Scleroderma Renal Crisis: Risk Factors for an Increasingly Rare Organ Complication
Scleroderma Renal Crisis: Risk Factors for an Increasingly Rare Organ Complication
复制标题
DOI：
10.3899/jrheum.180582
复制
发表时间：
2020-02-01
影响因子：
3.9
通讯作者：
Hunzelmann, Nicolas
中科院分区：
文献类型：
--
作者：
Moinzadeh, Pia;Kuhr, Kathrin;Hunzelmann, Nicolas
Objective.硬皮病肾危象（SRC）是系统性硬化症（SSc）患者的严重危及生命的表现。然而，关于SRC的危险因素的知识是有限的。我们确定了SRC的频率，并确定了预测SRC的危险因素。基于德国系统性硬皮病网络的定期随访数据，我们使用单变量和多变量广义估计方程来分析临床变量、SSc子集、治疗[即，血管紧张素转换酶抑制剂（ACEi）、皮质类固醇]和SRC的发生。2873例患者的10，425次访视数据可用于分析，平均登记研究访视次数为3.6 +/- 2.8，平均随访时间为3.6 +/- 3.8年。共有70例患者发生SRC（70/ 2873，2.4%）。在这些患者中，57.1%（40/70）被诊断为弥漫性皮肤SSc，31.4%（22/70）为局限性皮肤SSc，11.4%（8/70）为SSc重叠综合征。SRC发生概率最高的独立预测因素是抗RNA聚合酶抗体（RNAP）阳性、SRC发作前有蛋白尿史、DLCO减少和高血压史。有趣的是，抗拓扑异构酶自身抗体阳性并不能预测SRC的高风险。此外，SRC患者更频繁地接受ACEi和皮质类固醇治疗，而与SRC无关。在这个队列中，SRC已成为一种罕见的并发症。到目前为止，SRC的最高风险与抗RNAP和蛋白尿的检测相关。
Objective. Scleroderma renal crisis (SRC) is a severe life-threatening manifestation in patients with systemic sclerosis (SSc). However, the knowledge about risk factors for SRC is limited. We determined here the frequency of SRC and identified risk factors for the prediction of SRC.Methods. Based on regular followup data from the German Network for Systemic Scleroderma, we used univariate and multivariate generalized estimating equations to analyze the association between clinical variables, SSc subsets, therapy [i.e., angiotensin-converting enzyme inhibitors (ACEi), corticosteroids], and the occurrence of SRC.Results. Data of 2873 patients with 10,425 visits were available for analysis with a mean number of registry visits of 3.6 +/- 2.8 and a mean time of followup of 3.6 +/- 3.8 years. In total, 70 patients developed SRC (70/ 2873, 2.4%). Of these patients, 57.1% (40/70) were diagnosed with diffuse cutaneous SSc, 31.4% (22/70) with limited cutaneous SSc, and 11.4% (8/70) with SSc- overlap syndromes. Predictive independent factors with the highest probability for SRC were positive anti-RNA polymerase antibodies (RNAP), a history of proteinuria prior to SRC onset, diminished DLCO, and a history of hypertension. Interestingly, positive antitopoisomerase autoantibodies did not predict a higher risk for SRC. Further, patients with SRC were significantly more frequently treated with ACEi and corticosteroids without being independently associated with SRC.Conclusion. In this cohort, SRC has become a rare complication. By far the highest risk for SRC was associated with the detection of anti-RNAP and proteinuria.