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

Abstract Full-Text HTML XML Download Download as PDF (Size:771KB) PP. 62-67
DOI: 10.4236/ojra.2015.53011    3,812 Downloads   4,537 Views  


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.

Cite this paper

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.

comments powered by Disqus

Copyright © 2020 by authors and Scientific Research Publishing Inc.

Creative Commons License

This work and the related PDF file are licensed under a Creative Commons Attribution 4.0 International License.