系统性硬化症诊断时发生未来硬皮病性肾危象的风险因素。
Risk Factors for Future Scleroderma Renal Crisis at Systemic Sclerosis Diagnosis.
机构信息
From the Nephrology Department, and the Rheumatology Department, Walter Reed National Military Medical Center; the Uniformed Services University of the Health Sciences, Bethesda, Maryland, USA.
S.W. Olson, MD, Nephrology Department, Walter Reed National Military Medical Center; S.M. Gordon, MD, Nephrology Department, Walter Reed National Military Medical Center; R. Nee, MD, Nephrology Department, Walter Reed National Military Medical Center; R.S. Stitt, MD, Rheumatology Department, Walter Reed National Military Medical Center; W.T. Bailey, MD, Rheumatology Department, Walter Reed National Military Medical Center; D.J. Little, MD, Nephrology Department, Walter Reed National Military Medical Center; K.R. Knight, MD, Nephrology Department, Walter Reed National Military Medical Center; J.B. Hughes, Medical Student, Uniformed Services University of the Health Sciences; J.D. Edison, MD, Rheumatology Department, Walter Reed National Military Medical Center.
出版信息
J Rheumatol. 2019 Jan;46(1):85-92. doi: 10.3899/jrheum.171186. Epub 2018 Jul 15.
OBJECTIVE
Systemic sclerosis (SSc) is a disease of autoimmunity, fibrosis, and vasculopathy. Scleroderma renal crisis (SRC) is one of the most severe complications. Corticosteroid exposure, presence of anti-RNA polymerase III antibodies (ARA), skin thickness, and significant tendon friction rubs are among the known risk factors at SSc diagnosis for developing future SRC. Identification of additional clinical characteristics and laboratory findings could expand and improve the risk profile for future SRC at SSc diagnosis.
METHODS
In this retrospective cohort study of the entire military electronic medical record between 2005 and 2016, we compared the demographics, clinical characteristics, and laboratory results at SSc diagnosis for 31 cases who developed SRC after SSc diagnosis to 322 SSc without SRC disease controls.
RESULTS
After adjustment for potential confounding variables, at SSc diagnosis these conditions were all associated with future SRC: proteinuria (p < 0.001; OR 183, 95% CI 19.1-1750), anemia (p = 0.001; OR 9.9, 95% CI 2.7-36.2), hypertension (p < 0.001; OR 13.1, 95% CI 4.7-36.6), chronic kidney disease (p = 0.008; OR 20.7, 95% CI 2.2-190.7), elevated erythrocyte sedimentation rate (p < 0.001; OR 14.3, 95% CI 4.8-43.0), thrombocytopenia (p = 0.03; OR 7.0, 95% CI 1.2-42.7), hypothyroidism (p = 0.01; OR 2.8, 95% CI 1.2-6.7), Anti-Ro antibody seropositivity (p = 0.003; OR 3.9, 95% CI 1.6-9.8), and ARA (p = 0.02; OR 4.1, 95% CI 1.2-13.8). Three or more of these risk factors present at SSc diagnosis was sensitive (77%) and highly specific (97%) for future SRC. No SSc without SRC disease controls had ≥ 4 risk factors.
CONCLUSION
In this SSc cohort, we present a panel of risk factors for future SRC. These patients may benefit from close observation of blood pressure, proteinuria, and estimated glomerular filtration rate, for earlier SRC identification and intervention. Future prospective therapeutic studies could focus specifically on this high-risk population.
目的
系统性硬化症（SSc）是一种自身免疫、纤维化和血管病变疾病。硬皮病肾危象（SRC）是最严重的并发症之一。在 SSc 诊断时，皮质类固醇暴露、存在抗 RNA 聚合酶 III 抗体（ARA）、皮肤厚度和明显的肌腱摩擦音等是未来发生 SRC 的已知危险因素。确定其他临床特征和实验室发现可以扩展和改善 SSc 诊断时未来 SRC 的风险状况。
方法
在这项回顾性队列研究中，我们比较了 2005 年至 2016 年间整个军事电子病历中 31 例 SSc 诊断后发生 SRC 的患者和 322 例无 SRC 的 SSc 疾病对照患者的人口统计学、临床特征和实验室结果。
结果
在调整了潜在混杂变量后，在 SSc 诊断时，以下情况均与未来 SRC 相关：蛋白尿（p < 0.001；OR 183，95%CI 19.1-1750）、贫血（p = 0.001；OR 9.9，95%CI 2.7-36.2）、高血压（p < 0.001；OR 13.1，95%CI 4.7-36.6）、慢性肾脏病（p = 0.008；OR 20.7，95%CI 2.2-190.7）、红细胞沉降率升高（p < 0.001；OR 14.3，95%CI 4.8-43.0）、血小板减少症（p = 0.03；OR 7.0，95%CI 1.2-42.7）、甲状腺功能减退症（p = 0.01；OR 2.8，95%CI 1.2-6.7）、抗 Ro 抗体阳性（p = 0.003；OR 3.9，95%CI 1.6-9.8）和 ARA（p = 0.02；OR 4.1，95%CI 1.2-13.8）。在 SSc 诊断时存在三种或更多这些危险因素的患者对未来 SRC 的敏感性（77%）和特异性（97%）较高。在无 SRC 的 SSc 疾病对照组中，没有≥4 个危险因素。
结论
在这项 SSc 队列研究中，我们提出了一个未来 SRC 的风险因素组合。这些患者可能需要密切监测血压、蛋白尿和估计肾小球滤过率，以便更早地发现并干预 SRC。未来的前瞻性治疗研究可以专门针对这一高危人群。
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