CAJ | 학술논문

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x2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SSc 65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(0)以及左室射血分数减低(0)很少见.②70例PIF中加例(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<0.01).(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Scl-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(IcSSc)患者在脏器受累和实验室检查间的比较结果显示,dcSSc较IcSSc患者指溃疡(40%与20%)更多见,ACA抗体少见(4/52与10/43,P<0.05).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.
목적 탐토중국계통성경화병(SSc)환자장기수루적정황,이료해중국SSc환자적림상특점.방법 북경협화의원구주항풍습병련맹경피병시험연구조(EUSTAR)수거고중,재2009년2월지2010년1월간전첨성수집료SSc환자공119례,균만족1980년미국풍습병학회(ACR)SSc분류(진단)표준.대기림상표현、실험실검사진행분석.채용x2검험화독립양본t검험.결과 (1)류행병학방면:녀성109례,남성10례,평균년령(44±12)세,종뢰낙현상도출현장기수루적중위병정12개월,기중미만성SSc 65례.112례환자(94.1%)유뢰낙현상,뢰낙현상적기병중위년령36세.기중91례환자이뢰낙현상위수발표현.(2)림상표현방면:①이소화계통(70.6%),우기시식관수루(56.3%),관절수루(54.6%)이급폐간질섬유화(PIF)(58.8%)최위상견,단신위상(2.5%)、심장전도조체(0)이급좌실사혈분수감저(0)흔소견.②70례PIF중가례(29%)무호흡계통적림상증상,위상규사사시발현;65례재뢰낙현상후3～352개월출현,중위시간34개월;24례폐동맥고압(PAH)중3례무림상증상;22례재뢰낙현상후4～343개월출현,중위시간25개월;19례환자동시유PIF화PAH.③외주혈관:굉과지수수평현저강저(0.91±0.19여1.09±0.08,t=-2.288,P<0.01).(3)실험실검사:항핵항체검사균위양성;항Scl-70항체화항착사점항체(ACA)양성솔분별위56.0%화14.7%,미견ACA급Scl-70동시양성자;13%적환자RNA다취매Ⅲ항체양성.(4)미만성경피병(dcSSc)화국한성경피병(IcSSc)환자재장기수루화실험실검사간적비교결과현시,dcSSc교IcSSc환자지궤양(40%여20%)경다견,ACA항체소견(4/52여10/43,P<0.05).결론 SSc피부외장기수루병불소견,우기시폐간질병변화소화계통수루.재중국환자중,신위상급심장전도조체명현감소.유우부분폐간질병변환자병무림상표현,인차조기진행사사시조기진단급치료적관건.
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. Thex2 test and t-test were used to analyze the data. Results (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 (1cSSc) 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. ① The 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(3～352months); 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 simultaneously. ③Peripheral artery disease: SSc patients had a lower ankle brachial index (ABI) level (0.91± 0.19 vs 1.09±0.08, P<0.00l). (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<0.05). 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.