eMedicine Specialties > Ophthalmology > Iris & Ciliary Body

Uveitis, Juvenile Idiopathic Arthritis: Follow-up

Author: Manolette R Roque, MD, MBA, President and CEO, Service Chief of Ocular Immunology and Uveitis, Refractive Surgery, EYE REPUBLIC Ophthalmology Clinic; General Manager, Ophthalmic Consultants Philippines; Section Chief of Ocular Immunology and Uveitis, Department of Ophthalmology, Asian Hospital and Medical Center; Section Chief of Ocular Immunology and Uveitis, Department of Ophthalmology, St. Luke's Medical Center Global City; Senior Eye Surgeon, The LASIK Surgery Clinic
Coauthor(s): Barbara L Roque, MD, Full Partner, Ophthalmic Consultants Philippines Co, Chief of Service, Pediatric Ophthalmology and Strabismus, Consulting Staff, Orbit and Eye Plastics, Eye Republic Ophthalmology Clinic; Elisabetta Miserocchi, MD, Fellow in Immunology and Uveitis Service, Department of Ophthalmology, Harvard Medical School; 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
Contributor Information and Disclosures

Updated: Oct 24, 2008

Follow-up

Further Outpatient Care

  • Patients with JIA-associated uveitis need to be seen by an ophthalmologist regularly, every 3 or 4 months (more often if with active uveitis). It is easy to miss flare-ups due to the white and quiet presentation of this type of uveitis. Moreover, children often do not complain of visual problems, precluding the possibility of early detection.
  • Children with JIA-associated uveitis on systemic medications require meticulous monitoring (CBC, LFTs, BUN, creatinine) for drug toxicity (bone marrow, liver, kidney), disease complications (eg, glaucoma, cataract, band keratopathy), disease exacerbations, or breakthrough inflammation.

Inpatient & Outpatient Medications

  • The mainstay of therapy of the ocular inflammation in these patients consists of topical corticosteroids and cycloplegics.

Deterrence/Prevention

  • The cause of JIA-associated uveitis is unknown. Therefore, the prevention of the disease has not been established.
  • Evaluate children who are at risk for JIA-related ocular complications on a frequent regular basis and monitor closely for the development of ocular inflammation.

Complications

  • Ocular complications may be sight threatening and include glaucoma, cataract, cyclitic membrane and hypotony, and band keratopathy.30,31,8,5
  • Although uveitis in JIA usually is anterior, vitritis, CME, and optic nerve edema may be present.
  • Complications from lack of treatment32
    • Cataracts (40-80%)
    • Band keratopathy (30-80%)
    • Macular edema or epiretinal membrane formation (30-50% in chronic cases)
    • Vitreous haze/debris (20-30%)
    • Glaucoma (10-30%)
    • Chronic hypotony and phthisis (5-20%)
    • Other posterior pole complications (eg, disc neovascularization, macular hole) are rare.33,34
  • Complications from treatment35,32
    • Cataracts, keratitis, and steroid-induced glaucoma from topical steroids
    • Lid abnormalities, orbital socket contraction, and globe perforation from regional corticosteroids
    • Gastrointestinal bleeding from nonsteroidal NSAIDs
    • Growth retardation, weight gain, acne, mood swings, and infections from systemic corticosteroids
    • Bone marrow suppression and pancytopenia from immunosuppressive therapy (methotrexate, cyclosporine-A, cyclophosphamide, chlorambucil)

Prognosis

  • Most vision-threatening morbidities in JIA are secondary to intraocular inflammation (eg, severity, chronicity). The development of JIA-associated uveitis heralds a poor prognosis.
  • Of affected eyes, 30-40% maintain long-term acuity greater than 20/40. Conversely, approximately 30-40% develop severe visual disability with acuity less than 20/200.
  • Poor prognosticators (higher risk for chronic iridocyclitis) include the following15,35 :
    • Female sex
    • Early onset pauciarticular arthritis–JIA
      • Young age at disease onset
      • Pauciarticular arthritis
    • ANA - Positive
    • Others
      • Posterior synechiae
      • Secondary glaucoma

Patient Education

  • Patients (and relatives) with JIA-associated uveitis require a significant amount of education regarding the signs and symptoms of disease exacerbation. Emphasize the need for lifestyle changes to prevent exacerbations, to preserve vision, and for appropriate pain control.
  • The need to maintain regular eye visits to check for ocular activity cannot be overemphasized, if one is to preserve the patients remaining useful vision.
  • For excellent patient education resources, visit eMedicine's Arthritis Center. Also, see eMedicine's patient education articles Juvenile Rheumatoid Arthritis and Understanding Rheumatoid Arthritis Medications.

