系统性硬化病患者临床表现分析：来自中国欧洲抗风湿病联盟硬皮病试验研究组数据库资料

Preliminary analysis of Chinese patients with systemic sclerosis： from Chinese EULAR SclerodermaTrial ＆ Research Group database

目的探讨中国系统性硬化病（SSc）患者脏器受累的情况，以了解中国ssc患者的临床特点。方法北京协和医院欧洲抗风湿病联盟硬皮病试验研究组（EUSTAR）数据库中，在2009年2月至2010年1月间前瞻性收集了SSc患者共119例，均满足1980年美国风湿病学会（ACR）SSc分类（诊断）标准。对其临床表现、实验室检查进行分析。采用X^2矿检验和独立样本t检验。结果（1）流行病学方面：女性109例，男性10例，平均年龄（44±12）岁，从雷诺现象到出现脏器受累的中位病程12个月，其中弥漫性SSc65例。112例患者（94．1％）有雷诺现象，雷诺现象的起病中位年龄36岁。其中91例患者以雷诺现象为首发表现。（2）临床表现方面：①以消化系统（70．6％），尤其是食管受累（56．3％），关节受累（54．6％）以及肺间质纤维化（PIF）（58．8％）最为常见，但肾危象（2．5％）、心脏传导阻滞（O）以及左室射血分数减低（0）很少见。②70例PIF中20例（29％）无呼吸系统的临床症状，为常规筛查时发现；65例在雷诺现象后3-352个月出现，中位时间34个月；24例肺动脉高压（PAH）中3例无临床症状；22例在雷诺现象后4-343个月出现，中位时间25个月；19例患者同时有PIF和PAH。③外周血管：肱踝指数水平显著降低（0．91±0．19与1．09±0．08，t=-2．288，P〈O．01）。（3）实验室检查：抗核抗体检查均为阳性；抗Scl-70抗体和抗着丝点抗体（ACA）阳性率分别为56．0％和14．7％，未见ACA及Sel-70同时阳性者；13％的患者RNA多聚酶Ⅲ抗体阳性。（4）弥漫性硬皮病（dcSSc）和局限性硬皮病（1cSSc）患者在脏器受累和实验室检查问的比较结果显示，dcsSc较1cssc患者指溃疡（40％与20％）更多见，ACA抗体少见（4／52与10／43，P〈O．05）。结论SSc皮肤外脏器受累并不少见，尤其是肺间质病变和消化系统受累。在中国患者中，肾危象及心脏传导阻滞明显减少。由于部分肺间质病变患者并无临床表现，因此早期进行筛查是早期诊断及治疗的关键。

Objective To investigate the clinical and laboratory characteristics of SSc patients in China. Method The data of 119 consecutive SSc patients based on EUSTAR DATABASE in Peking Union Medical College Hospital from February 2009 to January 2010 were prospectively collected and analyzed. All patients fulfilled ACR classification criteria in 1980 for SSc. The X^2 test and t-test were used to analyze the data. Resdts （1） Demographic data. Sex ratio （F/M） was 109/10 and the age rang was （44±12） years. There were 65 diffuse cutaneous SSc （dcSSc） patients and 54 limited cutaneous SSc （lcSSc） patients. 112 patients （94.1%） had Raynaud＇s phenomenon （RP）, and the age of RP occurrence was 36 years （13-76 years）, among which it was the initial presentation in 91 patients （81.3%） and the disease duration from RP to other manifestation was 12 months. （2） Clinical manifestations. ①The gastrointestinal manifestations （70.6%）, especially esophageal involvement （56.3%）, articular involvement （54.6%）, pulmonary interstitial fibrosis （PIF） （58.8%） were frequently observed, but renal crisis （2.5%）, heart block （0） and reduced LVEF （0） were rarely detected. ② Twenty cases （28.6%） out of 70 PIF patients denied any respiratory symptom and were confirmed by HRCT screening. The disease duration from RP to PIF was 34 months（3N352 months）; 3 case of 24 pulmonary artery hypertension （PAH） patients had no clinical manifestations. The disease duration from RP to PAH was 25 months （4-343 months）. Nineteen patients had PIF and PAH simuhaneously. ③Peripheral artery disease： SSc patients had a lower ankle brachial index （ABI） level （0.91＋ 0.19 vs 1.09＋0.08, P〈O.O01）. （3） Laboratory finding. All patients had positive ANA. The positive rate of anti-Scl-70 antibody and ACA was 56.0% and 14.7% respectively. There was no serum sample positive for anti-Scl-70 antibody and ACA. The positive rate of anti-RNA polymerase Ⅲ antibody was 13%. （4） Compared the clinical characteristics and laboratory findings between dcSSc and lcSSc patients, we found that there were significant differences between dcSSc and lcSSc patients in finger ulcer （40.0% vs 20.4%）, ACA positive rates （7.7% vs 23.3%, P〈O.05）. Conclusion The system involvements besides skin in SSc is common, especially PIF and gastrointestinal involvement. According to our data, there are fewer cases with renal crisis and heart block. Because part of patients with PIF have almost no clinical manifestations, so early screening for PIF/PAH is important for early diagnosis and intervention.