CDC clinical advisory: Be on the lookout for ocular syphilis cases in the United States

Executive Summary

Since December 2014, 24 cases of ocular syphilis have been reported from California and Washington, with several other states reporting potential cases, according to the Centers for Disease Control and Prevention (CDC).

• Most cases have been among HIV-infected men who have sex with men, with a few cases occurring among HIV-uninfected persons, including heterosexual men and women, according to a CDC advisory.
• Ocular syphilis can involve almost any eye structure, with posterior uveitis and panuveitis the most common manifestations, followed by anterior uveitis, optic neuropathy, retinal vasculitis, and interstitial keratitis. Ocular syphilis might lead to decreased visual acuity, including permanent blindness.

Since December 2014, 24 cases of ocular syphilis have been reported from California and Washington, with several other states reporting potential cases, according to the Centers for Disease Control and Prevention (CDC). Most cases have been among HIV-infected men who have sex with men, with a few cases occurring among HIV-uninfected persons, including heterosexual men and women, according to a CDC advisory. (See the advisory, updated as of April 16, 2015, at http://1.usa.gov/1GCzphe.) Several of the cases have resulted in significant sequelae, including permanent blindness.

At press time, no further cases had been identified in Washington or San Francisco since the advisory was updated on April 16, confirms Robyn Neblett Fanfair, MD, MPH, a medical epidemiologist in the CDC’s Division of STD Prevention. However, California has several unconfirmed, possible cases under review, she notes.

Ocular syphilis is a manifestation of neurosyphilis, which is an infection of the brain or spinal cord. It usually occurs in people with untreated syphilis, states Fanfair. It can occur during any stage of syphilis, including primary and secondary syphilis. Ocular syphilis can involve almost any eye structure, with posterior uveitis and panuveitis the most common manifestations, followed by anterior uveitis, optic neuropathy, retinal vasculitis, and interstitial keratitis. Ocular syphilis can lead to decreased visual acuity, including permanent blindness.

A previous study among HIV-positive men who have sex with men (MSM) found that the estimated risk for having symptomatic early neurosyphilis was 1.7%, notes Fanfair.¹ Most cases the CDC has seen this year are among MSM, she states.

“Syphilis is on the rise in the United States, in particular among MSM, and neurosyphilis can occur at any stage of syphilis,” says Fanfair. “We are working with select jurisdictions to determine how 2015 case counts compare to previous years to see whether this is a significant increase from previous years or an artifact of increased awareness.”

One agency’s response

Six people in Washington have been diagnosed with ocular syphilis since mid-December 2014, with four of them in King County, states Matt Golden, MD, director of the HIV/STD Program at Public Health – Seattle & King County in Seattle. All cases occurred in MSM; three of the men also had HIV infection. At press time, the health department had not identified any additional cases of ocular syphilis since January, he states. Two of the individuals have permanent loss of vision, though they have had some recovery with therapy, notes Golden.

The Public Health – Seattle & King County’s HIV/STD unit has worked to alert healthcare providers, as well as members of the community, about ocular syphilis, says Golden. “We issued a healthcare alert to healthcare providers and sent a letter about the problem to all medical providers who reported a case of STD in 2014,” Golden states. “We also have worked with community-based organizations, particularly those that serve gay and bisexual men, to raise awareness of ocular syphilis among MSM and have also communicated with other health departments in the region and with the CDC.” (Take a look at the agency’s communications with providers and the community at http://1.usa.gov/1zSibKL. The provider fact sheet is accessed at http://1.usa.gov/1HpReDk. The community fact sheet is at http://1.usa.gov/1Gjlhv2.)

Public health officials continue to provide syphilis testing in the agency’s STD clinic, through community-based testing sites, and in bathhouses serving MSM, says Golden.

Public Health – Seattle & King County created its SyphilisRising campaign in a response to the rise of syphilis in the area, particularly among gay and bisexual men. In the early 1990s, syphilis was almost eliminated in King County. Now the agency sees almost 300 cases per year, and nine out of 10 infections are in MSM.

According to the agency’s SyphilisRising website, www.syphilisrising.com, there are more new cases of syphilis each year than HIV in the area. Syphilis rates are 15 times higher in MSM who have HIV than in MSM who do not have HIV.

Symptoms are subtle

The symptoms of primary syphilis, caused by the bacterium Treponema pallidum, are easy to miss and easy for patients to mistake for something else.

Primary syphilis presents as a painless, raised open sore (chancre) on the genitals, mouth, or rectum. The sore usually shows up one to three weeks after exposure, although it can sometimes take months to appear. While the sore can last several weeks and go away by itself, the infection is not gone.

Syphilis is passed from person to person through direct contact with a syphilis sore. Sores occur mainly on the external genitals, vagina, anus, or in the rectum. Sores also can occur on the lips and in the mouth. Transmission of the organism occurs during vaginal, anal, or oral sex. Correct and consistent use of latex condoms can reduce the risk of syphilis only when the infected area or site of potential exposure is protected.

Initial symptoms of ocular syphilis also can be subtle and can include:

• blurry vision;
• floaters (spots that float by through one’s vision);
• a blue tinge in vision;
• flashing lights;
• eye pain.

If not treated, these symptoms can progress to loss of vision. If treated early, the symptoms usually will go away. However, delayed treatment can result in permanent blindness.

The CDC classifies the case definition for an ocular syphilis case as follows: a person with clinical symptoms or signs consistent with ocular disease (i.e. uveitis, panuveitis, diminished visual acuity, blindness, optic neuropathy, interstitial keratitis, anterior uveitis, and retinal vasculitis) with syphilis of any stage.

Clinicians should be aware of ocular syphilis and screen for visual complaints in any patient at risk for syphilis, including men who have sex with men, HIV-infected persons, others with risk factors, and persons with multiple or anonymous partners, advises the CDC. All patients with syphilis should receive an HIV test if their status is unknown or they previously were HIV-negative.

Patients with positive syphilis serology test and early syphilis without ocular symptoms should receive a careful neurologic exam including all cranial nerves, states the CDC. Those patients with syphilis and ocular complaints should receive immediate ophthalmologic evaluation. A lumbar puncture with cerebrospinal fluid (CSF) examination should be performed in patients with syphilis and ocular complaints, according to the CDC.

How should the disease be managed? Use treatment recommendations for neurosyphilis, which include aqueous crystalline penicillin G by intravenous therapy or procaine penicillin by intramuscular injection with probenecid for 10-14 days, the CDC advises. Cases of ocular syphilis should be reported to your state or local health department within 24 hours of diagnosis. Ocular syphilis cases diagnosed since Dec. 1, 2014, should be reported through local or state health department to the CDC by e-mail at [email protected]. If possible, pre-antibiotic clinical samples (whole blood, primary lesions and moist secondary lesions, CSF, or ocular fluid) should be saved and stored at -80°C for molecular typing.

Clinicians who need advice from the CDC regarding clinical management of ocular syphilis or assistance with shipment of clinical samples for molecular typing may contact Fanfair at (404) 639-6044 or [email protected].

REFERENCE

1. Centers for Disease Control and Prevention. Symptomatic early neurosyphilis among HIV-positive men who have sex with men — four cities, United States, January 2002-June 2004. Morb Mortal Wkly Rep 2007; 56(25):625-628.