Miscellaneous

Medicolegal Pitfalls

  • Missed diagnosis of JIA-associated uveitis may lead to litigation. The inability to ascribe the diagnosis to a particular patient may result in chronic uncontrolled subclinical activity leading to permanent loss of useful vision.
  • Prolonged aggressive treatment with NSAIDs may result in GI hemorrhage.
  • Prolonged treatment with oral corticosteroids may result in numerous adverse effects (eg, weight gain, stunted growth, mood swings).
  • Failure to arrange for close follow-up may complicate JIA-associated uveitis.
  • Inadequate systemic monitoring for use of immunosuppressive agents may lead to significant bone marrow suppression and pancytopenia.

Special Concerns

  • A young patient with systemic and ocular problems necessitating multiple and/or prolonged medications, and visits to the hospital is subjected to an enormously abnormal amount of stress. The physician and the family should be extremely sensitive to the emotional needs of the patient. Address the child's mental health accordingly if the need arises. Appropriate referral to a child psychiatrist may be necessary in these situations.
 
Acknowledgments

The authors and editors of eMedicine gratefully acknowledge the assistance of Ryan I Huffman, MD, with the literature review and referencing for this article.



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References
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References

  1. Nguyen QD, Foster CS. Saving the vision of children with juvenile rheumatoid arthritis-associated uveitis. JAMA. Oct 7 1998;280(13):1133-4. [Medline].

  2. Päivönsalo-Hietanen T, Tuominen J, Saari KM. Uveitis in children: population-based study in Finland. Acta Ophthalmol Scand. Feb 2000;78(1):84-8. [Medline].

  3. Boone MI, Moore TL, Cruz OA. Screening for uveitis in juvenile rheumatoid arthritis. J Pediatr Ophthalmol Strabismus. Jan-Feb 1998;35(1):41-3. [Medline].

  4. Kotaniemi K, Kaipiainen-Seppanen O, Savolainen A, et al. A population-based study on uveitis in juvenile rheumatoid arthritis. Clin Exp Rheumatol. Jan-Feb 1999;17(1):119-22. [Medline].

  5. Malleson P. Prevalence and outcome of uveitis in a regional cohort of patients with juvenile rheumatoid arthritis. J Rheumatol. Jun 1998;25(6):1242. [Medline].

  6. Kesen MR, Setlur V, Goldstein DA. Juvenile idiopathic arthritis-related uveitis. Int Ophthalmol Clin. Summer 2008;48(3):21-38. [Medline].

  7. Wright T, Cron RQ. Pediatric rheumatology for the adult rheumatologist II: uveitis in juvenile idiopathic arthritis. J Clin Rheumatol. Aug 2007;13(4):205-10. [Medline].

  8. Kanski JJ. Anterior uveitis in juvenile rheumatoid arthritis. Arch Ophthalmol. Oct 1977;95(10):1794-7. [Medline].

  9. Gallagher KT, Bernstein B. Juvenile rheumatoid arthritis. Curr Opin Rheumatol. Sep 1999;11(5):372-6. [Medline].

  10. Kotaniemi K. Late onset uveitis in juvenile-type chronic polyarthritis controlled with prednisolone, cyclosporin A and methotrexate. Clin Exp Rheumatol. Jul-Aug 1998;16(4):469-71. [Medline].

  11. Donn RP, Farhan AJ, Barrett JH, et al. Absence of association between interleukin 1 alpha and oligoarticular juvenile chronic arthritis in UK patients. Rheumatology (Oxford). Feb 1999;38(2):171-5. [Medline].

  12. Reininga JK, Los LI, Wulffraat NM, et al. The evaluation of uveitis in juvenile idiopathic arthritis (JIA) patients: are current ophthalmologic screening guidelines adequate?. Clin Exp Rheumatol. Mar-Apr 2008;26(2):367-72. [Medline].

  13. Yen JH, Tsai WC, Tsai JJ, et al. HLA-DMA and HLA-DMB genotyping in patients with rheumatic diseases. Kaohsiung J Med Sci. May 1999;15(5):263-7. [Medline].

  14. Cassidy JT. Medical management of children with juvenile rheumatoid arthritis. Drugs. Nov 1999;58(5):831-50. [Medline].

