Department of Rheumatology.
Department of Clinical Chemistry and Laboratory Medicine.
Rheumatology (Oxford). 2017 Jul 1;56(7):1081-1088. doi: 10.1093/rheumatology/kew402.
To evaluate the prevalence of anti-extractable nuclear antigen (anti-ENA) antibodies in Dutch SSc patients and the predictive power of the combination of specific anti-ENA antibodies and nailfold videocapillaroscopy (NVC) patterns to improve identification of patients with high risk for cardiopulmonary involvement.
A total of 287 patients (79%) from the Leiden SSc-Cohort had data available on NVC-pattern (no SSc-specific, early, active, late) and anti-ENA antibodies. Associations between anti-ENA/NVC combinations with cardiopulmonary parameters were explored using logistic regression.
Prevalence of ACA was 37%, anti-Scl-70 24%, anti-RNP 9%, anti-RNAPIII 5%, anti-fibrillarin 4%, anti-Pm/Scl 3%, anti-Th/To 0.3% and anti-Ku 1.4%. NVC showed a SSc-specific pattern in 88%: 10% early, 42% active and 36% late. The prevalence of different NVC patterns was equally distributed among specific anti-ENA antibodies, except for the absence of early pattern in anti-RNP positive patients. Fifty-one percent had interstitial lung disease (ILD), 59% had decreased diffusion capacity for carbon monoxide and 16% systolic pulmonary artery pressure >35 mmHg (sPAP↑). Regardless of ENA-subtype, NVC-pattern showed a stable association with presence of ILD or sPAP↑. For ILD, the odds ratios (ORs) were 1.3-1.4 ( P < 0.05 for analyses with anti-RNAPIII, anti-RNP). For diffusion capacity for carbon monoxide, the OR was 1.5 ( P < 0.05 for analyses with ACA, anti-Scl-70, anti-RNAPIII, anti-RNP). For sPAP↑, the ORs were 2.2-2.4 ( P < 0.05 for analyses with anti-RNAPIII, anti-RNP).
In Dutch SSc patients, all SSc-specific auto-antibodies were found, with ACA and anti-Scl-70 being the most prevalent. Strikingly, the association between NVC-pattern and heart/lung involvement was independent of specific anti-ENA antibodies, which might indicate microangiopathy is an important cause of organ involvement.
评估荷兰系统性硬化症（SSc）患者中抗可提取核抗原（抗-ENA）抗体的流行情况，以及特定抗-ENA 抗体与甲襞毛细血管镜（NVC）模式相结合对提高心血管受累高风险患者识别率的预测能力。
莱顿 SSc 队列中的 287 名患者（79%）有 NVC 模式（无 SSc 特异性、早期、活跃、晚期）和抗 ENA 抗体的数据。使用逻辑回归探讨抗 ENA/NVC 组合与心肺参数之间的关系。
ACA 的患病率为 37%，抗 Scl-70 为 24%，抗 RNP 为 9%，抗 RNApIII 为 5%，抗纤维蛋白原为 4%，抗 Pm/Scl 为 3%，抗 Th/To 为 0.3%，抗 Ku 为 1.4%。NVC 表现为 88%的 SSc 特异性模式：10%早期，42%活跃，36%晚期。不同 NVC 模式的患病率在特定抗 ENA 抗体中均匀分布，除了抗 RNP 阳性患者中没有早期模式。51%的患者有间质性肺病（ILD），59%的患者一氧化碳弥散能力下降，16%的患者收缩期肺动脉压（sPAP↑）>35mmHg。无论 ENA 亚型如何，NVC 模式均与 ILD 或 sPAP↑存在稳定的相关性。对于 ILD，比值比（ORs）为 1.3-1.4（抗 RNApIII、抗 RNP 分析中 P<0.05）。对于一氧化碳弥散能力，OR 为 1.5（ACA、抗 Scl-70、抗 RNApIII、抗 RNP 分析中 P<0.05）。对于 sPAP↑，ORs 为 2.2-2.4（抗 RNApIII、抗 RNP 分析中 P<0.05）。
在荷兰 SSc 患者中，发现了所有的 SSc 特异性自身抗体，其中 ACA 和抗 Scl-70 最为常见。值得注意的是，NVC 模式与心肺受累之间的关联独立于特定的抗 ENA 抗体，这可能表明微血管病变是器官受累的一个重要原因。
