Diagnosis and risk stratification in patients with anti-RNP autoimmunity.
Diagnosis and risk stratification in patients with anti-RNP autoimmunity.
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DOI：
10.1177/0961203315575586
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发表时间：
2015-09
期刊：
影响因子：
2.6
通讯作者：
Greidinger EL
中科院分区：
文献类型：
--
作者：
Carpintero MF;Martinez L;Fernandez I;Romero AC;Mejia C;Zang YJ;Hoffman RW;Greidinger EL
抗RNP自身抗体可见于混合性结缔组织病(MCTD)(通常预后良好)，也可见于系统性红斑狼疮(SLE)伴侵袭性主要器官疾病。目前还不确定如何评估抗RNP+患者患严重疾病的风险。根据IRB批准的方案，在一项队列研究中，从已知或疑似抗RNP自身免疫的患者和正常对照中收集临床数据和血液。对样本进行免疫激活参数筛选。根据临床诊断、疾病分类标准、疾病活动性和特定的终末器官临床表现对两组进行比较。97%的符合Alarcon-Segovia MCTD标准的患者也符合SLICC SLE标准，而47%的抗RNP+SLE患者也符合MCTD标准。在SLICC SLE患者中，MCTD标准与肾脏疾病发生率降低(优势比4.3，95%可信区间1.3-14.0)、雷诺现象发生率增加(OR 3.5，95%可信区间)相关。1.3-9.5)，并升高血清BCMA、TACI和TNFa水平。循环免疫标记物和I型干扰素激活标记物不能有效区分临床亚组。在抗RNP患者中，MCTD和SLE的问题不是非此即彼的：大多数MCTD患者也有狼疮。MCTD分类标准(但不是一组广泛的免疫标记物)区分出患肾脏疾病风险较低的SLE患者的子集。
Anti-RNP autoantibodies occur either in Mixed Connective Tissue Disease (MCTD) (with a frequently favorable prognosis), or in systemic lupus (SLE) cases with aggressive major organ disease. It is uncertain how to assess for the risk of severe disease in anti-RNP+ patients. Following IRB-approved protocols, clinical data and blood was collected from patients with known or suspected anti-RNP autoimmunity and normal controls in a cohort study. Samples were screened for parameters of immune activation. Groups were compared based on clinical diagnoses, disease classification criteria, disease activity, and specific end-organ clinical manifestations. 97% of patients satisfying Alarcon-Segovia MCTD criteria also met SLICC SLE criteria, while 47% of the anti-RNP+ SLE patients also met MCTD criteria. Among SLICC SLE patients, MCTD criteria were associated with reduced rates of renal disease (Odds Ratio 4.3, 95% confidence interval 1.3–14.0), increased rates of Raynaud’s Phenomenon (OR 3.5, 95% c.i. 1.3–9.5), and increased serum BCMA, TACI, and TNFa levels. Circulating immune markers and markers of Type I Interferon activation were not effective at distinguishing clinical subgroups. Among anti-RNP patients, the question of MCTD versus SLE is not either/or: most MCTD patients also have lupus. MCTD classification criteria (but not a broad set of immune markers) distinguish a subset of SLE patients at reduced risk for renal disease.