Skip to main content
PMC home page
The British Journal of Ophthalmology logoLink to The British Journal of Ophthalmology
. 1998 Jul;82(7):737–742. doi: 10.1136/bjo.82.7.737

Cyclosporin A therapy in refractory non-infectious childhood uveitis

D Kilmartin 1, J Forrester 1, A Dick 1
PMCID: PMC1722678  PMID: 9924362

Abstract

AIMS—To assess the immunosuppressive efficacy, steroid sparing effect and adverse effects of cyclosporin A (CsA) therapy in refractory non-infectious childhood uveitis.
METHODS—A retrospective case series review of the medical records of children on CsA therapy attending a tertiary referral centre for refractory endogenous uveitis was performed. Low dose (⩽5.0 mg/kg/day) CsA therapy was started either as monotherapy or in combination with other agents. The CsA immunosuppressive efficacy was assessed by visual acuity and binocular indirect ophthalmoscopy (BIO) score outcomes and steroid sparing effect by growth charts and ability to withdraw or maintain a low steroid dose. Possible CsA adverse effects were monitored by routine biochemistry (including serum creatinine) and haematological tests, blood pressure recordings, and symptoms.
RESULTS—14 patients (25 eyes, 10 males, four females) were recruited with steroid failure as the most common CsA indication. Age (mean (SD)) at start of CsA therapy was 8.7 (4.1) years with a duration of CsA therapy of 20.9 (range 3.5-88.3) months at a maintenance CsA dose of 4.0 (1.0) mg/kg/day. From baseline, visual acuity improved or was maintained in 23 (92%) eyes and BIO score improved in 19 (76%) eyes. Height centiles were preserved and the maintenance prednisolone dose was 6.3 (3.3) mg/day, where required, in 10 (71%) patients. Nephrotoxicity was not observed, with transient systemic hypertension developing in one patient. Minor adverse effects were more common but were well tolerated.
CONCLUSIONS—Cyclosporin A therapy is effective and safe in the medium term, if closely monitored, in refractory non-infectious childhood uveitis. 

 Keywords: cyclosporin A; childhood uveitis

Full Text

The Full Text of this article is available as a PDF (110.2 KB).

Figure 1  .

Figure 1  

Open in a new tab

Best corrected visual acuity (BCVA) outcome on cyclosporin A (CsA) therapy. Affected eyes n = 25. 

Figure 2  .

Figure 2  

Open in a new tab

Binocular indirect ophthalmoscopy (BIO) outcome on cyclosporin A (CsA) therapy. Affected eyes n=25.

