eMedicine Specialties > Ophthalmology > Iris & Ciliary Body

Uveitis, Juvenile Idiopathic Arthritis: Differential Diagnoses & Workup

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

Differential Diagnoses

Ankylosing Spondylitis
Ocular Manifestations of Syphilis
Behcet Disease
Psoriasis
Glaucoma, Uveitic
Reactive Arthritis
Herpes Simplex
Retinoblastoma
Herpes Zoster
Sarcoidosis
Inflammatory Bowel Disease
Tuberculosis
Juvenile Xanthogranuloma
Uveitis, Anterior, Childhood
Kawasaki Disease
Uveitis, Anterior, Nongranulomatous
Keratopathy, Band
Uveitis, Fuchs Heterochromic
Lyme Disease
Vogt-Koyanagi-Harada Disease

Other Problems to Be Considered

Trauma
Idiopathic uveitis
Arthritis
Sympathetic ophthalmia
Keratouveitis
Chronic iridocyclitis in girls
Heterochromic cyclitis
Pars planitis
Masquerade syndromes (leukemia)
Lupus erythematosus
Infantile multisystem inflammatory disease
Juvenile rheumatoid arthritis

Workup

Laboratory Studies

  • The different subtypes of JIA have individual serologic characteristics. Specific laboratory studies may be helpful to gauge the risk for developing uveitis and to rule out other possible diagnoses.8,3,7,12
  • Antinuclear antibody
    • The term ANA encompasses all the antibodies that can be demonstrated to react with nuclei in tissue sections by the classic indirect immunofluorescence test (IFA).
    • ANA-positivity is present in most children with oligoarticular onset JIA and uveitis; however, it is present in up to 80% of those without uveitis. For that reason, ANA negativity may be some help in predicting that a child will not develop uveitis, but their positivity does not assist in the prediction of the development of uveitis.
    • ANA-positivity in young girls with pauciarticular JIA presents the highest risk of developing uveitis.
  • Rheumatoid factor
    • RF classically is defined as an IgM antibody to the Fc portion of human immunoglobulin G (IgG) antibody; it often is present in serum in a complex with IgG antibody.
    • Many patients with JIA who develop uveitis are RF negative. A few adolescent girls who are RF positive have juvenile variant idiopathic arthritis, and they are not at significant risk for developing ocular disease.
  • Human leukocyte antigen B27
    • The only HLA antigen with a disease association strong enough to be useful in differential diagnosis is human leukocyte antigen B27 (HLA-B27).
    • A subgroup of older boys having pauciarticular arthritis with high risk for developing uveitis often is positive for HLA-B27 and negative for both RF and ANA.
    • A significant percentage of patients with JIA with spinal involvement are HLA-B27 positive.
    • HLA-B27 determination also is useful in ruling out seronegative spondyloarthropathies.

Imaging Studies

  • X-ray imaging of joints: Radiographic studies of affected joints typically reveal nondestructive but chronic articular changes.

Other Tests

  • Additional serologic tests: After undergoing a complete history, a detailed review of systems, and a comprehensive examination, additional serologic tests may be requested based on the findings (differentials). These tests may include the following:
    • Syphilis serologies (treponemal and nontreponemal)
    • Lyme titers
    • Angiotensin-converting enzyme (ACE)
    • Serum lysozyme
  • Serologic prognosticators presently under study13
    • Recent investigations are concentrated on determining which specific subsets of ANA and human leukocyte antigen D (human leukocyte antigen DR, human leukocyte antigen DP, and human leukocyte antigen DQ) have significant associations with early onset pauciarticular (EOPA) JIA uveitis.
    • Human leukocyte antigen DR5 is associated with uveitis in children with oligoarticular JIA. On the other hand, human leukocyte antigen DR1 and human leukocyte antigen DR4 are associated negatively with uveitis.

Histologic Findings

Pathologic results demonstrate that the synovium becomes hyperplastic, with subsynovial lymphocytic infiltration, vascular endothelial hyperplasia, and edema. A comparable histologic picture is observed in the eyes of patients with JIA-associated uveitis where lymphocytes, plasma cells, and scattered giant cells infiltrate the iris and ciliary body.

More on Uveitis, Juvenile Idiopathic Arthritis

Overview: Uveitis, Juvenile Idiopathic Arthritis
Differential Diagnoses & Workup: Uveitis, Juvenile Idiopathic Arthritis
Treatment & Medication: Uveitis, Juvenile Idiopathic Arthritis
Follow-up: Uveitis, Juvenile Idiopathic Arthritis
Multimedia: Uveitis, Juvenile Idiopathic Arthritis
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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