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Association of antiphospholipid antibodies with active digital ulceration in systemic sclerosis
  1. Mickaël Martin1,
  2. Camille Martinez2,
  3. Laurent Arnaud3,
  4. Jean-Christophe Weber4,
  5. Vincent Poindron5,
  6. Gilles Blaison2,
  7. Pierre Kieffer6,
  8. Bernard Bonnotte7,
  9. Sabine Berthier7,
  10. Denis Wahl8,9,
  11. Francois Maurier10,
  12. Jean-Loup Pennaforte11,
  13. Philip Bielefeld7,
  14. Nadine Magy-Bertrand12,
  15. Hervé Devilliers7 and
  16. Thierry Martin5
  1. 1Internal Medicine, Poitiers University Hospital, Poitiers, France
  2. 2Internal Medicine, Colmar Civilian Hospitals, Colmar, France
  3. 3Rheumatology, National Reference Center for Rare Autoimmune Diseases (RESO), Strasbourg University Hospital, Strasbourg, France
  4. 4Internal Medicine, National Reference Center for Rare Autoimmune Diseases (RESO), Strasbourg University Hospital, Strasbourg, France
  5. 5Clinical Immunology, National Reference Center for Rare Autoimmune Diseases (RESO), Strasbourg University Hospital, Strasbourg, France
  6. 6Internal Medicine, Mulhouse Regional Hospital Center, Mulhouse, France
  7. 7Internal Medicine and Clinical Immunology, Dijon University Hospital, Dijon, France
  8. 8Vascular Medicine, Regional Center for Rare Vascular and Systemic Autoimmune Diseases, Nancy University Hospital, Vandoeuvre-Les-Nancy, France
  9. 9Medicine Faculty, University of Lorraine, Vandoeuvre-Les-Nancy, France
  10. 10Internal Medicine, Metz-Thionville Regional Hospital Center, Metz, France
  11. 11Rheumatology, Reims University Hospital, Reims, France
  12. 12Internal Medicine, Besancon University Hospital, Besançon, France
  1. Correspondence to Dr Mickaël Martin; mickael-martin{at}

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Key messages

What is already known about this subject?

  • Antiphospholipid antibodies (aPL) prevalence seemed increased in patients with systemic sclerosis (SSc), but their clinical signification is unclear.

What does this study add?

  • Description of aPL positivity and antiphospholipid syndrome prevalence in a multicentre cross-sectional SSc cohort (confirmed and high aPL titre level).

  • Anti-beta-2 glycoprotein 1 (β2GP1) positivity is an independent factor associated with active digital ulceration.

How might this impact on clinical practice?

  • aPL and especially anti-β2GP1 should be screened in SSc to detect patients potentially at higher risk of developing digital ulcers.

Raynaud’s phenomenon (RP) in systemic sclerosis (SSc) can be severe with active digital ulceration (ADU) and gangrene. RP physiopathology includes early endothelial cell injury, vascular dysfunction and sometimes microvascular thrombosis.1 Antiphospholipid antibodies (aPL) activate endothelial cells and platelets by complexes of beta-2 glycoprotein 1 (β2GP1) and anti-β2GP12 and could therefore contribute to the initiation of and/or aggravate SSc-related RP. aPL prevalence seems increased in patients with SSc compared with controls,3 but aPL-associated clinical features are often contradictory. The aims of this study were to assess aPL and antiphospholipid syndrome (APS) prevalence in patients with SSc and their association with ADU.

Consecutive patients aged more than 18 years, fulfilling the American College of Rheumatology/EULAR classification criteria for SSc4 and followed in seven French expert centres for autoimmune diseases labelled by the French national network for autoimmune disease care, were enrolled prospectively during 8 months. Patients with other associated autoimmune disease were excluded. All patients provided written informed consent. SSc subtype was classified based on LeRoy and Medsger’s criteria,5 and skin involvement was assessed according to the modified Rodnan skin score (mRSS).6 Interstitial lung disease …

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