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