肝脏结节性再生性增生：系统性硬化症中的一种罕见血管并发症。
Nodular Regenerative Hyperplasia of the Liver: A Rare Vascular Complication in Systemic Sclerosis.
机构信息
From the Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland.
L. Graf, cand.med., Department of Rheumatology, University Hospital Zurich; R. Dobrota, MD, Department of Rheumatology, University Hospital Zurich; S. Jordan, MD, Department of Rheumatology, University Hospital Zurich; L.M. Wildi, MD, Department of Rheumatology, University Hospital Zurich; O. Distler, MD, Department of Rheumatology, University Hospital Zurich; B. Maurer, MD, Department of Rheumatology, University Hospital Zurich.
出版信息
J Rheumatol. 2018 Jan;45(1):103-106. doi: 10.3899/jrheum.170292. Epub 2017 Nov 1.
OBJECTIVE
To investigate nodular regenerative hyperplasia (NRH) as a vascular complication of systemic sclerosis (SSc) with microvasculopathy as a common denominator.
METHODS
Cases of SSc-NRH were identified by systematic literature review and by screening the Zurich cohort. NRH had to be diagnosed by liver biopsy.
RESULTS
Literature review retrieved 22 cases. In our cohort, 1.4% of patients with SSc were diagnosed with NRH. Most had vasculopathy, were positive for anticentromere antibodies, had elevated alkaline phosphatase and gamma-glutamyl transferase levels, normal liver morphology on ultrasound yet increased stiffness on ultrasound elastography, and had portal hypertension.
CONCLUSION
NRH might represent a rare yet potentially life-threatening vascular complication in SSc.
目的
探讨结节性再生性增生（NRH）作为系统性硬化症（SSc）的血管并发症，其微血管病变为共同特征。
方法
通过系统文献回顾和苏黎世队列筛查确定 SSc-NRH 病例。NRH 必须通过肝活检诊断。
结果
文献回顾检索到 22 例。在我们的队列中，1.4%的 SSc 患者被诊断为 NRH。大多数患者存在血管病变，抗着丝点抗体阳性，碱性磷酸酶和γ-谷氨酰转移酶水平升高，超声检查肝脏形态正常，但超声弹性成像显示硬度增加，且存在门脉高压。
结论
NRH 可能代表 SSc 中一种罕见但潜在危及生命的血管并发症。
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