Validation of the preliminary criteria for the classification of catastrophic antiphospholipid syndrome
Validation of the preliminary criteria for the classification of catastrophic antiphospholipid syndrome
复制标题
DOI：
10.1136/ard.2004.025759
复制
发表时间：
2005-08-01
影响因子：
27.4
通讯作者：
Asherson, RA
中科院分区：
文献类型：
--
作者：
Cervera, R;Font, J;Asherson, RA
目的：描述灾难性抗磷脂综合征（APS）患者的特征，这些患者被纳入该疾病患者的国际登记研究（CAPS登记），并分析最近提出的灾难性APS初步分类标准的价值。对CAPS注册网站中纳入的前220例患者进行了审查，其初步分类标准为测试;结果：175例SLE、APS或两者兼有的SLE患者年龄7 ~ 74岁，平均38（14）岁，女性占多数（153/67）。主要临床表现包括肾脏受累154例（70%），肺部受累146例（66%），脑部受累133例（60%），心脏受累115例（52%），皮肤受累104例（47%）; 114例患者（52%）在灾难性APS事件后恢复（死亡率48%）。接受抗凝+类固醇+血浆置换或静脉注射免疫球蛋白联合治疗的患者生存率最高（63%，p = 0.09）。可以对176例患者的分类标准进行充分的数据分析。根据初步标准，89名患者（51%）可归类为“明确”患有APS，70名患者（40%）可归类为“可能”患有灾难性APS，因此灵敏度为90.3%，特异性为99.4%。阳性和阴性预测值分别为99.4%和91.1%，respectively.Conclusions：灾难性APS和CAPS登记的初步标准的分类流行病学研究是有用的工具。
Objective: To describe the characteristics of patients with catastrophic antiphospholipid syndrome (APS) included in the International Registry of patients with this condition (CAPS registry) and to analyse the value of the recently proposed preliminary criteria for the classification of catastrophic APS.Methods: A review of the first 220 patients included in the website based CAPS registry was undertaken and the preliminary criteria for their classification were tested; 175 unselected patients with systemic lupus erythematosus or APS, or both, acted as controls.Results: The mean age of the patients was 38 (14) years ( range 7 to 74), with a female preponderance (F/M, 153/67). The main clinical manifestations included renal involvement in 154 (70%), pulmonary in 146 (66%), cerebral in 133 (60%), cardiac in 115 (52%), and cutaneous in 104 (47%); 114 patients (52%) recovered after the catastrophic APS event ( mortality 48%). Patients who received the combination of anticoagulation plus steroids plus plasma exchange or intravenous immunoglobulins had the best survival rate (63%, p = 0.09). Sufficient data could be analysed for application of the classification criteria in 176 patients. According to the preliminary criteria, 89 patients (51%) could be classified as having "definite'' and 70 (40%) as having "probable'' catastrophic APS, thus given a sensitivity of 90.3% with a specificity of 99.4%. Positive and negative predictive values were 99.4% and 91.1%, respectively.Conclusions: The preliminary criteria for the classification of catastrophic APS and the CAPS registry are useful tools for epidemiological studies.