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International Journal of Rheumatology
Volume 2011 (2011), Article ID 175973, 3 pages
http://dx.doi.org/10.1155/2011/175973
Case Report

A Child with X-Linked Agammaglobulinemia and Enthesitis-Related Arthritis

1Division of Rheumatology, Children's Hospital Los Angeles, Los Angeles, CA 90027, USA
2Division of Clinical Immunology and Allergy, Children's Hospital Los Angeles, Los Angeles, CA 90027, USA

Received 3 January 2011; Revised 11 April 2011; Accepted 11 April 2011

Academic Editor: Ruben Burgos-Vargas

Copyright © 2011 Sukesh Sukumaran et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

X-linked agammaglobulinemia (XLA) is a primary immune deficiency characterized by recurrent bacterial infections and profoundly depressed serum immunoglobulin levels and circulating mature B cells. We describe a 12-year-old boy with XLA and enthesitis-related arthritis (ERA). To date, there has been a paucity of reports of noninfectious inflammatory arthritis in children with XLA. This case illustrates that functional B cells and/or immunoglobulin are not required for ERA pathogenesis. In addition, this case suggests a possible link between immune deficiency, immune dysregulation, and rheumatic illness.