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Ocular , the Great Masquerader Justin Lewis, MD; Christina Flaxel, MD Oregon Health & Science University

[ ] Introduction Macular Edema Throughout Treatment Course Discussion • Syphilis typically advances through four • Ocular syphilis can present with any visual different stages symptom, thus internists should be on the • PrimaryDevelopment of a New Practice Model lookout for signs that will guide us towards the • Secondary diagnosis • Latent • Early treatment drastically improves • Tertiary outcomes • One retrospective chart review determined • Ocular syphilis can develop during any of that visual acuity improved significantly in these stages! 89% of patients treated • It is a rare condition that can involve any • The main factor associated with poor structure of the eye and mimic a variety prognosis was over 28 days of ocular of eye diseases: symptoms before diagnosis (1) • • Early recognition and treatment is key! • Diminished visual acuity • Optic neuropathy • Detecting ocular syphilis early is difficult • because of its varied symptomatology • One case series reported that macular edema was present in 52% of ocular syphilis Case Description patients (2) • Another case series reported that uveitis was • A 47-year-old man with no PMH presented with the most common symptom, and that case six months of decreasing vision in his right eye • The patient displayed evidence of a Jarisch-Herxheimer reaction via Optical series featured six patients with retinal • Initial ophthalmology exam revealed cystoid References [ ] Coherence Tomography (OCT) imaging detachment (3) macular edema 1. [ ] • Pre-penicillin: OCT displays mild cystoid macular edema • As internists, if we see mention of such • He was treated with local steroids with symptoms on chart review in a patient with • 17 days post-treatment: OCT displays paradoxical worsening of minimal relief, and then developed retinal unspecified ocular symptoms and high-risk detachment and macular edema sexual behavior, then a syphilis work-up • After retina surgery, his vision did not • This is secondary to lysis of organisms, release of endotoxin-like lipoproteins, should be pursued! improve and he had worsening inflammation and increase in TNF-a, IL-6, and IL-8 • A broad serological workup later revealed RPR • Note: although OCT shows worsening of macular edema during this stage, • While ocular syphilis is typically associated with of 1:128 and +FTA, prompting hospital clinical symptoms improved with the IV penicillin (PCN) neurosyphilis, this is not always the case admission • 28 days post-treatment: improved macular edema, completely resolved • A case series determined that only 22% of • He denied STIs, but had penile lesions in the patients with ocular syphilis had past and had a history of high-risk sexual neurological symptoms (3) behavior Review: Stages and Treatment of Syphilis • Thus, a benign neurologic exam does not • Physical exam showed diffuse, pruritic, rule out ocular syphilis, as was the case with maculopapular scattered throughout this patient! his body (see below) Clinical Treatment Post-treatment • The patient later endorsed that the rashes manifestations monitoring [ ] had been present for roughly three months Teaching Points prior to admission • Early diagnosis and treatment (within 28 days of symptom onset) is key to good patient outcomes • Primary: single painless • Penicillin G • Clinical exam and blood Early syphilis +/- regional benzathine IM x1 testing with RPR at 6 and • Patients respond well to IV penicillin G lymphadenopathy • Alternatives: Doxycycline, 12 months • If a patient presents with macular edema, • Secondary: systemic ceftriaxone, tetracycline, illness + (often palms amoxicillin + probenecid uveitis and/or retinal detachment, think of and soles) ocular syphilis, especially if they have high- • Early latent: infected by risk sexual behavior blood testing, but no symptoms (< 1yr) • The majority of patients with ocular syphilis have benign neurological exams, thus not a • Tertiary: late syphilis • Penicillin G • Clinical exam and blood rule out test! Late syphilis with cardiovascular benzathine IM once a testing with RPR at 6, 12, • Visual acuity was 20/400 right eye, 20/30 left manifestations and week for 3 weeks and 24 months gummatous disease • Alternatives: doxycycline References eye with bilateral uveitis • Late latent: infected by or ceftriaxone • Neurological exam was benign blood testing, but no 1) Tsuboi M, et al. Prognosis of ocular syphilis in patients • Lumbar puncture showed 101 WBC (86% symptoms (> 1 yr) infected with HIV in the antiretroviral therapy era. ), with CSF VDRL titer 1:8 Sexually Transmitted . 2016;92(8):605-610. • The patient was started on IV penicillin G and • Early Neurosyphilis: can • Aqueous penicillin G • Clinical exam and blood 2) Shen J, Feng L, Li Y. Ocular syphilis: an alarming continued on anti-inflammatory eye drops Neurosyphilis have , IV for 10-14 days testing with RPR infectious eye disease. International Journal of Clinical • He completed a 14-day infusion of IV (can occur at meningovascular disease • Patients allergic to (frequency depends on and Experimental Medicine. 2015;8(5):7770-7777. (meningitis + ), vision penicillin should be stage) Penicillin G, with progressive improvement in or hearing loss 3) Oliver SE, Aubin M, Atwell L, et al. Ocular Syphilis – desensitized • May need to do CSF any time of • Late neurosyphilis: Eight Jurisdictions, United States, 2014 – 2015. MMWR his visual and dermatologic symptoms • Alternatives: ceftriaxone testing course) involves the brain and spinal Morb Mortal Wkly Rep 2016;65:1185-1188. • Follow up labs showed decreased RPR titer cord (dementia and tabes dorsalis)