Birdshot Retinopathy Treatment & Management

  • Author: C Michael Samson; Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: Nov 28, 2011
 

Medical Care

The appropriate level of treatment is determined by the severity of the inflammation. Conflicting reports exist regarding the efficacy of steroids. Some patients with mild inflammation may respond well to regional injection of steroids. Other patients require the use of systemic prednisone for control of the inflammation. Some patients may be controlled on less than 10 mg/d, while other patients require higher doses. Long-term treatment, even 10 mg/d of steroids, is undesirable, considering the high risk of significant morbidity and mortality of such treatment. Many patients show no significant response to steroid therapy.

Cyclosporine has been shown to have a beneficial effect on birdshot retinochoroidopathy inflammation in retrospective case series. Initial reports demonstrated improved visual acuity, decreased vitritis, and stabilization of eyes with cyclosporine dosages of 10 mg/kg/d. However, this dose also was associated with a high incidence of nephrotoxicity and hypertension. Vitale and colleagues reported a series of 19 cases of birdshot retinochoroidopathy, which demonstrated that cyclosporine treatment with lower dosages, from 2.5-5 mg/kg, can be effective.[8] This series showed control of vitreal inflammation in 88.5% of eyes and improved or stable visual acuity in 83.3% of eyes. However, the low incidence of drug toxicity was most striking; there were only 2 cases of hypertension and no cases of nephrotoxicity.

One suggestion is to initially start cyclosporine dosages at 2.5 mg/kg and then to increase to the level necessary to control the inflammation, while ensuring avoidance of drug adverse effects. The maximum dosage is 5 mg/kg according to this author. Monitoring for blood counts and renal function is performed every 4-6 weeks, along with blood pressure monitoring. Cyclosporine serum levels are not followed at these dosing regimens. Other potential adverse effects, such as hirsutism, paresthesias, tremor, and gingival hyperplasia, are not risks for morbidity, but are mentioned, since lowering of drug dosage or discontinuation of the medication may be indicated if such adverse effects occur to a point of affecting the quality of the patient's life.

One study reports the use of ketoconazole as adjunct therapy to cyclosporine. Ketoconazole delays metabolism of cyclosporine; hence, it may lower the dose of cyclosporine required to maintain control of inflammation. Silverstein and Wong demonstrated that cyclosporine trough levels could be maintained in a patient when the cyclosporine dosage was dropped from 200 mg/d (3 mg/kg) to 50 mg/d (0.75 mg/kg) with the addition of ketoconazole at 200 mg/d. This amounts to an 80% reduction of cyclosporine consumption. While this may be cost-saving, one cannot necessarily equate stabilization of cyclosporine serum levels with adequate control of inflammation nor with reduced potential toxicity. After all, the serum cyclosporine levels are still in the therapeutic range, and one might expect cyclosporine toxicity prevalence to be unchanged. Additionally, ketoconazole is not without potential adverse effects, especially the risk of hepatitis.

Other immunomodulatory therapies have been described. Kiss and colleagues reported the use of mycophenolate mofetil, azathioprine, methotrexate, and daclizumab in a series of 28 patients with birdshot retinochoroidopathy; however, the small size of the study precludes any comment on the efficacy of any single drug.[9, 10] LeHoang and colleagues reported the use of intravenous immunoglobulin in a series of 18 patients as initial therapy for active birdshot retinochoroidopathy, and they noted stable vision in 33 of 36 eyes over a mean follow-up period of 39 months.[11]

Proceed to Medication
 
 
Contributor Information and Disclosures
Author

C Michael Samson  MD, Associate Professor, Department of Ophthalmology, New York Medical College; Consulting Staff, Co-director of Uveitis Service, Director, Uveitis Fellowship, Department of Ophthalmology, New York Eye and Ear Infirmary; Director, Adesso Biosciences, Ltd.; President and CEO, CLS Pharmaceuticals; Private Practice, Vitreous Retina Macula Consultants of New York

C Michael Samson is a member of the following medical societies: American Academy of Ophthalmology, American Medical Association, American Uveitis Society, and Association for Research in Vision and Ophthalmology

Disclosure: Nothing to disclose.

