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Syphilitic uveitis misdiagnosed as viral retinitis—a misleading history
© The Author(s). 2018
- Received: 20 August 2018
- Accepted: 18 November 2018
- Published: 4 December 2018
Syphilitic uveitis is an infective uveitis and a great mimicker. Misdiagnosis can lead to delay in the specific treatment resulting in deterioration of uveitis and loss of vision.
A 38-year-old unmarried female presented with pain, redness, and blurring of vision in the left eye for the last 5 days. She denied history of any sexual exposure in the past. Anterior segment examination of the right eye was normal and the left eye showed keratic precipitates with anterior chamber cells and iris pigments on anterior lens capsule. Fundus examination of the right eye showed a hyperemic disc with posterior placoid retinochoroiditis and the left eye showed dense vitritis, hyperemic disc, and superficial retinal precipitates. She was misdiagnosed as viral retinitis elsewhere and started on antivirals with oral corticosteroids which resulted in deterioration of uveitis and progression to bilateral involvement. Further systemic investigations confirmed the diagnosis of syphilis and human immunodeficiency virus infection. She was then started on anti-syphilitic and anti-retroviral therapy which resulted in restoration of the vision in one eye.
Syphilitic uveitis does not occur in primary disease and is common in secondary and early latent phase of syphilis. History given by the patient is often contributory however at times can be misleading. A high clinical suspicion and thorough investigation is necessary for the correct diagnosis and timely intervention in preventing loss of vision.
- Human immunodeficiency virus
Acquired syphilis is a sexually transmitted disease caused by spirochetal bacterium Treponema pallidum. It affects most of the organ systems including skin, heart, blood vessels, bone, nervous system, and eye [1, 2]. Ocular syphilis has varied manifestations and may present as anterior uveitis, posterior uveitis, panuveitis, retinitis, papillitis, and even scleritis, making it a “great masquerade” . It may result in visual loss if diagnosed and treated as a non-infectious uveitis. We report a case of syphilitic uveitis misdiagnosed as viral retinitis resulting in loss of vision in one eye.
She was advised investigations but was lost to follow-up for 1 month and diagnosed elsewhere as viral retinitis. Investigations done showed decreased white blood cell (WBC) count—3980 mm3 and raised erythrocyte sedimentation rate (ESR)—35 mm/h. Mantoux test − 0 mm induration after 72 h. She was started on oral valacyclovir 1 g three times a day with topical prednisolone acetate 1% and oral corticosteroids 1 mg/kg weight which she was using for the last 2 weeks.
Syphilis is a sexually transmitted chronic disease caused by the spirochete Treponema pallidum, which may spread via mother-to-child transmission (congenital syphilis) or acquired in adulthood (acquired syphilis). Syphilitic uveitis is an infectious uveitis. It does not occur in primary disease and is the most common in the secondary stage of acquired syphilis.
Ocular involvement by syphilis has varied manifestations such as iridocyclitis, papillitis, retinochoroiditis, retinal pigment epithelitis, serous retinal detachment, cystoid macular edema, vitreous opacity, and neuroretinitis [5, 6]. The most common manifestation being retinochoroiditis, in which infiltration of inflammatory cells from the choroidal capillaries, afflicting the Bruch’s membrane and retinal pigment epithelium, is thought to constitute the pathological progression .
Ocular syphilis is a great mimicker [8, 9]. Misdiagnosis may lead to an undue delay in initiating the treatment resulting in deterioration of uveitis and patient’s general health. Our patient was misdiagnosed as viral retinitis and started on anti-viral therapy and corticosteroids elsewhere resulting in worsening with progression to bilateral involvement and a permanent damage to the left eye with no recovery of vision. The clinical signs were very classical of syphilitic uveitis especially the presence of superficial retinal precipitates which are pathognomonic of syphilitic uveitis; however, syphilitic uveitis is more common in the males  and our patient was an unmarried female who denied any sexual exposure in the past. This was an important misleading history given by the patient leading to improper investigations and delay in confirmation of the diagnosis. The diagnosis of ocular syphilis leads to the diagnosis of HIV in our patient. As reported in literature, the frequency of syphilitic uveitis in HIV-infected individuals has significantly increased in recent years [11, 12]. Initiation of both anti-syphilitic and anti-retroviral therapy resulted in resolution of the retinochoroiditis and salvaging the vision in at least one eye.
Various risks factors associated with combined syphilis and HIV infection as reported in the literature include homosexual male, age group 18–30 years, multiple sexual partners [13, 14]. Our patient did not fit into any of the common risk factors and also denied history of any sexual exposure which led to delay in the diagnosis.
On review of literature, we found a similar case reported by Zambon F et al. , where a case of syphilitic uveitis was misdiagnosed as acute retinal necrosis (ARN) and started on antivirals and oral corticosteroids leading to no light perception in the affected eye.
Diagnosis of syphilitic uveitis or viral retinitis may be difficult in the presence of severe vitritis. Syphilitic retinitis is a form of necrotizing retinitis [6, 16]. The common manifestation is in the form of one or more yellowish white patches of necrosis, often associated with vasculitis, vitreous inflammation, and anterior segment inflammation. This presentation often mimics ARN of herpetic origin. However, in ARN, the necrotic lesions start in the peripheral retina, whereas in syphilitic retinitis they are often located at the posterior pole. In syphilitic retinal necrosis, one has the impression that the surface of the lesion is somewhat indistinct, as if a layer of exudates obscure the underlying retina from view, whereas in ARN one can clearly identify the surface of the lesions as the surface of the thickened, necrotic retina. The retinal necrotic tissue tends to be homogeneous in ARN, whereas the areas of necrosis in syphilitic retinitis have a mottled aspect that becomes even more obvious in the healing phase. Superficial retinal precipitates are pathognomonic of syphilitic retinitis and are absent in ARN. Most often these two entities are diagnosed clinically; however, in case a doubt exists, we may confirm through vitreous sampling and subjection to polymerase chain reaction (PCR).
Syphilitic uveitis shows a good response to penicillin; however, one may encounter an increased ocular inflammation known as Jarrisch Herxheimer reaction after the initiation of the treatment requiring oral corticosteroids. However in our case, the general health of the patient was not good with underlying HIV infection and she was intolerant to oral corticosteroids (esophageal reflux disease); therefore, it was decided to stop them by the treating physician.
We report this case as syphilis which is uncommon in our country; however, there has been a resurgence in the last few years. We routinely investigate our patients based on clinical suspicion and the history given by the patient is often contributory however at times like in our case was misleading, leading to a delay in the management. We need to be aware of the varied manifestations of syphilis and suspect immunosuppression when a patient presents with a zero Mantoux reading in a tuberculosis endemic country like ours. We need to have a high level of clinical suspicion and thoroughly investigate the patient before initiating corticosteroid therapy in an infective uveitis as it may lead to delay in a specific treatment and result in permanent loss of vision.
We have received no funding for this work from any organization. We have no financial interest to disclose.
Availability of data and materials
MA provided ophthalmic care to the patient and drafted the manuscript. RR drafted the manuscript and reviewed the literature. LP provided ophthalmic care to the patient. DS provided ophthalmic care to the patient. All the authors have reviewed the manuscript and approved the same.
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