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Allen D. B., Goldberg B. D. Stimulation of collagen synthesis and linear growth by growth hormone in glucocorticoid-treated children. Pediatrics. 1992 Mar;89(3):416–421. [PubMed] [Google Scholar]
  2. Assan R., Timsit J., Feutren G., Bougnères P., Czernichow P., Hannedouche T., Boitard C., Noel L. H., Mihatsch M. J., Bach J. F. The kidney in cyclosporin A-treated diabetic patients: a long-term clinicopathological study. Clin Nephrol. 1994 Jan;41(1):41–49. [PubMed] [Google Scholar]
  3. Au J., Gregory J. W., Colquhoun I. W., Scott C. D., Hilton C. J., Hunter S., Dark J. H. Paediatric cardiac transplantation with steroid-sparing maintenance immunosuppression. Arch Dis Child. 1992 Oct;67(10):1262–1266. doi: 10.1136/adc.67.10.1262. [DOI] [PMC free article] [PubMed] [Google Scholar]
  4. Bloch-Michel E., Nussenblatt R. B. International Uveitis Study Group recommendations for the evaluation of intraocular inflammatory disease. Am J Ophthalmol. 1987 Feb 15;103(2):234–235. doi: 10.1016/s0002-9394(14)74235-7. [DOI] [PubMed] [Google Scholar]
  5. Brodehl J., Bökenkamp A., Hoyer P. F., Offner G. Long-term results of cyclosporin A therapy in children. J Am Soc Nephrol. 1992 Jun;2(12 Suppl):S246–S254. doi: 10.1681/ASN.V212s246. [DOI] [PubMed] [Google Scholar]
  6. Burren C. P., Jones C. L., Francis D. M., Powell H. R., Walker R. G. Renal transplantation in young children. Aust N Z J Med. 1995 Apr;25(2):122–126. doi: 10.1111/j.1445-5994.1995.tb02823.x. [DOI] [PubMed] [Google Scholar]
  7. Bökenkamp A., Offner G., Hoyer P. F., Vester U., Wonigeit K., Brodehl J. Improved absorption of cyclosporin A from a new microemulsion formulation: implications for dosage and monitoring. Pediatr Nephrol. 1995 Apr;9(2):196–198. doi: 10.1007/BF00860745. [DOI] [PubMed] [Google Scholar]
  8. Chamberlin P., Meyer W. J., 3rd Management of pituitary-adrenal suppression secondary to corticosteroid therapy. Pediatrics. 1981 Feb;67(2):245–251. [PubMed] [Google Scholar]
  9. Chao S. M., Jones C. L., Powell H. R., Johnstone L., Francis D. M., Becker G. J., Walker R. G. Triple immunosuppression with subsequent prednisolone withdrawal: 6 years' experience in paediatric renal allograft recipients. Pediatr Nephrol. 1994 Feb;8(1):62–69. doi: 10.1007/BF00868264. [DOI] [PubMed] [Google Scholar]
  10. DAVIS M. D. Endogenous uveitis in children; associated band-shaped keratopathy and rheumatoid arthritis. AMA Arch Ophthalmol. 1953 Oct;50(4):443–454. [PubMed] [Google Scholar]
  11. Dick A. D., Azim M., Forrester J. V. Immunosuppressive therapy for chronic uveitis: optimising therapy with steroids and cyclosporin A. Br J Ophthalmol. 1997 Dec;81(12):1107–1112. doi: 10.1136/bjo.81.12.1107. [DOI] [PMC free article] [PubMed] [Google Scholar]
  12. Dijkmans B. A. Safety aspects of cyclosporin in rheumatoid arthritis. Drugs. 1995;50 (Suppl 1):41–47. doi: 10.2165/00003495-199500501-00007. [DOI] [PubMed] [Google Scholar]
  13. Feutren G., Mihatsch M. J. Risk factors for cyclosporine-induced nephropathy in patients with autoimmune diseases. International Kidney Biopsy Registry of Cyclosporine in Autoimmune Diseases. N Engl J Med. 1992 Jun 18;326(25):1654–1660. doi: 10.1056/NEJM199206183262502. [DOI] [PubMed] [Google Scholar]
  14. Giles C. L. Pediatric intermediate uveitis. J Pediatr Ophthalmol Strabismus. 1989 May-Jun;26(3):136–139. doi: 10.3928/0191-3913-19890501-10. [DOI] [PubMed] [Google Scholar]
  15. Giles C. L. Uveitis in childhood--Part I. Anterior. Ann Ophthalmol. 1989 Jan;21(1):13-9, 28. [PubMed] [Google Scholar]
  16. Giles C. L. Uveitis in childhood--Part II. Intermediate. Ann Ophthalmol. 1989 Jan;21(1):20–22. [PubMed] [Google Scholar]
  17. Giles C. L. Uveitis in childhood--Part III. Posterior. Ann Ophthalmol. 1989 Jan;21(1):23–28. [PubMed] [Google Scholar]
