Ocular Syphilis

Ocular Syphilis
SYMPTOMS Decreased central vision, Metamorphopsia, Visual field loss, Floaters, Flashes, Eye pain, Photophobia
SIGNS Interstitial keratitis, Episcleritis, Scleritis, Dilated iris vessels (known as iris roseolae), Granulomatous or non-granulomatous anterior uveitis, Posterior synechiae, Panuveitis or Posterior uveitis (most common sign), Vitritis, White retinal precipitates, Retinal vasculitis, Chorioretinitis, Acute syphilitic posterior placoid chorioretinopathy, Optic neuropathy, Neuroretinitis, Necrotizing or non-necrotizing retinitis, Cystoid macular edema, Epiretinal membrane, Secondary Glaucoma (inflammatory), Ocular Hypertension Syndrome, Retinal detachment, Retinal vein occlusion, Argyll Robertson pupil
Active uveitis is associated with secondary and tertiary syphilis
WORK-UP Pupils, Full eye exam with dilated retinal exam, IOP with GAT or ORA, Gonioscopy, OCT analysis of the macula, OCT analysis of the optic nerve/RNFL/GCL, OCT-Angiography, Visual field with Humphrey 24-2/30-2/24-2C threshold or 10-2 threshold in more advanced glaucoma, Fluorescein Angiography, Indocyanine Green Angiography, Fundus Autofluorescence, Fundus photos, Infrared photos, B-scan ultrasound, Pachymetry (thin corneas carry a higher risk of glaucoma), Corneal hysteresis (low hysteresis carries a higher risk of glaucoma), VEP/Pattern ERG (decrease in ganglion cell function), Watzke-Allen test, Macular photostress test, Amsler grid
TREATMENT Refer to PCP/Neurology for a full neurologic exam, lab testing including testing for HIV and other sexually transmitted diseases, and treatment (IV or IM Penicillin G)
The key to preventing and treating ocular syphilis is treating the systemic syphilis itself
Give take home Amsler grid in order to monitor for change
Interstitial keratitis secondary to syphilis: Begin topical steroid (Prednisilone acetate or Durezol). If ineffective, begin Cyclosporine or Tacrolimus (if recurrent, Cyclosporine or Tacrolimus should be used over a topical steroid)
Episcleritis and/or scleritis secondary to syphilis: Topical and oral steroids show minimal or no benefit. The key for resolution is treating the syphilis itself
If patient presents with an anterior uveitis with or without posterior synechiae, the patient should be started on a topical steroid (Prednisolone acetate or Durezol) and a topical cycloplegic (refer to retinal specialist ASAP as patient will most likely have a panuveitis, posterior uveitis, and/or retinal complications as well)
Secondary glaucoma and ocular hypertension syndrome secondary to syphilis: Begin topical steroid (Prednisilone acetate or Durezol), Cyclopentolate 1%, and IOP lowering medications
First-line of treatment would typically be a combo drop which includes Cosopt 1 gtt BID, Combigan 1 gtt BID, or Simbrinza 1 gtt TID
If adjunct drop needed, consider Rhopressa which is to be used 1 gtt QHS
Topical carbonic anhydrase inhibitors to be used 1 gtt BID. alpha-2-agonists to be used 1 gtt BID, and/or topical beta blockers to be used 1 gtt once a day in the morning up to BID (the evening dose is thought to be minimally effective) can be considered as adjunct treatment (as long as class of medication is not currently being used in the first-line combo glaucoma drop)
Topical carbonic anhydrase inhibitors and alpha-2-agonists are to be used 1 gtt TID if utilized as stand-alone therapy
Topical apraclonidine and oral acetazolamide can be given in-office or on a short-term basis to quickly lower IOPs
Prostaglandins, pilocarpine, and Rocklatan (due to prostaglandin component) should be avoided
Always keep in mind the contraindications and side effects before prescribing any glaucoma medications
Steroids should only be given to a patient with syphilis when the patient is already on antibiotics (using steroids without antibiotic coverage could exacerbate the disease)
Patients will most likely need to be co-managed with a glaucoma specialist
If patient presents with ocular syphilis with active posterior inflammation and/or retinal complications such as cystoid macular edema and a retinal detachment, refer to a retinal specialist ASAP for further evaluation and treatment
If patient presents with ocular syphilis with signs of neurosyphilis such as optic neuropathy and/or argyll robertson pupil, refer to a neuro-ophthalmologist for further evaluation and treatment
FOLLOW-UP Interstitial keratitis secondary to syphilis: Patient should be seen back in 1-2 weeks for a follow-up until resolution
Episcleritis and/or scleritis secondary to syphilis: Patient should be seen back in 1-2 weeks for a follow-up until resolution
Secondary glaucoma and ocular hypertension syndrome secondary to syphilis: Patient should be seen back in 1-2 weeks for follow-up until intraocular pressure returns to normal (may have to use glaucoma drops on a permanent basis and get glaucoma testing every 3-4 months). Patient may also continue care with the glaucoma specialist
Once the patient's ocular syphilis is treated by the retinal specialist and/or neuro-ophthalmologist and the ocular inflammation is no longer active with a stable retina/macula/optic nerve, the patient should be seen back every 3-6 months
ADDITIONAL LAB | TESTS VDRL/RPR, FTA-ABS/MHA-TA/T, Pallidum-particle agglutination (TP-PA), Lumbar puncture and cerebrospinal fluid analysis (if suspecting neurosyphilis), CD4 count, Viral load, CBC with differential
ETIOLOGY Sexually transmitted disease caused by the spirochete Treponema pallidum
DIFFERENTIAL DX Acute retinal necrosis, Ocular toxoplasmosis, White dot syndromes, Progressive outer retinal necrosis, Ocular tuberculosis
NOTES Ocular syphilis is known as “the great masquerader” as its presentation can mimic many other ocular inflammatory conditions
Ocular syphilis can occur during any stage of syphilis
A panuveitis is more common to see if a patient is also HIV positive and a posterior uveitis is more common to see if a patient is HIV negative
Syphilis can be congenital as well (typically transferred through the placenta). Signs include bilateral interstitial keratitis (most common sign), “Salt and pepper” fundus, Hutchinson teeth, and Deafness
Ocular Syphilis: Fundus photo demonstrating acute syphilitic posterior placoid chorioretinopathy https://imagebank.asrs.org/file/269/acute-syphilitic-posterior-placoid-chorioretinitis