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X linked agammaglobulinaemia and rheumatoid arthritis
  1. G Verbruggen1,
  2. S De Backer2,
  3. D Deforce2,
  4. P Demetter3,
  5. C Cuvelier3,
  6. E Veys1,
  7. D Elewaut1
  1. 1Department of Rheumatology, University Hospital Gent, Belgium
  2. 2Laboratory of Pharmaceutical Biotechnology, University of Gent, Belgium
  3. 3Department of Pathology, University of Gent, Belgium
  1. Correspondence to:
    Assistant Professor D Elewaut
    Department of Rheumatology, University Hospital Gent, De Pintelaan 185, 9000 Gent, Belgium; dirk.elewautugent.be

Abstract

Background: Much interest has been shown recently in the pathogenic role of B cells in rheumatoid arthritis (RA) owing to the marked clinical responses to anti-CD20 treatment in RA.

Case report: A patient with X linked agammaglobulinaemia (XLA) presented with an erosive symmetric polyarthritis with histological features of RA, including formation of a destructive pannus. Furthermore, the patient developed subcutaneous nodules that were histologically indistinguishable from rheumatoid nodules. Surprisingly, lymphocytic infiltrates in both the synovium and nodule consisted almost exclusively of CD8+ T cells.

Discussion: Although some peculiar B cell subsets have been described in patients with XLA, no B cell subsets could be demonstrated in synovial tissue or the subcutaneous nodule. This case illustrates that classical RA can develop in the absence of mature B cells.

  • Btk, Bruton’s tyrosine kinase
  • GPI, glucose-6-phosphate isomerase
  • MC, metacarpophalangeal
  • PCR, polymerase chain reaction
  • RA, rheumatoid arthritis
  • XLA, X linked agammaglobulinaemia
  • B cells
  • X linked agammaglobulinaemia
  • rheumatoid arthritis

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Footnotes

  • * GV and SDB contributed equally to this manuscript.