title: A child with x-linked agammaglobulinemia and enthesitis-related arthritis.
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.
authors.affiliation: Division of Rheumatology, Children's Hospital Los Angeles, Los Angeles, CA 90027, USA.
authors.forename: Sukesh
authors.initials: S
authors.lastname: Sukumaran
authors.forename: Katherine
authors.initials: K
authors.lastname: Marzan
authors.forename: Bracha
authors.initials: B
authors.lastname: Shaham
authors.forename: Joseph A
authors.initials: JA
authors.lastname: Church