The Plasma Levels of ADAMTS-13, von Willebrand Factor, VWFpp, andFibrin-Related Markers in Patients With Systemic Sclerosis HavingThrombosis

This study aimed to examine the hemostatic abnormalities in patients with systemicsclerosis (SSc) and the relationship between these abnormalities and thrombotic events(THEs), focusing on the difference in diffuse cutaneous SSc (dcSSc) and limited cutaneousSSc (lcSSc). The plasma levels of ADAMTS-13 (a disintegrin-like and metalloproteinase withthrombospondin type 1 motifs 13), von Willebrand factor (VWF), VWF propeptide (VWFpp),d-dimer, and soluble fibrin (SF) were measured in 233 patients with SSc. Therelationship between their levels and organ involvement, including THEs and interstitiallung disease (ILD), was evaluated. The plasma levels of VWF and VWFpp were significantlyelevated and ADAMTS-13 activity was significantly decreased in patients with SSc comparedto healthy participants. The VWFpp in dcSSc was significantly higher than in lcSSc. Twelvepatients with SSc were complicated with acute THE, and 25 patients with SSc werecomplicated with past THE. The plasma levels of d-dimer and SF were significantlyelevated in patients with SSc having THE. The plasma levels of VWF and VWFpp weresignificantly elevated in patients with SSc having ILD. The plasma levels ofd-dimer were elevated in patients with SSc having other connective tissuediseases (CTDs). The plasma levels of ADAMTS-13 were significantly decreased and VWF,VWFpp, and SF were increased in patients with a d-dimer level of ≥1 μg/mL.Systemic sclerosis carries a high risk of THE, especially in patients with other CTDs.Plasma hemostasis-related markers are closely related to ILD and THE. These markers areimportant as markers of organ involvement as well as THE.