Coauthor(s)

Amro Mohamed Mohamoud Ali, MB, ChB  Consulting Staff, New York Eye and Ear Infirmary

Amro Mohamed Mohamoud Ali, MB, ChB is a member of the following medical societies: American Academy of Ophthalmology

Disclosure: Nothing to disclose.

C Stephen Foster, MD, FACS, FACR, FAAO  Clinical Professor of Ophthalmology, Harvard Medical School; Consulting Staff, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary; Founder and President, Ocular Immunology and Uveitis Foundation, Massachusetts Eye Research and Surgery Institution

C Stephen Foster, MD, FACS, FACR, FAAO is a member of the following medical societies: Alpha Omega Alpha, American Academy of Ophthalmology, American Association of Immunologists, American College of Rheumatology, American College of Surgeons, American Federation for Clinical Research, American Medical Association, American Society for Microbiology, American Uveitis Society, Association for Research in Vision and Ophthalmology, Massachusetts Medical Society, Royal Society of Medicine, and Sigma Xi

Disclosure: Nothing to disclose.

Specialty Editor Board

Russell P Jayne, MD  Consulting Vitreoretinal Surgeon, The Retina Center at Las Vegas

Russell P Jayne, MD is a member of the following medical societies: American Medical Association, American Society of Cataract and Refractive Surgery, and American Society of Retina Specialists

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Steve Charles, MD  Director of Charles Retina Institute; Clinical Professor, Department of Ophthalmology, University of Tennessee College of Medicine; Adjunct Professor of Ophthalmology, Columbia College of Physicians and Surgeons; Clinical Professor Ophthalmology, Chinese University of Hong Kong

Steve Charles, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Retina Specialists, Club Jules Gonin, Macula Society, and Retina Society

Disclosure: Alcon Laboratories Consulting fee Consulting; OptiMedica Ownership interest Other; Topcon Medical Lasers Consulting fee Consulting

Lance L Brown, OD, MD  Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri

Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD  Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

References

  1. Ryan SJ, Maumenee AE. Birdshot retinochoroidopathy. Am J Ophthalmol. Jan 1980;89(1):31-45. [Medline].

  2. Kuiper JJ, Mutis T, de Jager W, de Groot-Mijnes JD, Rothova A. Intraocular interleukin-17 and proinflammatory cytokines in HLA-A29-associated birdshot chorioretinopathy. Am J Ophthalmol. Aug 2011;152(2):177-182.e1. [Medline].

  3. LeHoang P, Ozdemir N, Benhamou A, et al. HLA-A29.2 subtype associated with birdshot retinochoroidopathy. Am J Ophthalmol. Jan 15 1992;113(1):33-5. [Medline].

  4. Levinson RD, Rajalingam R, Park MS, et al. Human leukocyte antigen A29 subtypes associated with birdshot retinochoroidopathy. Am J Ophthalmol. Oct 2004;138(4):631-4. [Medline].

  5. Nussenblatt RB, Mittal KK, Ryan S, et al. Birdshot retinochoroidopathy associated with HLA-A29 antigen and immune responsiveness to retinal S-antigen. Am J Ophthalmol. Aug 1982;94(2):147-58. [Medline].

  6. Fuerst DJ, Tessler HH, Fishman GA, et al. Birdshot retinochoroidopathy. Arch Ophthalmol. Feb 1984;102(2):214-9. [Medline].

  7. Gaudio PA, Kaye DB, Crawford JB. Histopathology of birdshot retinochoroidopathy. Br J Ophthalmol. Dec 2002;86(12):1439-41. [Medline].

