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

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...

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Bibliographic Details
Main Authors: Sukesh Sukumaran, Katherine Marzan, Bracha Shaham, Joseph A. Church
Format: Article
Language:English
Published: Hindawi Limited 2011-01-01
Series:International Journal of Rheumatology
Online Access:http://dx.doi.org/10.1155/2011/175973
Description
Summary: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.
ISSN:1687-9260
1687-9279