Abstract

FR.20.02

Characteristics, complications and therapeutic options in Birdshot chorioretinopathy

Roland Rieger, Peter C. Ruokonen, Klaus Rüther, Uwe Pleyer

Augenklinik, Campus Virchow Klinikum, Charité - Universitätsmedizin Berlin, Berlin

Objective
Birdshot chorioretinopathy is a rare bilateral posterior uveitis of presumeably autoimmunological origin. Monotherapy with corticosteroids is often insufficient and limited due to the known adverse side effects. Therefore Steroid-sparing strategies with more specific agents such as cyclosporine (Cyclosporin A, CSA) have been suggested as first line treatment for this disease. Aim of this study is to analyse the clinical course of patients receiving immunomodulatory therapy.
Methods
A retrospective analysis of the clinical course of 13 patients suffering from bilateral chorioretinopathy and expressing HLA- A29 was performed by evaluating visual acuity, fundoscopy, optical coherence tomography, fluorescent angiography and in selected cases electroretinography.
Results
Age at onset ranged from 40 to 63 years (mean, 53 years) in 4 men and 9 women. The median follow-up from disease onset was 32 months. After initial treatment with corticosteroids longterm stabilization of the clinical course could only be achieved by steroid-sparing immunomodulatory therapy. 6 patients were treated with low-dose (2.5-5 mg/kg/d) CSA alone and 8 received Myophenolat as systemic immunosuppressive therapy. Vitreous inflammation was controlled in 16  treated eyes and improvement or stabilization of visual acuity was achieved in 14 eyes. Nephrotoxic side effects or hypertension due to CSA therapy developed in 6 patients.
Conclusions
Due to steroid-sparing immunomodulatory therapy stabilization of the clinical course of patients suffering from birdshot chorioretinopathy can be achieved in many cases. Adequate therapy is currently hindered due to difficulties associated with the off-label use of some immunomodulatory agents.

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