We report a case of a fifty three year old female born in the Dominican Republic, migrated to the Netherlands 33 years prior, with no relevant medical history, who presented to the ophthalmologist with pain and redness around the right eye, initially classified as a scleritis. She was treated with topical steroids and referred to the rheumatologist for further evaluation of possible underlying auto-inflammatory syndromes or infectious diseases. She tested positive for syphilis by positive chemiluminescence immunoassay (CLIA) screening and Treponema pallidum hemagglutination assay (THPA), with a negative rapid plasma antigen (RPR) test, supporting the diagnosis of a preexisting latent or tertiary syphilis infection. She reported no earlier syphilis infection and no prior antibiotics use.
At this time it was unclear whether the syphilis infection was directly related to the reported scleritis of the right eye. Our patient did not report any promiscuous sexual relations and tested negative for other sexually transmitted diseases (STDs) such as Chlamydia trachomatis, gonorrhea, hepatitis B and C. Importantly, she also tested negative for HIV, which is often simultaneously present as a co-infection. She was planned to receive intramuscular benzyl penicillin (2.4 million units, administered three times at one week intervals) as a treatment for latent syphilis. Also, her partner tested negative for syphilis, HIV and other STDs at his general practitioner.
Shortly thereafter, she presented with an acute episode of loss of vision in de left eye, upon ophthalmological examination a measured vision of 0.3, with papilledema/papillitis. She also reported a unilateral, left sided headache, with jaw claudication and left sided pain in the scalp, clinically highly suspect for Giant Cell Arteritis (GCA, also known as arteriitis temporalis). Laboratory results however, showed a mildly elevated C-reactive protein (CRP) of 33 mg/l and erythrocyte sedimentation rate (ESR/BSE) of 40 mm/hour and other laboratory findings were normal (Table 1). Lumbar puncture (Table 1) showed no spirochete activity in the liquor cerebrospinalis (negative THPA-test and negative RPR test without signs of inflammation). As a GCA can cause permanent blindness if not treated swiftly, she was admitted to our hospital and empirically started on high-dose intravenous methylprednisolone (1000 mg per 24 h) during three days according to national and local guidelines. An atypical presentation of an anterior ischemic optic neuropathy (AION) or non-arteritic anterior optic neuropathy (N-AION) was also considered, but appeared less likely clinically. While using steroids, a concomitantly diagnosed latent tuberculosis infection was treated with isoniazide/rifampicine (600 mg/300 mg once daily for four months).
Surprisingly, further evaluation using positron emission tomography (PET-CT) and biopsy of the temporal artery showed no signs of GCA, after which the prednisone was quickly tapered and stopped. Moreover, PET-CT did not show any other signs of inflammatory/infectious activity throughout the body. After multidisciplinary reevaluation, we concluded that the most likely diagnosis is a tertiary stage ocular infection of syphilis, also known as syphilitic uveitis. She was subsequently treated with high-dose intravenous benzyl penicillin (24million units/24 h) during two weeks. A few weeks later, she reported markedly improved vision, with eventually no residual vision loss.