended dosage. It is also reasonable to consider annual examinations in patients taking high-dose nonsteroidal antiinflammatory drugs from the initiation of the medication.

Periorbital nodular fasciitis arising during pregnancy.

Nodular fasciitis (NF) is a benign proliferation of fibroblasts and myofibroblasts that rarely occurs in the periorbital region. We report what we believe to be the first case of periorbital NF associated with pregnancy. A case of intravascular fasciitis, a NF variant, has been reported during pregnancy, but it was not located in the periorbital region. A weak presence of estrogen receptors has been reported in NF. This may make it more susceptible to the hormone-related changes during pregnancy and contribute to the development of the lesion by stimulating fibroblasts and smooth muscle cell types. Although rare, NF should be considered in the differential diagnosis of periorbital soft-tissue masses arising during pregnancy.

Closed globe macular injuries after blasts in combat.

PURPOSE: To describe the macular findings after closed globe ocular injuries sustained from blasts. METHODS: A retrospective chart review from February 2003 to March 2010 of all soldiers with closed globe ocular injuries sustained during combat with macular findings of trauma on examination was completed. RESULTS: There were 36 eyes that met the inclusion criteria. The mean age of the soldiers was 29.5 years and 97% were men. The average follow-up time was 18.6 months. Improvised explosive device blasts accounted for 86% of injuries. Forty-five percent of soldiers had bilateral ocular injuries. Eight of 36 eyes (22.2%) developed a macular hole. One eye had spontaneous closure and five eyes underwent surgical repair. There was a range of macular findings from retinal pigment epitheliopathy alone to retinal pigment epitheliopathy with full-thickness atrophy. Eight eyes (22.2%) had macular scarring on examination but no optical coherence tomography study. One eye (2.8%) developed phthisis bulbi. Fifteen eyes (42%) had an orbital fracture. Seven eyes (19%) sustained optic neuropathy. CONCLUSION: Closed globe injuries after blasts resulted in a spectrum of macular findings. The integrity of the foveal inner segment/outer segment junction was the most important retinal factor in visual outcomes. Orbital fractures were not found to be a risk factor for developing optic neuropathy and may improve visual outcomes. The rate of long-term complications is unknown, and it is important for ophthalmologists to follow these patients closely.

A retrospective review of oral low-dose sirolimus (rapamycin) for the treatment of active uveitis.

PURPOSE: The purpose of this study is to elicit the role of oral low-dose sirolimus as a corticosteriod-sparing agent for active uveitis. METHODS: A retrospective, interventional case series was performed by reviewing the clinical records of all patients treated with oral, low-dose sirolimus (1-4 mg daily) for severe uveitis. Data reviewed included symptomatic improvement, Snellen best-corrected visual acuity, corticosteroid requirement, sirolimus levels, intraocular inflammation, spectral-domain optical coherence tomography, and fluorescein angiogram. Primary outcome measures were determined by the ability to decrease the intraocular inflammation, corticosteroid requirement, and frequency of flares. RESULTS: Eight patients with varied diagnoses were treated with oral low-dose sirolimus for severe, chronic uveitis between 2008 and 2010. In four of the eight patients, there was an improvement of all primary outcome measures. While sirolimus monotherapy was successful in only one patient, a sirolimus/methotrexate combination was successful in three patients. Although there was a good initial response in three patients, treatment was a failure after serious side effects forced the cessation of sirolimus therapy. One patient was lost to follow-up. CONCLUSION: Sirolimus may have a limited role in severe uveitis as an adjunct corticosteroid-sparing agent in combination with more standard immunosuppressive agents. Oral low-dose sirolimus appeared to be better tolerated than higher doses, but there were a significant number of adverse events, requiring therapy to be stopped.