posner-schlossman syndrome bianka sobolewska, md manfred zierhut, md centre of ophthalmology...

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April 2010: First Presentation April 2010: First Presentation  VA: OD 20/63, OS 20/20  IOP: OD 38 mmHg, OS 17 mmHg  OD: non-granulomatous central KPs, iris pigment defect, anterior chamber cells 1+, posterior subcapsular cataract, the iridocorneal angle open with pigment  OS: regular  Fundus:  OD/OS: regular with physiologic excavation of the optic nerve

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Posner-Schlossman Syndrome Bianka Sobolewska, MD Manfred Zierhut, MD Centre of Ophthalmology University of Tuebingen, Germany Ocular History 49 year old healthy white women 1999: OD recurrent anterior uveitis IOP up to 60 mmHg no response to oral aciclovir April 2010: First Presentation April 2010: First Presentation VA: OD 20/63, OS 20/20 IOP: OD 38 mmHg, OS 17 mmHg OD: non-granulomatous central KPs, iris pigment defect, anterior chamber cells 1+, posterior subcapsular cataract, the iridocorneal angle open with pigment OS: regular Fundus: OD/OS: regular with physiologic excavation of the optic nerve non-granulomatous central KPs April 2010: First Presentation First Presentation - Diagnostics negative anterior chamber fluid analysis (PCR) for CMV Epstein-Barr virus herpes simplex virus varicella zoster virus all other tests negative: serology for syphilis, borreliosis and Bartonella, Quantiferon test, ANA, ANCA, chest CT Diagnosis CMV- indduced anterior uveitis based on: Clinical findings with classical Posner-Schlossman Syndrome behaviour of intraocular pressure Missing response to acyclovir Treatment topical antiglaucomatosa prednisolone eye drops (3x/day) systemic valganciclovir 3 weeks: 900 mg b.i.d. followed by 450 mg b.i.d. Follow up After 1-6 Months Mai-October 2010 no recurrence since the start of valganciclovir therapy VA: OD 20/1000 IOP: OD mmHg only two old KPs, no cells, posterior subcapsular cataract therapy: reduction of topical therapy systemic valganciclovir 450 mg b.i.d clear corneal phacoemulsification 11/2010 Follow up After 7 Months October 2011 OD: 20/20 no recurrence termination of valganciclovir therapy Follow up After 11 Months March 2012 recurrence with IOP of 40 mmHg, anterior chamber cells 1+ therapy valganciclovir (450 mg 2x/day topical rimexolone (4x/day) topical ganciclovir (5x/day) acetacolamide (2x125 mg/day) Follow-up After 1-2 Years April 2012 April 2013 IOP mmHg no recurrence reduction of topical therapy termination of oral therapy in Follow-up After 28 Months August 2013 recurrence with IOP of 60 mmHg acetacolamide 2x250 mg/day start therapy with leflunomide 20mg/d topical therapy with antiglaucomatosa, prednisolone 3x/day ganciclovir 3x/day Follow-up After 29 Months September 2013 twice recurrences with IOP of 60 mmHg stop of leflunomide (headache, nausea) topical therapy with antiglaucomatosa prednisolone 3x/day ganciclovir 3x/day Follow-up After Months October 2013-October 2014 no recurrences reduction of topical therapy Final Ocular Investigation After 42 Months October 2014 VA: 0.7 anterior chamber no cells optic disc: physiological excavation no topical treatment besides arteficial tears Final Diagnosis Possner-Schlossman Syndrome (PSS) Differential diagnosis Herpetic anterior uveitis (HSV, VZV) Fuchs` uveitis Problems rare disease often misdiagnosed negative AC tap (PCR) does not exclude PSS unclear treatment regimen when AC tap is negative recurrences after termination of systemic valganciclovir therapy often reported Conclusion CMV anterior Uveitis Clinical signs can mimic other viral uveitis disorders When AC tap is negative but the clinical signs are suggestive for PSS, systemic acyclovir ineffective: systemic valganciclovir is recommended In case of recurrences, oral valganciclovir therapy with additional topical ganciclovir can be repeated In long-lasting undiagnosed PSS treated only with aciclovir, the response to valgancilovir seems limited