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Anti-heparin platelet factor 4 antibodies in systemic lupus erythematosus are associated with IgM antiphospholipid antibodies and the antiphospholipid syndrome
  1. Deborah Alpert (alperd01{at}
  1. Jersey Shore University Medical Center, United States
    1. Lisa A Mandl (mandll{at}
    1. Hospital for Special Surgery, United States
      1. Doruk Erkan (erkand{at}
      1. Hospital for Special Surgery, United States
        1. Wei Yin (wey2005{at}
        1. Weill Medical College of Cornell University, United States
          1. Ellinor I Peerschke (epeersch{at}
          1. Weill Medical College of Cornell University, United States
            1. Jane E Salmon (salmonj{at}
            1. Hospital for Special Surgery, United States


              Objective: To investigate the prevalence and clinical correlates of anti-heparin platelet factor 4 antibodies (anti-HPF4) in SLE patients with and without antiphospholipid antibodies (aPL).

              Methods: Sera and clinical data were obtained from the Hospital for Special Surgery Autoimmune Disease Registry for 78 aPL-positive and 91 aPL-negative SLE patients without heparin-induced thrombocytopenia (HIT). Controls were 90 blood donors of comparable age and sex. Sera were assayed for anti-HPF4, IgG/IgM antiphospholipid antibodies (APhL), and IgG/IgM-β2-glycoprotein 1 antibodies (anti-β2GP1). Serotonin release assays (SRAs) were performed for subjects with positive anti-HPF4.

              Results: Positive anti-HPF4 was seen in 9% of aPL-positive SLE patients, 4% of aPL-negative SLE patients and 1% of controls (p-value = 0.026, aPL-positive SLE versus controls). Two of 12 subjects with positive anti-HPF4 had reactive SRAs. In SLE patients, anti-HPF4 significantly correlated with IgM APhL, IgM anti-β2GP1, and inversely with complement C4. In immunoabsorption experiments, there was partial cross-reactivity of IgM anti-HPF4 with IgM APhL, but not with IgM anti-β2GP1. SLE patients with positive anti-HPF4 had increased odds of the antiphospholipid syndrome (APS; OR = 4.5, p-value = 0.019), and APS with arterial thrombosis (OR = 6.1, p-value = 0.007). In multivariate linear regression analyses, APS and IgM APhL were independently associated with anti-HPF4.

              Conclusions: Anti-HPF4 is detectable in SLE patients with and without aPL in the absence of HIT, and is most prevalent in aPL-positive SLE patients. In this SLE cohort, anti-HPF4 correlates with IgM APhL, IgM anti-β2GP1 and inversely with C4, and is associated with manifestations of APS.

              • antiphospholipid antibodies
              • complement
              • heparin-induced thrombocytopenia
              • platelet factor 4
              • systemic lupus erythematosus

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