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  • Title: Von Willebrand factor cleaving protease (ADAMTS-13) in 123 patients with connective tissue diseases (systemic lupus erythematosus and systemic sclerosis).
    Author: Mannucci PM, Vanoli M, Forza I, Canciani MT, Scorza R.
    Journal: Haematologica; 2003 Aug; 88(8):914-8. PubMed ID: 12935979.
    Abstract:
    BACKGROUND AND OBJECTIVES: Autoantibodies inactivating the von Willebrand factor (VWF) cleaving protease, ADAMTS-13, are among the most frequent causes of thrombotic thrombocytopenic purpura (TTP). We evaluated whether or not ADAMTS-13 deficiency and autoantibodies inactivating the protease prevalent in patients with the prototypic autoimmune diseases systemic lupus erythematosus (SLE) and systemic sclerosis (SSc). DESIGN AND METHODS: We measured, in parallel, the protease and VWF antigen (VWF:Ag) in 123 patients, 36 of whom had SLE and 87 of whom had SSc. In 14 patients with either disease who had low plasma protease levels (below 40%) we also looked for anti-ADAMTS-13 inactivating antibodies. RESULTS: ADAMTS-13 levels were significantly lower in SLE (p=0.0013) and in SSc (p=0.0002) than in normal controls. No anti-ADAMTS activity was measurable in patients with low ADAMTS-13 levels. VWF:Ag was high in both SLE and SSc (p=0.001). INTERPRETATION AND CONCLUSIONS: Systemic connective tissue diseases are other conditions besides TTP that are associated in some instances with low but detectable levels of ADAMTS-13. Autoantibodies inactivating protease activity are not the cause of the low plasma levels of ADAMTS-13.
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