Behçet´s Disease Christoph Deuter Centre for Ophthalmology, University Hospital, Tuebingen, Germany

History  25, female, German origin  emergency referral from external hospital  OU: retinitis, visual impairment, visual field defects

General History  common cold with fever, 39°C,  generalized exanthema,  purulent genital ulcerations,  dizziness  treatment: Aciclovir and Ganciclovir intravenously, low dose prednisolone

First Presentation – Ocular Examination VA: OD 20/125, OS 20/50 VA: OD 20/125, OS 20/50 anterior chamber: unremarkable anterior chamber: unremarkable IOP: 16/16 mmHg IOP: 16/16 mmHg

First Presentation - Fundus  OU: retinal infiltration of macular region

First Presentation - Investigations  oral and genital ulcers  papulopustulosis  retinitis / retinal vasculitis both eyes  HLA-B51 positive  pathergy test negative  bilateral vestibularis affection

Diagnosis  complete Behçet´s Disease with ocular and neurological involvement  based on:  clinical findings  fullfills the Classification Criteria of the International Study Group (1990)

Treatment Aciclovir and Ganciclovir discontinued Aciclovir and Ganciclovir discontinued IFN alpha-2a (4.5 mio IU per day) IFN alpha-2a (4.5 mio IU per day) Prednisolone (10 mg per day continued) Prednisolone (10 mg per day continued)

Follow up – After 2 Weeks OD VA 20/50 OS VA 20/20

Follow up – After 6 Months  VA: OD 20/20, OS 20/20  free of recurrences  current treatment  IFN alpha-2a 3 mio IU every 2nd day  prednisolone 5 mg /day

Follow up – After 6 Months  normal fundus without signs of inflammation

Follow up – After 5 Years  VAA: OD 20/25, OS 20/20  free of recurrences  current treatment  off IFN alpha-2a since 4 years,  prednisolone 5 mg /day

Final follow up – After 5 years  Normal fundus, no signs of inflammation

Conclusion Typical for uveitis with Behcet´s Disease are retinal infiltrations, occlusive retinal vasculitis Typical for uveitis with Behcet´s Disease are retinal infiltrations, occlusive retinal vasculitis In case of retinal involvement it is recommended to start biological treatment with TNF-alpha blocking agents or alpha-interferon In case of retinal involvement it is recommended to start biological treatment with TNF-alpha blocking agents or alpha-interferon This may result in long term remission even after stop of treatment This may result in long term remission even after stop of treatment