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  • Increased B-cell activating factor (BAFF)/B-lymphocyte stimulator (BLyS) in primary antiphospholipid syndrome is associated with higher adjusted global antiphospholipid syndrome scores

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Connective tissue diseases
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Increased B-cell activating factor (BAFF)/B-lymphocyte stimulator (BLyS) in primary antiphospholipid syndrome is associated with higher adjusted global antiphospholipid syndrome scores
  1. Lucas L van den Hoogen1,2,
  2. Giovanni Palla1,2,
  3. Cornelis P J Bekker1,2,
  4. Ruth D E Fritsch-Stork3,
  5. Timothy R D J Radstake1,2 and
  6. Joel A G van Roon1,2
  1. 1 Laboratory of Translational Immunology, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands
  2. 2 Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands
  3. 3 1st Medical Department and Ludwig Boltzmann Institute of Osteology at the Hanusch Hospital of WGKK, AUVA Trauma Centre Meidling, Hanusch Hospital, Sigmund Freud University, Vienna, Austria
  1. Correspondence to Dr Lucas L van den Hoogen; l.l.vandenhoogen{at}umcutrecht.nl

https://doi.org/10.1136/rmdopen-2018-000693

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    Key messages

    What is already known about this subject?

    • BAFF/BLyS is a key cytokine in systemic lupus erythematosus (SLE) and targeting BAFF by belimumab is used to treat patients with SLE.

    • Antiphospholipid syndrome (APS) is a major cause of thrombosis and pregnancy morbidity in patients with rheumatic diseases and immunomodulatory treatments are novel drug candidates to treat APS.

    What does this study add?

    • BAFF levels are elevated in patient with primary APS and correlate with higher adjusted global antiphospholipid syndrome scores.

    • In SLE, but not in APS, the expression of BAFF receptors is altered on peripheral blood B cells.

    How might this impact on clinical practice?

    • A subset of patients with APS may benefit from BAFF- targeting therapies such as belimumab.

    Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterised by antiphospholipid antibodies (aPL), thrombosis and obstetric complications. Other manifestations seen in APS include thrombocytopenia, heart valve disease and leg ulcers. APS was first described in patients suffering from systemic lupus erythematosus (SLE). However, APS may also affect patients not suffering from SLE or another underlying disease, termed primary APS (PAPS). Current treatment strategies for APS relying on anticoagulants are suboptimal, particularly for non-thrombotic manifestations and immunomodulatory drugs have been suggested as potential novel drug candidates in APS.1 2

    B-cell activating factor (BAFF), also known as B-lymphocyte stimulator (BLyS), is an important growth factor for B cells. The importance of BAFF in SLE is supported by animal models as mice transgenic for BAFF spontaneously develop SLE-like autoimmunity including the production of antibodies against double-stranded DNA (anti-dsDNA).3 In …

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