  15. Ceisler EJ, Foster CS. Juvenile rheumatoid arthritis and uveitis: minimizing the blinding complications. Int Ophthalmol Clin. Winter 1996;36(1):91-107. [Medline].

  16. Hemady RK, Baer JC, Foster CS. Immunosuppressive drugs in the management of progressive, corticosteroid-resistant uveitis associated with juvenile rheumatoid arthritis. Int Ophthalmol Clin. Winter 1992;32(1):241-52. [Medline].

  17. Yu EN, Meniconi ME, Tufail F, et al. Outcomes of treatment with immunomodulatory therapy in patients with corticosteroid-resistant juvenile idiopathic arthritis-associated chronic iridocyclitis. Ocul Immunol Inflamm. Sep-Oct 2005;13(5):353-60. [Medline].

  18. Kilmartin DJ, Forrester JV, Dick AD. Cyclosporin A therapy in refractory non-infectious childhood uveitis. Br J Ophthalmol. Jul 1998;82(7):737-42. [Medline].

  19. Okada AA. Immunomodulatory therapy for ocular inflammatory disease: a basic manual and review of the literature. Ocul Immunol Inflamm. Sep-Oct 2005;13(5):335-51. [Medline].

  20. Shetty AK, Zganjar BE, Ellis GS Jr, et al. Low-dose methotrexate in the treatment of severe juvenile rheumatoid arthritis and sarcoid iritis. J Pediatr Ophthalmol Strabismus. May-Jun 1999;36(3):125-8. [Medline].

  21. Singer NG, McCune WJ. Update on immunosuppressive therapy. Curr Opin Rheumatol. May 1998;10(3):169-73. [Medline].

  22. Weiss AH, Wallace CA, Sherry DD. Methotrexate for resistant chronic uveitis in children with juvenile rheumatoid arthritis. J Pediatr. Aug 1998;133(2):266-8. [Medline].

  23. Imrie FR, Dick AD. Biologics in the treatment of uveitis. Curr Opin Ophthalmol. Nov 2007;18(6):481-6. [Medline].

  24. Tynjala P, Kotaniemi K, Lindahl P, et al. Adalimumab in juvenile idiopathic arthritis-associated chronic anterior uveitis. Rheumatology (Oxford). Mar 2008;47(3):339-44. [Medline].

  25. Heiligenhaus A, Mingels A, Heinz C, et al. Methotrexate for uveitis associated with juvenile idiopathic arthritis: value and requirement for additional anti-inflammatory medication. Eur J Ophthalmol. Sep-Oct 2007;17(5):743-8. [Medline].

  26. Ilowite NT. Update on biologics in juvenile idiopathic arthritis. Curr Opin Rheumatol. Sep 2008;20(5):613-8. [Medline].

  27. BenEzra D, Cohen E. Cataract surgery in children with chronic uveitis. Ophthalmology. Jul 2000;107(7):1255-60. [Medline].

  28. Holland GN. Intraocular lens implantation in patients with juvenile rheumatoid arthritis-associated uveitis: an unresolved management issue. Am J Ophthalmol. Aug 1996;122(2):255-7. [Medline].

  29. Acevedo S, Quinones K, Rao V, et al. Cataract surgery in children with juvenile idiopathic arthritis associated uveitis. Int Ophthalmol Clin. Spring 2008;48(2):1-7. [Medline].

  30. Dana MR, Merayo-Lloves J, Schaumberg DA, et al. Visual outcomes prognosticators in juvenile rheumatoid arthritis-associated uveitis. Ophthalmology. Feb 1997;104(2):236-44. [Medline].

  31. Fox GM, Flynn HW Jr, Davis JL, et al. Causes of reduced visual acuity on long-term follow-up after cataract extraction in patients with uveitis and juvenile rheumatoid arthritis. Am J Ophthalmol. Dec 15 1992;114(6):708-14. [Medline].

  32. Ozdal PC, Vianna RN, Deschênes J. Visual outcome of juvenile rheumatoid arthritis-associated uveitis in adults. Ocul Immunol Inflamm. Feb 2005;13(1):33-8. [Medline].

  33. Gray T, Kanski J, Lightman S. Steroid responsive disc neovascularisation in uveitis associated with juvenile chronic arthritis. Br J Ophthalmol. Mar 1998;82(3):327-8. [Medline].

  34. Ducos de Lahitte G, Terrada C, Tran TH, et al. Maculopathy in uveitis of juvenile idiopathic arthritis: an optical coherence tomography study. Br J Ophthalmol. Jan 2008;92(1):64-9. [Medline].

