Anti-Ku syndrome with elevated CK and anti-Ku syndrome with anti-dsDNA are two distinct entities with different outcomes
Anti-Ku syndrome with elevated CK and anti-Ku syndrome with anti-dsDNA are two distinct entities with different outcomes
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DOI：
10.1136/annrheumdis-2018-214439
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发表时间：
2019-08-01
影响因子：
27.4
通讯作者：
Meyer, Alain
中科院分区：
文献类型：
--
作者：
Spielmann, Lionel;Nespola, Benoit;Meyer, Alain
目的 完善抗 Ku 相关疾病的谱系，目前结缔组织疾病的诊断标准对这种疾病的描述模棱两可。 方法 在 42 例连续携带抗 Ku 抗体的患者中，通过对随访期间记录的累积临床生物学特征进行层次聚类分析，在没有先验诊断的情况下描绘出具有相似表型和预后的亚组。然后使用敏感性特异性总和最大化方法确定基线时存在的最有效预测结果的特征。结果临床生物学特征分为三组。肾小球肾炎和间质性肺病（ILD）是该队列中的两种致命并发症，在三个组之间分布不均，这三个组在六个临床生物学特征上也存在差异。在基线时出现的特征中，肌酸激酶 (CK) 血清水平升高和抗 dsDNA 抗体通常是相互排斥的，并且最有效地预测了最后一次随访时的聚类归属。 CK 升高的抗 Ku 患者患 ILD 的风险高出 22 倍，而具有抗 dsDNA 抗体的抗 Ku 患者患肾小球肾炎的风险高出 13 倍。
Objective To refine the spectrum of anti-Ku associated disease, a condition that is equivocally described by current diagnostic criteria for connective tissue diseases.Methods Among 42 consecutive patients harbouring anti-Ku antibodies, subgroups with similar phenotypes and prognosis were delineated without an a priori diagnosis using hierarchical clustering analysis of the cumulative clinico-biological features recorded during the follow-up. Features present at baseline that most efficiently predicted the outcomes were then identified using a sensitivity specificity sum maximisation approach.Results Clinico-biological features were clustered into three groups. Glomerulonephritis and ILD, the two fatal complications in this cohort, were unequally distributed between the three clusters that additionally differed on six clinico-biological features. Among features present at baseline, elevated serum level of creatine kinase (CK) and anti-dsDNA antibodies were generally mutually exclusive and most efficiently predicted the cluster belonging at last follow-up. Anti-Ku patients with elevated CK had a 22-fold higher risk of ILD while anti-Ku patients with anti-dsDNA antibodies had a 13-fold higher risk of glomerulonephritisConclusion "Anti-Ku with elevated CK" syndrome and "anti-Ku with anti-dsDNA" syndrome represent two distinct entities that are important to recognise in order to best tailor patient care.