Mannucci Pier Mannuccio, Vanoli Massimo, Forza Ileana, Canciani Maria Teresa, Scorza Raffaella
Angelo Bianchi Bonomi Hemophilia and Thrombosis Center and Department of Internal Medicine, IRCCS maggiore Hospital and University of Milan, Milan, Italy.
Haematologica. 2003 Aug;88(8):914-8.
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).
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.
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).
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.
使血管性血友病因子（VWF）裂解蛋白酶ADAMTS - 13失活的自身抗体是血栓性血小板减少性紫癜（TTP）最常见的病因之一。我们评估了在典型自身免疫性疾病系统性红斑狼疮（SLE）和系统性硬化症（SSc）患者中普遍存在的ADAMTS - 13缺乏和使该蛋白酶失活的自身抗体情况。
我们同时检测了123例患者的蛋白酶和VWF抗原（VWF:Ag），其中36例为SLE患者，87例为SSc患者。在14例血浆蛋白酶水平较低（低于40%）的这两种疾病患者中，我们还检测了抗ADAMTS - 13失活抗体。
SLE患者（p = 0.0013）和SSc患者（p = 0.0002）的ADAMTS - 13水平显著低于正常对照组。ADAMTS - 13水平低的患者中未检测到抗ADAMTS活性。SLE和SSc患者的VWF:Ag均较高（p = 0.001）。
除TTP外，系统性结缔组织疾病在某些情况下也与ADAMTS - 13水平低但可检测到有关。使蛋白酶活性失活的自身抗体不是ADAMTS - 13血浆水平低的原因。
