SYPHILITIC UVEITIS: A THERAPEUTIC CHALLENGE.
Uveitis is the most common ocular manifestation of acquired syphilis. Syphilitic uveitis is estimated to account for approximately 1% of all cases of uveitis1, with often polymorphic and atypical manifestations. Since syphilitic uveitis has no specific pattern of ocular involvement2 so a high index of suspicion is mandatory for its diagnosis. Here we present a case of syphilitic uveitis who posed us a therapeutic challenge due to its severity, recurrences and relative resistance to topical and oral steroids. Due to aggressive treatment by intravitreal steroids and immunosuppressive therapy and frequent follow up visits, vision of the patient was salvaged.
A 38 year old male patient, resident of Peshawar, presented in eye OPD of CMH Peshawar with complaints of progressive blurring of vision, floaters and mild pain in both eyes. He denied any history of ocular trauma or surgery. He was non diabetic and had no history of joint pain, oral or genital ulcer, urethral discharge, drug abuse, sexual malpractice and contact with cats or puppies. His best corrected visual acuity (VA) in right eye was 6/36 on Snellen's chart and VA in left eye was counting finger (CF) at one foot distance. Slit lamp biomicroscopic examination of both eyes revealed keratic precipitates (KP), severe inflammatory reaction in anterior chamber and vitreous humour (cellular reaction +3), posterior synechiae and opacities in the inferior part of vitreous. Due to vitreous haze, clear view of fundus was not possible. Intraocular pressure was 18 and 16 mm Hg in right and left eye respectively.
His systemic examination showed maculopapular rash on trunk but no oral or genital lesion or ulcer was found. His laboratory investigations including erythrocyte sedimentation rate (ESR), angiotensin converting enzyme (ACE), Venereal Disease Research Laboratory (VDRL), fluorescent treponemal antibody absorption test (FTA-ABS), chest radiograph, antinuclear antibodies (ANA) and HIV serology were carried out. Results of VDRL and FTA-ABS were positive with titres of 1:4 and 1/320 respectively. Rest of the investigations was normal. This clinical workup and lab reports led to the diagnosis of syphilitic uveitis. Consultation of a dermatologist was sought who prescribed him intravenous aqueous penicillin G, 18 million units daily for 2 weeks. The patient was prescribed tablet prednisolone acetate 70mg daily, topical prednisolone acetate 1% eye drops 2 hourly and atropine eye drops 1% 8 hourly.
Subtenon injection of triamcinolone acetate 40mg/ml was administered in both eyes. Ocular condition of the patient did not improve over next 4 weeks. Optical coherence tomography (OCT) of both eyes was performed (Fig-1 and 2).
Injection triamcinolone acetate 4 mg/0.1ml was injected into right eye vitreous. After 3 weeks it was administered in left eye vitreous. These intravitreal injections of triamcinolone were repeated in both eyes after 3 months. Dose of oral prednisolone was gradually tapered and then stopped. His intraocular inflammation gradually reduced. Topical prednisolone 1% was also gradually tapered. But unfortunately after 3 months his visual acuity deteriorated again. On ocular examination of both eyes, marked cellular reaction in anterior chamber and bilateral posterior subcapsular cataract was observed. The combination of oral and topical prednisolone along with atropine was instituted. Patient was also prescribed tablet Azathioprine, 150mg daily. After 2 weeks, ocular condition of the patient improved clinically and so oral prednisolone was gradually tapered over next 2 weeks and stopped. Dose of topical prednisolone was also tapered but maintained at one drop daily.
The intraocular inflammation completely settled within 10 weeks. A month after controlling inflammation he underwent cataract extraction along with intraocular lens implantation in both eyes. Presently patient's both eyes are quiet and without any cellular reaction for the last 4 months. His best corrected visual acuity is 6/9 in each eye and he is taking tab Azathioprine 300 mg daily only.