  35. Wolf MD, Lichter PR, Ragsdale CG. Prognostic factors in the uveitis of juvenile rheumatoid arthritis. Ophthalmology. Oct 1987;94(10):1242-8. [Medline].

  36. Aggarwal A, Misra RN. Juvenile rheumatoid arthritis in India--rarity of antinuclear antibody and uveitis. Indian J Pediatr. May-Jun 1996;63(3):301-4. [Medline].

  37. Akinci A, Cakar N, Uncu N, et al. Keratoconjunctivitis sicca in juvenile rheumatoid arthritis. Cornea. Sep 2007;26(8):941-4. [Medline].

  38. Baldassano VF Jr. Ocular manifestations of rheumatic diseases. Curr Opin Ophthalmol. Dec 1998;9(6):85-8. [Medline].

  39. Dollfus H. Eye involvement in children's rheumatic diseases. Baillieres Clin Rheumatol. May 1998;12(2):309-28. [Medline].

  40. Singh S, Salaria M, Kumar L, et al. Clinico-immunological profile of juvenile rheumatoid arthritis at Chandigarh. Indian Pediatr. May 1999;36(5):449-54. [Medline].

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Further Reading

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Keywords

uveitis, juvenile idiopathic arthritis, juvenile idiopathic arthritis associated uveitis, JIA-associated uveitis, JIA uveitis, JRA-associated uveitis, JRA uveitis, juvenile rheumatoid arthritis, juvenile rheumatoid arthritis associated uveitis, vision loss, blindness, chronic iridocyclitis, chronic intraocular inflammation

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Contributor Information and Disclosures

Author

Manolette R Roque, MD, MBA, President and CEO, Service Chief of Ocular Immunology and Uveitis, Refractive Surgery, EYE REPUBLIC Ophthalmology Clinic; General Manager, Ophthalmic Consultants Philippines; Section Chief of Ocular Immunology and Uveitis, Department of Ophthalmology, Asian Hospital and Medical Center; Section Chief of Ocular Immunology and Uveitis, Department of Ophthalmology, St. Luke's Medical Center Global City; Senior Eye Surgeon, The LASIK Surgery Clinic
Manolette R Roque, MD, MBA is a member of the following medical societies: American Academy of Ophthalmic Executives, American Academy of Ophthalmology, American Society of Cataract and Refractive Surgery, American Society of Ophthalmic Administrators, American Uveitis Society, International Ocular Inflammation Society, Philippine Medical Association, Philippine Ocular Inflammation Society, and Philippine Society of Cataract and Refractive Surgery
Disclosure: Nothing to disclose.

Coauthor(s)

Barbara L Roque, MD, Full Partner, Ophthalmic Consultants Philippines Co, Chief of Service, Pediatric Ophthalmology and Strabismus, Consulting Staff, Orbit and Eye Plastics, Eye Republic Ophthalmology Clinic
Disclosure: Nothing to disclose.

Elisabetta Miserocchi, MD, Fellow in Immunology and Uveitis Service, Department of Ophthalmology, Harvard Medical School
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.

Medical Editor

Andrew A Dahl, MD, Director of Ophthalmology Teaching, Mid-Hudson Family Practice Institute, The Institute for Family Health; Assistant Professor of Surgery (Ophthalmology), New York College of Medicine
Andrew A Dahl, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Ophthalmology, American College of Surgeons, American Medical Association, American Society of Cataract and Refractive Surgery, and Wilderness Medical Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Simon K Law, MD, PharmD, Assistant Professor of Ophthalmology, Jules Stein Eye Institute; Chief of Section of Ophthalmology Surgical Services, Department of Veterans Affairs Healthcare Center, West Los Angeles
Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, and Association for Research in Vision and Ophthalmology
Disclosure: Nothing to disclose.

Managing Editor

R Christopher Walton, MD, Professor, Director of Uveitis and Ocular Inflammatory Disease Service, Department of Ophthalmology, Assistant Dean for Graduate Medical Education, University of Tennessee College of Medicine; Consulting Staff, Regional Medical Center, Memphis Veterans Affairs Medical Center, St Jude Children's Research Hospital
R Christopher Walton, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Healthcare Executives, American Uveitis Society, Association for Research in Vision and Ophthalmology, and Retina Society
Disclosure: Nothing to disclose.

CME Editor

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.

 
 
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