硬皮病脏器表现的临床危险评估

  背景：系统性硬化症是一种多系统性自身免疫性疾病，根据皮肤受累可分为弥漫皮肤型和局限皮肤型两个亚型。为更好地了解本病的血管、免疫和纤维化病变及指导治疗，2004年6月成立了欧洲抗风湿联盟硬皮病试验和研究组（EUSTAR）。

目的和方法：EUSTAR前瞻性地收集了所有符合美国风湿病学院诊断标准、到各试验中心就诊的系统性硬化症患者的极小本质数据集（MEDS），分析他们第一次就诊时的人口学、临床和实验室特点。

结果：到2006年4月，共有30个国家、102个研究中心的3656例患者（其中1349例弥漫皮肤型和2101例局限皮肤型）入组，其中1330例患者有抗Scl-70抗体，1106例患者有抗着丝点抗体，87%的患者为女性。通过多元回归分析发现，系统性硬化症分型、抗体状态和雷诺现象发生年龄（而非性别）独立地与脏器表现相关。抗体状态比系统性硬化症分型与临床表现的关系更紧密。

结论：弥漫皮肤型和局限皮肤型亚型与器官表现相关，但以抗体为基础的分类似乎更能预测硬皮病的某些并发症。EUSTAR MEDS数据库有助于系统性硬化症的临床类型分析，并有助于国际上系统性硬化症的标准评估和监测。

 BACKGROUND: Systemic sclerosis (SSc) is a multisystem autoimmune disease, which is classified into a diffuse cutaneous (dcSSc) and a limited cutaneous (lcSSc) subset according to the skin involvement. In order to better understand the vascular, immunological and fibrotic processes of SSc and to guide its treatment, the EULAR Scleroderma Trials And Research (EUSTAR) group was formed in June 2004.

AIMS AND METHODS: EUSTAR collects prospectively the Minimal Essential Data Set (MEDS) on all sequential patients fulfilling theAmericanCollegeof Rheumatology diagnostic criteria in participating centres. We aimed to characterise demographic, clinical and laboratory characteristics of disease presentation in SSc and analysed EUSTAR baseline visits.

RESULTS: In April 2006, a total of 3656 patients (1349 with dcSSc and 2101 with lcSSc) were enrolled in 102 centres and 30 countries. 1330 individuals had autoantibodies against Scl70 and 1106 against anticentromere antibodies. 87% of patients were women. On multivariate analysis, scleroderma subsets (dcSSc vs lcSSc), antibody status and age at onset of Raynaud's phenomenon, but not gender, were found to be independently associated with the prevalence of organ manifestations. Autoantibody status in this analysis was more closely associated with clinical manifestations than were SSc subsets.

CONCLUSION: dcSSc and lcSSc subsets are associated with particular organ manifestations, but in this analysis the clinical distinction seemed to be superseded by an antibody-based classification in predicting some scleroderma complications. The EUSTAR MEDS database facilitates the analysis of clinical patterns in SSc, and contributes to the standardised assessment and monitoring of SSc internationally.（Walker UA，Tyndall A,Czirják L, et al.Clinical risk assessment of organ manifestations in systemic sclerosis: a report from the EULAR Scleroderma Trials And Research group database.Ann Rheum Dis, 2007, 66(6):754-63.）