Long-term follow-up of patients with birdshot retinochoroidopathy treated with systemic immunosuppression.
OBJECTIVE
Birdshot retinochoroidopathy (BRC) is a rare uveitis syndrome of presumed autoimmune etiology. Therapy with systemic and periocular corticosteroids is of inconsistent efficacy, attendant with numerous potential long-term side effects. Corticosteroid-sparing strategies with agents such as cyclosporine A or azathioprine have been suggested for this disease.


METHODS
We retrospectively reviewed the medical charts of patients with BRC who were evaluated consecutively at a tertiary-care, referral-based North American uveitis clinic over a 15-year period.


RESULTS
Eleven Caucasian patients (22 eyes) were diagnosed with BRC, representing approximately 1% of all cases seen at the uveitis clinic. HLA-A29 was positive in all 11 patients. We elected to treat five patients with azathioprine, methotrexate, cyclosporine A, mycophenolate mofetil, and/or IvIg, as well as systemic or periocular corticosteroid injections. The median period of follow-up for the five treated patients was six years (range: 8 months-13 years). Inflammation was reduced or stabilized in five of five patients.


CONCLUSIONS
Although the definitive strategy for the management of BRC is unknown, control of intraocular inflammation and preservation of vision is possible with corticosteroid-sparing immunosuppressive agents.
Becker
Matthias D
MD
Casey Eye Institute, Oregon Health Sciences University, Portland, OR, USA. Matthias.Becker@urz.uni-heidelberg.de
Wertheim
Michael S
MS
Smith
Justine R
JR
Rosenbaum
James T
JT
D000328:Adult; D001327:Autoimmune Diseases / Q000188:drug therapy; D001379:Azathioprine / Q000627:therapeutic use; D015862:Choroid Diseases / Q000188:drug therapy*; D016572:Cyclosporine / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005260:Female; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007165:Immunosuppression Therapy; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008297:Male; D008727:Methotrexate / Q000627:therapeutic use; D008875:Middle Aged; D009173:Mycophenolic Acid / Q000031:analogs & derivatives / Q000627:therapeutic use; D012164:Retinal Diseases / Q000188:drug therapy*; D012189:Retrospective Studies; D013577:Syndrome; D016896:Treatment Outcome; D014605:Uveitis / Q000188:drug therapy*
D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D016572:Cyclosporine; D009173:Mycophenolic Acid; D001379:Azathioprine; D008727:Methotrexate