  18. Hemady R., Tauber J., Foster C. S. Immunosuppressive drugs in immune and inflammatory ocular disease. Surv Ophthalmol. 1991 Mar-Apr;35(5):369–385. doi: 10.1016/0039-6257(91)90186-j. [DOI] [PubMed] [Google Scholar]
  19. Hokken-Koelega A. C., Stijnen T., de Muinck Keizer-Schrama S. M., Blum W. F., Drop S. L. Levels of growth hormone, insulin-like growth factor-I (IGF-I) and -II, IGF-binding protein-1 and -3, and cortisol in prednisone-treated children with growth retardation after renal transplantation. J Clin Endocrinol Metab. 1993 Oct;77(4):932–938. doi: 10.1210/jcem.77.4.7691864. [DOI] [PubMed] [Google Scholar]
  20. Hokken-Koelega A. C., Van Zaal M. A., de Ridder M. A., Wolff E. D., De Jong M. C., Donckerwolcke R. A., De Muinck Keizer-Schrama S. M., Drop S. L. Growth after renal transplantation in prepubertal children: impact of various treatment modalities. Pediatr Res. 1994 Mar;35(3):367–371. doi: 10.1203/00006450-199403000-00020. [DOI] [PubMed] [Google Scholar]
  21. Hoyer P. F., Offner G., Wonigeit K., Brodehl J., Pichlmayr R. Dosage of Cyclosporin A in children with renal transplants. Clin Nephrol. 1984 Aug;22(2):68–71. [PubMed] [Google Scholar]
  22. Hulton S. A., Neuhaus T. J., Dillon M. J., Barratt T. M. Long-term cyclosporin A treatment of minimal-change nephrotic syndrome of childhood. Pediatr Nephrol. 1994 Aug;8(4):401–403. doi: 10.1007/BF00856511. [DOI] [PubMed] [Google Scholar]
  23. Ishioka M., Ohno S., Nakamura S., Isobe K., Watanabe N., Ishigatsubo Y., Tanaka S. FK506 treatment of noninfectious uveitis. Am J Ophthalmol. 1994 Dec 15;118(6):723–729. doi: 10.1016/s0002-9394(14)72551-6. [DOI] [PubMed] [Google Scholar]
  24. KIMURA S. J., HOGAN M. J., THYGESON P. Uveitis in children. AMA Arch Ophthalmol. 1954 Jan;51(1):80–88. doi: 10.1001/archopht.1954.00920040082011. [DOI] [PubMed] [Google Scholar]
  25. KIMURA S. J., HOGAN M. J. UVEITIS IN CHILDREN: ANALYSIS OF 274 CASES. Trans Am Ophthalmol Soc. 1964;62:173–192. [PMC free article] [PubMed] [Google Scholar]
  26. Kanski J. J. Juvenile arthritis and uveitis. Surv Ophthalmol. 1990 Jan-Feb;34(4):253–267. doi: 10.1016/0039-6257(90)90026-r. [DOI] [PubMed] [Google Scholar]
  27. Kanski J. J., Shun-Shin G. A. Systemic uveitis syndromes in childhood: an analysis of 340 cases. Ophthalmology. 1984 Oct;91(10):1247–1252. doi: 10.1016/s0161-6420(84)34177-x. [DOI] [PubMed] [Google Scholar]
  28. Krmar R. T., Wühl E., Ding R., Aulmann M., Schärer K. Pharmacokinetics of a new microemulsion formulation of cyclosporin A (Neoral) in young patients after renal transplantation. Transpl Int. 1996;9(5):476–480. doi: 10.1007/BF00336825. [DOI] [PubMed] [Google Scholar]
  29. Lane L., Tamesis R., Rodriguez A., Christen W., Akova Y. A., Messmer E., Pedroza-Seres M., Barney N., Foster C. S. Systemic immunosuppressive therapy and the occurrence of malignancy in patients with ocular inflammatory disease. Ophthalmology. 1995 Oct;102(10):1530–1535. doi: 10.1016/s0161-6420(95)30835-4. [DOI] [PubMed] [Google Scholar]
  30. Neuhaus T. J., Burger H. R., Klingler M., Fanconi A., Leumann E. P. Long-term low-dose cyclosporin A in steroid dependent nephrotic syndrome of childhood. Eur J Pediatr. 1992 Oct;151(10):775–778. doi: 10.1007/BF01959089. [DOI] [PubMed] [Google Scholar]
  31. Nussenblatt R. B., Palestine A. G., Chan C. C. Cyclosporin A therapy in the treatment of intraocular inflammatory disease resistant to systemic corticosteroids and cytotoxic agents. Am J Ophthalmol. 1983 Sep;96(3):275–282. doi: 10.1016/s0002-9394(14)77814-6. [DOI] [PubMed] [Google Scholar]
  32. Nussenblatt R. B., Palestine A. G., Chan C. C., Roberge F. Standardization of vitreal inflammatory activity in intermediate and posterior uveitis. Ophthalmology. 1985 Apr;92(4):467–471. doi: 10.1016/s0161-6420(85)34001-0. [DOI] [PubMed] [Google Scholar]