  8. Vitale AT, Rodriguez A, Foster CS. Low-dose cyclosporine therapy in the treatment of birdshot retinochoroidopathy. Ophthalmology. May 1994;101(5):822-31. [Medline].

  9. Kiss S, Ahmed M, Letko E, et al. Long-term follow-up of patients with birdshot retinochoroidopathy treated with corticosteroid-sparing systemic immunomodulatory therapy. Ophthalmology. Jun 2005;112(6):1066-71. [Medline].

  10. Rothova A, Ossewaarde-van Norel A, Los LI, Berendschot TT. Efficacy of low-dose methotrexate treatment in birdshot chorioretinopathy. Retina. Jun 2011;31(6):1150-5. [Medline].

  11. LeHoang P, Cassoux N, George F, et al. Intravenous immunoglobulin (IVIg) for the treatment of birdshot retinochoroidopathy. Ocul Immunol Inflamm. Mar 2000;8(1):49-57. [Medline].

  12. Bloch-Michel E, Frau E. Birdshot retinochoroidopathy and HLA-A29+ and HLA-A29- idiopathic retinal vasculitis: comparative study of 56 cases. Can J Ophthalmol. Dec 1991;26(7):361-6. [Medline].

  13. Brucker AJ, Deglin EA, Bene C, et al. Subretinal choroidal neovascularization in birdshot retinochoroidopathy. Am J Ophthalmol. Jan 15 1985;99(1):40-4. [Medline].

  14. Caballero-Presencia A, Diaz-Guia E, Lopez-Lopez JM. Acute anterior ischemic optic neuropathy in birdshot retinochoroidopathy. Ophthalmologica. 1988;196(2):87-91. [Medline].

  15. de Smet MD, Yamamoto JH, Mochizuki M, et al. Cellular immune responses of patients with uveitis to retinal antigens and their fragments. Am J Ophthalmol. Aug 15 1990;110(2):135-42. [Medline].

  16. Godel V, Baruch E, Lazar M. Late development of chorioretinal lesions in birdshot retinochoroidopathy. Ann Ophthalmol. Feb 1989;21(2):49-52. [Medline].

  17. Hirose T, Katsumi O, Pruett RC, et al. Retinal function in birdshot retinochoroidopathy. Acta Ophthalmol (Copenh). Jun 1991;69(3):327-37. [Medline].

  18. Kaplan HJ, Aaberg TM. Birdshot retinochoroidopathy. Am J Ophthalmol. Dec 1980;90(6):773-82. [Medline].

  19. Kiss S, Anzaar F, Stephen Foster C. Birdshot retinochoroidopathy. Int Ophthalmol Clin. Spring 2006;46(2):39-55. [Medline].

  20. Levinson RD, Gonzales CR. Birdshot retinochoroidopathy: immunopathogenesis, evaluation, and treatment. Ophthalmol Clin North Am. Sep 2002;15(3):343-50, vii. [Medline].

  21. Noble KG, Greenberg J. Appearance of birdshot retinochoroidopathy in a patient with myelodysplasia syndrome. Am J Ophthalmol. Jan 1998;125(1):108-9. [Medline].

  22. Oh KT, Christmas NJ, Folk JC. Birdshot retinochoroiditis: long term follow-up of a chronically progressive disease. Am J Ophthalmol. May 2002;133(5):622-9. [Medline].

  23. Rosenberg PR, Noble KG, Walsh JB, et al. Birdshot retinochoroidopathy. Ophthalmology. Mar 1984;91(3):304-6. [Medline].

  24. Soubrane G, Bokobza R, Coscas G. Late developing lesions in birdshot retinochoroidopathy. Am J Ophthalmol. Feb 15 1990;109(2):204-10. [Medline].

  25. Soubrane G, Coscas G, Binaghi M, et al. Birdshot retinochoroidopathy and subretinal new vessels. Br J Ophthalmol. Jul 1983;67(7):461-7. [Medline].

 
Previous
Next
 
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.