B cells in primary antiphospholipid syndrome: Review and remaining challenges

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Dieudonné, Yannick | Guffroy, Aurélien | Poindron, Vincent | Soulas-Sprauel, Pauline | Martin, Thierry | Korganow, Anne-Sophie | Gies, Vincent

Edité par CCSD ; Elsevier -

It is now widely accepted that antiphospholipid antibodies (aPL) have direct pathogenic effects and that B cells, notably through aPL production, play a key role in the development of antiphospholipid syndrome (APS). Recent findings strengthened the implication of B cells with the description of specific B cell phenotype abnormalities and inborn errors of immunity involving B cell signaling in APS patients. In addition, it has been shown in preclinical models that cross-reactivity between APS autoantigens and mimotopes expressed by human gut commensals can lead to B cell tolerance breakdown and are sufficient for APS development. However, B cell targeting therapies are surprisingly not as effective as expected in APS compared to other autoimmune diseases. Elucidation of the B cell tolerance breakdown mechanisms in APS patients may help to develop and guide the use of novel therapeutic agents that target B cells or specific immune pathway.

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