灾难性抗磷脂综合症的诊断分类标准

灾难性抗磷脂综合症的诊断分类标准

    1.≥3个器官系统或组织受累证据[A]

    2.临床症状一周内出现

    3.≥1个器官组织的小血管栓塞,并经病理证实[B]

    4.实验室:抗磷脂抗体(LA或aCL或抗β2 ?GP1)阳性[C]

    确诊:符合以上所有4项；河南大学淮河医院中西医结合风湿科娄广亮

    可能诊断:(1)虽有以上4项，但累及的器官系统或组织仅2个；

    (2)虽有以上4项，但因患者死亡早，未能隔6周再次检测抗磷脂抗体，以前也未检测过；

    (3)仅有第1、2、4项；

    (4)仅有第1、3和4项，不管抗凝情况如何，1周~1个月内出现第三项。

    注: A=通常情况下，血管闭塞的临床证据由影像学来证实。肾受累的定义是血肌酐增高50%、严重的高血压(>180/100mmHg)和/或蛋白尿(0.5g/d)。

    B=对于组织学的证实，虽然可能偶尔同时有血管炎，但必需有血栓形成的证据。

    C=如果患者以前未曾诊断抗磷脂综合征，则应至少隔6周检测抗磷脂抗体2次以上(不一定是疾病发作时检测）。

   附原文: Preliminary criteria for the classification of catastrophic antiphospholipid syndrome

    (1) Evidence of involvement of three or more organs, systems, and/or tissues*

    (2) Development of manifestations simultaneously or in less than one week

    (3) Confirmation by histopathology of small vessel occlusion in at least one organ or tissue#

    (4) Laboratory confirmation of the presence of antiphospholipid antibodies (lupus anticoagulant and/or anticardiolipin antibodies)**

    Definite catastrophic APS: all four criteria Probable catastrophic APS?any of the following: (a) All four criteria, except for only two organs, systems, and/or tissues involved. (b) All four criteria, except for the absence of laboratory confirmation (within at least 6 weeks) owing to the early death of a patient never tested for aPL before the catastrophic APS.(c) Criteria (1), (2), and (4) (d) Criteria (1), (3), and (4) and the development of a third event between one week and one month after presentation, despite anticoagulation.

 *Usually clinical evidence of vessel occlusions, confirmed by imaging techniques when appropriate. Renal involvement is defined by a 50% rise in serum creatinine, severe systemic hypertension (.180/100 mm Hg). and/or proteinuria (500 mg/24 hours).

#For histopathological confirmation, significant evidence of thrombosis must be present, although vasculitis may coexist occasionally.

    **If the patient had not previously been diagnosed as having an APS, the laboratory confirmation requires that the presence of antiphospholipid antibodies must be detected on two or more occasions at least six weeks apart (not necessarily at the time of the event), according to the proposed preliminary criteria for the classification of definite APS.

    引自：Asherson RA, Cervera R, de Groot PG, et al. Catastrophic antiphospholipid syndrome: international consensus statement on classification criteria and treatment guidelines. Lupus 2003;12:530?4.