Steen Virginia D
Department of Medicine, Georgetown University, Washington, DC 2007, USA.
Semin Arthritis Rheum. 2005 Aug;35(1):35-42. doi: 10.1016/j.semarthrit.2005.03.005.
To describe the clinical, laboratory, and prognostic features associated with the scleroderma-specific autoantibodies.
Using the Pittsburgh Scleroderma Databank, all consecutive patients seen between 1980 and 1995 who had autoantibody studies performed were studied. Anticentromere antibodies (ACA), antitopoisomerase (TOPO), anti-U1-RNP (U1-RNP), anti-RNA Polymerase III (Pol 3), anti-U3-RNP (U3-RNP), anti-Th/To (Th/To), and anti-Pm/Scl (Pm/Scl) were determined according to previously described methods. The frequency of clinical features, organ system outcomes, and survival within the patients with a specific antibody were cumulative over the course of the disease. The frequency of a specific feature was compared across groups to identify significant manifestations and outcomes in patients with a specific antibody.
Some demographic, clinical, and organ system findings were associated with the specific antibody, and other features with the scleroderma subtype (limited cutaneous or diffuse cutaneous scleroderma). U3-RNP, U1-RNP, and TOPO were seen more commonly in African-American patients, and ACA was seen in older, female Caucasians. Muscle inflammation was seen in patients with U1-RNP and U3-RNP. Digital tip ulcers and digital tuft resorption were seen more frequently in those with ACA and TOPO. A vasculopathy causing pulmonary hypertension typically occurs with ACA and pulmonary fibrosis with TOPO; however, both types of lung disease were seen in patients with nucleolar antibodies, Th/To and U3-RNP. Importantly, severe interstitial fibrosis was rarely seen in cases with Pol 3. Renal crisis was strongly associated with Pol 3. Survival within limited scleroderma was decreased in the Th/To patients compared with ACA patients. Within the diffuse scleroderma group, patients with Pol 3 had the best survival.
Scleroderma autoantibodies are associated with very specific demographic, clinical, organ system, and survival features.
The determination of scleroderma autoantibodies may be helpful in assessing the prognosis, monitoring, and treatment of scleroderma patients.
描述与硬皮病特异性自身抗体相关的临床、实验室及预后特征。
利用匹兹堡硬皮病数据库，对1980年至1995年间所有进行过自身抗体检测的连续患者进行研究。根据先前描述的方法测定抗着丝点抗体（ACA）、抗拓扑异构酶（TOPO）、抗U1核糖核蛋白（U1-RNP）、抗RNA聚合酶III（Pol 3）、抗U3核糖核蛋白（U3-RNP）、抗Th/To（Th/To）和抗Pm/Scl（Pm/Scl）。特定抗体患者的临床特征、器官系统结局及生存率在疾病过程中进行累积。比较不同组间特定特征的频率，以确定特定抗体患者的显著表现和结局。
一些人口统计学、临床及器官系统发现与特定抗体相关，其他特征与硬皮病亚型（局限性皮肤型或弥漫性皮肤型硬皮病）相关。U3-RNP、U1-RNP和TOPO在非裔美国患者中更常见，而ACA在老年白人女性中更常见。U1-RNP和U3-RNP患者可见肌肉炎症。指端溃疡和指腹吸收在ACA和TOPO患者中更频繁出现。导致肺动脉高压的血管病变通常与ACA相关，肺纤维化与TOPO相关；然而，两种类型的肺部疾病在有核仁抗体、Th/To和U3-RNP的患者中均可见。重要的是，Pol 3患者很少出现严重的间质性纤维化。肾危象与Pol 3密切相关。与ACA患者相比，Th/To患者局限性硬皮病的生存率降低。在弥漫性硬皮病组中，Pol 3患者的生存率最佳。
硬皮病自身抗体与非常特定的人口统计学、临床、器官系统及生存特征相关。
硬皮病自身抗体的测定可能有助于评估硬皮病患者的预后、监测及治疗。
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