  33. Nussenblatt R. B., Palestine A. G., Chan C. C., Stevens G., Jr, Mellow S. D., Green S. B. Randomized, double-masked study of cyclosporine compared to prednisolone in the treatment of endogenous uveitis. Am J Ophthalmol. 1991 Aug 15;112(2):138–146. doi: 10.1016/s0002-9394(14)76692-9. [DOI] [PubMed] [Google Scholar]
  34. Perkins E. S. Pattern of uveitis in children. Br J Ophthalmol. 1966 Apr;50(4):169–185. doi: 10.1136/bjo.50.4.169. [DOI] [PMC free article] [PubMed] [Google Scholar]
  35. Pivetti-Pezzi P., Accorinti M., Abdulaziz M. A., La Cava M., Torella M., Riso D. Behçets disease in children. Jpn J Ophthalmol. 1995;39(3):309–314. [PubMed] [Google Scholar]
  36. Schwartz G. J., Haycock G. B., Spitzer A. Plasma creatinine and urea concentration in children: normal values for age and sex. J Pediatr. 1976 May;88(5):828–830. doi: 10.1016/s0022-3476(76)81125-0. [DOI] [PubMed] [Google Scholar]
  37. Somerville M. F., Scott D. G. Neoral--new cyclosporin for old? Br J Rheumatol. 1997 Oct;36(10):1113–1115. doi: 10.1093/rheumatology/36.10.1113. [DOI] [PubMed] [Google Scholar]
  38. Tamesis R. R., Rodriguez A., Christen W. G., Akova Y. A., Messmer E., Foster C. S. Systemic drug toxicity trends in immunosuppressive therapy of immune and inflammatory ocular disease. Ophthalmology. 1996 May;103(5):768–775. doi: 10.1016/s0161-6420(96)30618-0. [DOI] [PubMed] [Google Scholar]
  39. Tanner J. M., Whitehouse R. H., Takaishi M. Standards from birth to maturity for height, weight, height velocity, and weight velocity: British children, 1965. II. Arch Dis Child. 1966 Dec;41(220):613–635. doi: 10.1136/adc.41.220.613. [DOI] [PMC free article] [PubMed] [Google Scholar]
  40. Tarantino A., Montagnino G., Ponticelli C. Corticosteroids in kidney transplant recipients. Safety issues and timing of discontinuation. Drug Saf. 1995 Sep;13(3):145–156. doi: 10.2165/00002018-199513030-00002. [DOI] [PubMed] [Google Scholar]
  41. Towler H. M., Cliffe A. M., Whiting P. H., Forrester J. V. Low dose cyclosporin A therapy in chronic posterior uveitis. Eye (Lond) 1989;3(Pt 3):282–287. doi: 10.1038/eye.1989.39. [DOI] [PubMed] [Google Scholar]
  42. Towler H. M., Whiting P. H., Forrester J. V. Combination low dose cyclosporin A and steroid therapy in chronic intraocular inflammation. Eye (Lond) 1990;4(Pt 3):514–520. doi: 10.1038/eye.1990.68. [DOI] [PubMed] [Google Scholar]
  43. Tugal-Tutkun I., Havrlikova K., Power W. J., Foster C. S. Changing patterns in uveitis of childhood. Ophthalmology. 1996 Mar;103(3):375–383. doi: 10.1016/s0161-6420(96)30682-9. [DOI] [PubMed] [Google Scholar]
  44. Tugwell P. International consensus recommendations on cyclosporin use in rheumatoid arthritis. Drugs. 1995;50 (Suppl 1):48–56. doi: 10.2165/00003495-199500501-00008. [DOI] [PubMed] [Google Scholar]
  45. Vitale A. T., Rodriguez A., Foster C. S. Low-dose cyclosporin A therapy in treating chronic, noninfectious uveitis. Ophthalmology. 1996 Mar;103(3):365–374. doi: 10.1016/s0161-6420(96)30683-0. [DOI] [PubMed] [Google Scholar]
  46. Weijtens O., van der Sluijs F. A., Schoemaker R. C., Lentjes E. G., Cohen A. F., Romijn F. P., van Meurs J. C. Peribulbar corticosteroid injection: vitreal and serum concentrations after dexamethasone disodium phosphate injection. Am J Ophthalmol. 1997 Mar;123(3):358–363. doi: 10.1016/s0002-9394(14)70131-x. [DOI] [PubMed] [Google Scholar]
  47. Whitcup S. M., Salvo E. C., Jr, Nussenblatt R. B. Combined cyclosporine and corticosteroid therapy for sight-threatening uveitis in Behçet's disease. Am J Ophthalmol. 1994 Jul 15;118(1):39–45. doi: 10.1016/s0002-9394(14)72840-5. [DOI] [PubMed] [Google Scholar]

Articles from The British Journal of Ophthalmology are provided here courtesy of BMJ Publishing Group

RESOURCES