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Article Dans Une Revue Autoimmunity Reviews Année : 2010

B-cell tolerance breakdown in Sjögren's syndrome: focus on BAFF.

Marie-Michèle Varin
  • Fonction : Auteur
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  • IdRef : 162021186
Damien Saulep
  • Fonction : Auteur
Fabienne Mackay
  • Fonction : Auteur
Jacques-Olivier Pers

Résumé

Sjögren's syndrome (SS) is an autoimmune epithelitis hallmarked by a destruction of epithelial cells, the subsequent lymphocytic infiltration of exocrine glands and their ensuing dryness. Given the prominent role currently assigned to B cells in SS, it is not surprising that the B cell activating factor belonging to the Tumor Necrosis Factor family (BAFF) is involved in its pathogenesis. When overexpressed, this cytokine leads to self-reactive B cells emergence at the transitional B-cell stage. BAFF overexpression that has been observed in SS, is associated with B-cell tolerance breakdown and autoantibody production. Furthermore, BAFF is responsible for the abnormal distribution of B cells subsets and B-cell hyperactivity described in the blood. In the salivary glands, a minority of B-cell clusters represent ectopic germinal center cells, while the majority manifest features consistent with transitional type 2 (T2) and marginal-zone (MZ)-like B cells. Interestingly, both types of B-cell cluster include autoreactive B cells and BAFF is associated with expansion of T2 B cells and MZ-like B cells in the salivary glands. Finally, BAFF plays a major role in B-cell repopulation after their depletion by rituximab in SS.

Domaines

Immunologie
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Dates et versions

hal-01103731 , version 1 (15-01-2015)

Identifiants

  • HAL Id : hal-01103731 , version 1
  • PUBMED : 20457281

Citer

Marie-Michèle Varin, Laëtitia Le Pottier, Pierre Youinou, Damien Saulep, Fabienne Mackay, et al.. B-cell tolerance breakdown in Sjögren's syndrome: focus on BAFF.. Autoimmunity Reviews, 2010, pp.604-8. ⟨hal-01103731⟩

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