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

Sukesh Sukumaran | Zendy; Katherine Marzan | Zendy; Bracha Shaham | Zendy; Joseph A. Church | Zendy

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

Author(s) -

Sukesh Sukumaran,

Katherine Marzan,

Bracha Shaham,

Joseph A. Church

Publication year - 2011

Publication title -

international journal of rheumatology

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 0.8

H-Index - 33

eISSN - 1687-9279

pISSN - 1687-9260

DOI - 10.1155/2011/175973

Subject(s) - medicine , immunology , x linked agammaglobulinemia , arthritis , immune system , antibody , pathogenesis , enthesitis , receptor , bruton's tyrosine kinase , tyrosine kinase , psoriatic 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 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.

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