Syphilitic uveitis was first described by Nicolaus Leonicenus, an Italian physician, at the end of the 15th century. Its causative agent a spirochete, Treponema pallidm, was first isolated in 1905 from the skin of infected people. Over the past few years, there has been a significant resurgence of syphilis4 especially in Western population and an increased number of patients with ocular syphilis have been reported2. Intravenous drug abuse and HIV infection has contributed significantly in resurgence of syphilis. HIV-infected patients usually have more florid and accentuated ocular disease with increased chances of recurrences5, 6.
Acute anterior uveitis occurs in about 4% of cases of secondary syphilis and is bilateral in 50% of cases3, though the severity may be asymmetrical. Syphilitic posterior uveitis may present as multifocal chorioretinitis, acute posterior placoid chorioretinitis4, neuroretinitis and retinal vasculitis. A correlation between syphilitic uveitis and neurosyphilis has been documented by several studies. That is why several authors have suggested that in patients with ocular syphilis especially in those with active chorioretinitis and papillitis, lumbar puncture for CSF evaluation be performed to rule out asymptomatic neurosyphilis3,5. A high index of suspicion is required for the diagnosis of syphilitic posterior uveitis. It is important to consider syphilis in the differential diagnosis of any posterior uveitis because delay in the institution of treatment can lead to permanent loss of vision.
Specific treponemal serologic testing of either the fluorescent treponemal antibody absorption test7 (FTA-ABS) or the microhemagglutination assay for antibodies to Treponema pallidum (MHA-TP) must be carried out even if the nontreponemal serologic test (VDRL or RPR) is negative3.
Penicillin is the antibiotic of choice for the treatment of syphilis5. The current recommended regimen for syphilitic uveitis or neurosyphilis is intravenous aqueous penicillin G, 18-24 million units daily for 10-14 days3. As alternative, intramuscular procaine penicillin 2.4 MU daily for 10-15 days is advocated. Benzathine penicillin G alone is not recommended in the treatment of ocular syphilis3 because of low drug levels in the CSF, presumably reflecting low tissue levels in the eye and increased chances of relapse in both immunocompetent and HIV-infected hosts.
Awareness of the multiple presentations of ocular syphilis will aid ophthalmologists in averting misdiagnosis or delayed diagnosis. Long-term ophthalmologic evaluation and follow-up serologic tests are important in the management of syphilitic eye disease.
1. Michitake S, Yukihiro S. A Case of Syphilitic Uveitis. Nippon Ganka Kiyo 2007;58:389-92.
2. Diaz-Valle D, Toledano N, Miguelez R, Barros C. Bilateral anterior uveitis as a presenting manifestation of sarcoidosis and syphilis. Br J Ophthalmol 2002;86(8):930-1.
3. Villanueva AV, Sahouri MJ, Ormerod LD, Puklin JE, Reyes MP. Posterior Uveitis in Patients with Positive Serology for Syphilis. Clin Infect Dis 2000;30:479-85.
4. Chao JR, Khurana RN, Fawzi AA, Reddy HS, Rao NA. Syphilis: reemergence of an old adversary. Ophthalmology 2006;113(11):2074-9.
5. Amaratunge BC, Camuglia JE, Hall AJ. Syphilitic uveitis: a review of clinical manifestations and treatment outcomes of syphilitic uveitis in human immunodeficiency virus-positive and negative patients. Clin Experiment Ophthalmol 2010;38(1):68-74.
6. Tran TH, Cassoux N, Bodaghi B, Fardeau C, Caumes E, Lehoang P. Syphilitic uveitis in patients infected with human immunodeficiency virus. Graefes Arch Clin Exp Ophthalmol 2005;243(9):863-9.
7. Akram A, Ameen SS, Naqvi A, Syed ZU, Niazi MK. Management Tips for Uveitis. Pak J Ophthalmol 2010;26:44-7.
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|Publication:||Pakistan Armed Forces Medical Journal|
|Date:||Sep 30, 2012|
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