Don't Forget What You Can't See: A Case of Ocular Syphilis

Monica I. Lee, MD; Annie W.C. Lee, MD; Sean M. Sumsion, MD; Julie A. Gorchynski, MD

Disclosures

Western J Emerg Med. 2016;17(4):473-476. 

In This Article

Discussion

This is the case of a 24-year-old male who was assumed to be otherwise healthy presenting with painless vision loss due to uveitis as an ocular manifestation and presenting symptom of syphilis and concurrently found to be HIV positive. Ocular syphilis is a slow painless decrease in vision and there are no signs that are pathognomonic.[12,13] A case report suggests a triad of headache, red eye or eye pain, and elevated erythrocyte sedimentation rate should prompt clinical suspicion for ocular syphilis.[12] The AR pupil is highly specific for neurosyphilis, but as in this case it was not present. AR pupil has historically been associated with neurosyphilis but it has also been associated with diabetic retinopathy, multiple sclerosis, Wernicke's encephalopathy, Dejerine-Sottas hypertrophic neuritis, Charcot-Marie-Tooth disease, herpes zoster, Lyme disease, sarcoidosis, midbrain lesions, and von Economo's encephalitis.[12,14] Other clinical findings of neurosyphilis may include third and sixth cranial nerve palsies, and visual field defects from brain involvement.[12]

Uveitis may manifest during secondary or tertiary syphilis, with iritis being the most common ocular finding in secondary syphilis.[5,7,8,15] Syphilitic uveitis is the most common presentation of syphilis in older adults.[3,6,8] The findings on the dilated eye exam were consistent with posterior uveitis, but non-specific for syphilis. However, in the setting of positive serum RPR and CSF leukocytosis and titers positive for VDRL confirmed the diagnosis of syphilitic uveitis, and other infectious and rheumatologic etiologies were concurrently excluded.[7,8,15,16] The differential at the time of presentation included but was not limited to the following: Lyme disease, sarcoidosis, tuberculosis (TB), toxoplasmosis, toxocariasis, bartonella, brucellosis, herpes simplex virus, inflammatory bowel disease and rheumatologic conditions such as juvenile idiopathic arthritis and human leukocyte antigen (HLA)-B27-associated disease.[12]

Uveitis is a state of inflammation involving the uvea (iris, ciliary body, choroid) or retina. This may be caused by autoimmune conditions, infections, or trauma, but up to 50% of cases are idiopathic.[8] Regardless of etiology, uveitis represents a breach in the blood-ocular barrier. Disruption of this barrier is the result of inflammation, a breakdown that allows neutrophils and other inflammatory mediators to incite the acute phase of uveitis. Both the anterior and posterior chambers, as well as the vitreous cavity, are susceptible to uveitis. Identification of the predominantly involved location can narrow the differential. Anterior uveitis (iritis, iridocyclitis), is primarily due to rheumatologic and idiopathic etiologies with herpes being the most common infectious cause. White blood cells invade the aqueous humor, with inflammatory changes often resulting in precipitation of inflammatory cells (neutrophils or macrophages) on the posterior cornea (keratic precipitates), as well as other iris changes. Intermediate uveitis or pars planitis, is rare and commonly idiopathic, but when present it is classically associated with multiple sclerosis. Posterior uveitis (choroiditis, retinitis, chorioretinitis, retina vasculitis) is more commonly associated with an infectious cause in up to 40% of reported cases with pathogens that include syphilis, toxoplasmosis, and cytomegalovirus. Panuveitis is the rarest form of uveitis that involves the entirety of the eye, which comprises only 10% of all cases of uveitis. Infectious causes include syphilis, TB and endophthalmitis, either bacterial or fungal.[8,15,16]

The presence of HIV may alter the presentation of syphilis, with possibly a more rapid progression to neurosyphilis.[1] Syphilis is an important facilitator of HIV transmission with current reported co-infection rates of 50–70%.[5,7,17] In 2001 the prevalence of syphilis was at its nadir, but infection rates have since reached their highest levels since 1995. A review of the current epidemiology of syphilis has described the syphilis epidemic occurring primarily among men, especially MSM.[1–4]

Similar case reports were published primarily in the ophthalmology literature in the late 1990s and early 2000s but none recently. [20–22] Following are the CDC guidelines for the treatment regimen, which have been in place since 2010, and new recommendations for LP for ocular syphilis.

Why Should Emergency Physicians be Aware of This?

As emergency physicians we commonly see patients with vision complaints who have not had any prior evaluation by an ophthalmologist or a primary care physician. For this reason, ocular syphilis should be considered in all patients presenting to the ED with non-traumatic bilateral vision loss, especially among MSM. 2010 CDC guidelines recommend that any ocular manifestations of syphilis now be treated as neurosyphilis regardless at which stage it occurs with a 14-day course of IV Penicillin G. In addition, an LP is no longer required for CSF VDRL titers to determine the treatment regimen as previously had been required. However, an LP is still recommended for analysis of other concomitant CSF infections since syphilis has a high co-infection rate with HIV. With the current syphilis epidemic it is important for emergency physicians to recognize ocular manifestations of syphilis, and to order emergency department RPR and HIV tests in order to facilitate an urgent ophthalmology consultation, early diagnosis and treatment and to prevent permanent vision loss.

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