Syphilitic uveitis is frequently the presenting manifestation of systemic syphilis caused by the spirochete bacterium Treponema pallidum. Primary syphilis is the first stage where patients develop a painless chancre, most commonly located on the genitalia, representing an accumulation of spirochetes that arises 10 to 90 days after inoculation and typically resolves 1 month later. The secondary stage arises 1 to 2 months following the primary stage. Multiple organ systems are involved, and patients can experience fever, headache, malaise, lymphadenopathy, joint pain, mouth ulcers, and characteristic maculopapular rash on the palms and soles. These manifestations typically resolve without intervention. The third stage is the latent phase, an asymptomatic period of indefinite duration. The final phase is the tertiary stage where nearly every organ system is affected. Patients may have gummas or gummata, namely a granulomatous reaction that can occur in skin, liver, bones, heart, brain, or many other tissues. Tertiary syphilis can induce widespread inflammation causing neurologic (e.g., Argyll Robertson pupil and tabes dorsalis) and cardiovascular (e.g., aortic aneurysms and mitral valve insufficiency) complications. Neurosyphilis can occur at any phase of syphilitic infection; any form of ocular involvement should be considered neurosyphilis and treated as such. Syphilitic uveitis can present in many ways, including anterior uveitis, posterior uveitis, panuveitis, vasculitis, and placoid chorioretinitis.
55.1.1 Common Symptoms
Blurry vision, photophobia, conjunctival injection, headache, epiphora, and pain.
Symptoms are similar to those of posterior syphilitic uveitis discussed below.
Blurry vision, floaters, photopsias, and eye pain.
55.1.2 Exam Findings
Anterior segment involvement is the most common with granulomatous iridocyclitis, occurring in nearly half of those with ocular syphilis. Syphilitic iridocyclitis may present either as granulomatous inflammation with large keratic precipitates, anterior chamber cell and flare, and iris nodules or as nongranulomatous anterior chamber inflammation. Synechiae, iris atrophy, elevated intraocular pressure, and the rare hypopyon may also be observed. Dilated iris vessels (iris roseolae) occur rarely but are relatively specific for this condition.
Other anterior segment findings include episcleritis, scleritis, or papillary conjunctivitis. Immune-mediated, noninfectious, nonsuppurative interstitial keratitis is considered an immune reaction and can present similarly to anterior uveitis.
While syphilitic uveitis can manifest as isolated intermediate uveitis with snowbanks and snowballs in the pars plana, it is much more common to see vitreous inflammation associated with retinal or choroidal inflammation, representing true posterior uveitis.
T. pallidum is unique in that it can affect all layers of the retina and choroid. As with anterior involvement, posterior uveitis can be focal or diffuse, unilateral, or bilateral. Posterior syphilitic uveitis can manifest as chorioretinitis, vitritis, retinal vasculitis, exudative retinal detachment, panuveitis, and rarely as necrotizing retinitis. Any combination of findings can be seen on examination, including vitreous cell and haze vascular sheathing, retinal whitening, preretinal precipitates, retinal necrosis, chorioretinitis, retinal elevation, hemorrhage, and chorioretinal infiltrate (▶ Fig. 55.1).
Fig. 55.1 Fundus photograph demonstrating diffuse vitritis with preretinal precipitates and optic nerve head edema.
Chorioretinitis is a frequent manifestation of posterior syphilitic uveitis. In its confluent form, there are large, multifocal swaths of creamy white retinal infiltrates tending to be similar in size to those seen in viral retinitis, but their cream color differs from the stark white infiltrates classic of viral infections. In the placoid form, more commonly referred to as acute syphilitic posterior placoid chorioretinitis, there is equal involvement of the outer retina and inner choroid. Large, circular, yellowish-gray areas of inflammation present are located in the deep retinal and retinal pigment epithelium (RPE) layers of the posterior pole and can be singular (▶ Fig. 55.2) or multifocal (▶ Fig. 55.3); they may have overlying vitreous inflammation or even be found adjacent to an area of exudative retinal detachment.
Fig. 55.2 Fundus photograph highlighting a large confluent peripapillary chorioretinal plaque-like infiltrate secondary to syphilis (i.e., posterior placoid chorioretinitis). (Photo courtesy of Petros E. Carvounis, MD.)