Differential B- and T-cell activation in Wegener's granulomatosis

J Allergy Clin Immunol. 1999 May;103(5 Pt 1):885-94. doi: 10.1016/s0091-6749(99)70434-3.


Background: Autoimmune mechanisms are postulated to play a role in the development and progression of Wegener's granulomatosis (WG), a form of systemic, idiopathic necrotizing vasculitis.

Objective: We investigated the relation between lymphocyte activation and disease activity in patients with WG.

Methods: B- and T-lymphocyte activation was studied by cytometric assessment of the expression of the activation markers CD38 on B cells and CD25 and HLA-DR on CD4(+) and CD8(+) T-cell subsets, respectively. Activation at the cellular level was related to serum levels of antineutrophil cytoplasmic antibodies and soluble IL-2 receptor, which can be regarded as soluble activation markers of B and T cells.

Results: Percentages of CD38(bright) activated B cells were higher in patients with active WG than in patients experiencing disease remission (P <.05) or in healthy control subjects (P <.05). Percentages of activated CD4(+) and CD8(+) T cells were higher in patients with active WG (CD4 subset, P <.0001; CD8 subset, P <.005) than in healthy individuals. An increased percentage of activated T cells of both subsets was also seen in patients whose condition was in remission, as compared with healthy control subjects (CD4 subset, P <.0005; CD8 subset, P <. 001). Lymphocyte activation at the cellular level did not correlate with plasma levels of antineutrophil cytoplasmic antibodies or soluble IL-2 receptor.

Conclusion: In WG, B-cell activation is related to active disease, whereas T-cell activation persists during remission of the disease, which points to an intrinsic disordered immune system in this disease.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Antibodies, Antineutrophil Cytoplasmic / blood
  • B-Lymphocytes / immunology*
  • Female
  • Fluorescent Antibody Technique, Indirect
  • Granulomatosis with Polyangiitis / immunology*
  • Humans
  • Lymphocyte Activation
  • Lymphocyte Subsets
  • Male
  • Middle Aged
  • Receptors, Interleukin-2 / blood
  • Solubility
  • T-Lymphocytes / immunology*


  • Antibodies, Antineutrophil Cytoplasmic
  • Receptors, Interleukin-2