Current Approach to Treatment of Juvenile Idiopathic Arthritis: Case Report of Hiperimmunglobulin E Syndrome Developed Juvenile Idiopathic Arthritis


Introduction: Juvenile idiopathic arthritis (JIA) represents a heterogeneous group of childhood chronic arthritic conditions. The pathogenesis of JIA remains incompletely understood. This disease can lead to a significant morbidity including joint deformity, growth impairment and a persistence of active arthritis into adulthood. The past two decades have witnessed significant advances in treatment and improved outcomes for affected children. With the current use of biologics, more target-specific, better tolerated, safer and more effective treatments have become possible. However, continuing, comprehensive follow-up is needed to characterize the long-term effects of such treatments. Hyperimmunoglobulin E syndrome (hyper-IgE, or Job’s syndrome) is a rare immune deficiency characterized by high IgE levels, atopic chronic eczema, tendency towards re-current pyogenic infection, neutrophil chemotaxis disorder and varying T-cell function impairment. Case Report: The case of a 17-year-old male patient with hyper-IgE who develops the oligoarticular subtype of JIA over a period of four years is discussed. The course of JIA is unfavorable, causing severe deformity of numerous joints (left elbow, right 3rd metacarpophalangeal, left knee, right ankle) and a fungal infection scar on the left eye. Blood tests show an ESR of 89 mm/h, rheumatoid factor (RF) 8.3 IU/mL (0 - 20) and positive antinuclear antibody (ANA). To improve gait, corrective surgery is performed on the right ankle, followed by rehabilitation and physical therapy. Conclusion: Developments in the near future will be crucial for understanding JIA pathophysiology and improving treatment.

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Koca, T. and Arslan, A. (2015) Current Approach to Treatment of Juvenile Idiopathic Arthritis: Case Report of Hiperimmunglobulin E Syndrome Developed Juvenile Idiopathic Arthritis. Open Journal of Rheumatology and Autoimmune Diseases, 5, 62-67. doi: 10.4236/ojra.2015.53011.

Conflicts of Interest

The authors declare no conflicts of interest.


[1] Rigante, D., Bosco, A. and Esposito, S. (2014) The Etiology of Juvenile Idiopathic Arthritis. Clinical Reviews in Allergy & Immunology.
[2] Huang, J.L. (2012) New Advances in Juvenile Idiopathic Arthritis. Chang Gung Medical Journal, 35, 1-14.
[3] Kahn, P. (2009) Juvenile Idiopathic Arthritis—Current and Future Therapies. Bulletin of the NYU Hospital for Joint Diseases, 67, 291-302.
[4] Kahn, P. (2012) Juvenile Idiopathic Arthritis: An Update for the Clinician. Bulletin of the NYU Hospital for Joint Diseases, 70, 152-166.
[5] Eisenstein, E.M. and Berkun, Y. (2014) Diagnosis and Classification of Juvenile Idiopathic Arthritis. Journal of Autoimmunity, 48-49, 31-33.
[6] Grimbacher, B., Schäffer, A.A., Holland, S.M., Davis, J., Gallin, J.I., et al. (1999) Genetic Linkage of Hyper-IgE Syndrome to Chromosome 4. American Journal of Human Genetics, 65, 735-744.
[7] Buckley, R.H., Wray, B.B. and Belmaker, E.Z. (1972) Extreme Hyperimmunoglobulinemia E and Undue Susceptibility to Infection. Pediatrics, 49, 59-70.
[8] Grimbacher, B., Holland, S.M., Gallin, J.I., Greenberg, F., Hill, S.C., et al. (1999) Hyper-IgE Syndrome with Recurrent Infections—An Autosomal Dominant Multisystem Disorder. New England Journal of Medicine, 340, 692-702.
[9] Tugal-Tutkun, I., Quartier, P. and Bodaghi, B. (2014) Disease of the Year: Juvenile Idiopathic Arthritis-Associated Uveitis—Classification and Diagnostic Approach. Ocular Immunology & Inflammation, 22, 56-63.
[10] Oktayoglu, P. and Tekeoglu, I. (2011) Juvenilidiopatikartritte tedavi ve biyolojik ajanlar [Juvenileidiopathicarthri- tistreatmentandbiologicagents]. Sakarya medical journal, 3, 76-85.
[11] Horneff, G. (2013) Update on Biologicals for Treatment of Juvenile Idiopathic Arthritis. Expert Opinion on Biological Therapy, 13, 361-376.
[12] Herlin, T. (2008) Biological Therapy Treatment of Juvenile Idiopathic Arthritis. Ugeskrift for Læger, 170, 2105-2108.
[13] Otten, M.H., Anink, J., Spronk, S. and van Suijlekom-Smit, L.W. (2013) Efficacy of Biological Agents in Juvenile Idiopathic Arthritis: A Systematic Review Using Indirect Comparions. Annals of the Rheumatic Diseases, 72, 1806- 1812.

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