系统性硬化病患者死亡危险因素分析

目的 了解预测系统性硬化病(SSc)患者死亡的相关因素,以指导临床治疗.方法 回顾性收集门诊和病房的资料完整诊断明确的146例SSc患者的临床资料,包括起病年龄、性别、病程、雷诺现象、皮肤受累程度、胃食管反流、肾损害、硬皮病肾危象、心脏损害等情况,检测其血清中抗Scl-70、抗着丝点抗体、抗RNP抗体,应用超声心动图方法 检测其肺动脉压,应用影像学方法 检测其是否具有间质性肺炎,使用Cox回归方法 分析患者死亡相关的危险因素.结果死亡组患者和存活组患者雷诺现象、胃食管反流、抗核抗体、抗scl-70抗体、抗着丝点抗体、间质性肺炎、弥散功能下降、冠心病、外周动脉硬化发生率差异均无统计学意义(P>0.05);死亡组患者中起病年龄>60岁者更多(P=0.002)、男性更多(P=0.023);死亡组患者弥漫型皮肤受累(P=0.000)、抗RNP抗体阳性(P=0.014)、肺动脉高压(P=0.000)、心脏损害(P=0.000)、脑梗死(P=0.035)、肾损害(P=0.000)、硬皮病肾危象较存活组更常见(P=0.000).Cox回归分析表明,发病年龄>60岁[OR=5.441,95%可信区间(CI)(2.126～13.926,P=0.000]、男性(OR=5.531,95%CI 2.014～15.190,P=0.001)、抗RNP抗体阳性(OR=2.664,95%CI 1.016～6.592,P=0.034)、弥漫型皮肤受累(OR=3.432.95%CI 1.400～8.411,P=0.007)、肺动脉高压(OR=25.718,95%CI 5.954～111.085,P=0.000)、心脏受累(OR=4.141,95%CI 1.685～10.159,P=0.002)、肾脏受累(OR=4.214,95%CI 1.654～10.737,P=0.003)、硬皮病肾危象(OR=20.677,95%CI 4.161～102.764,P=0.000)是预测SSc死亡的危险因素,尤其是严重肺动脉高压(OR=55.809,95%CI 12.879～241.832,P=0.000)是SSc患者死亡的最强预测因素.结论 对于发病年龄>60岁、男性、弥漫型皮肤受累、抗RNP抗体阳性、心脏受累、肾受累、硬皮病肾危象及肺动脉高压患者,尤其是重度肺动脉高压患者,应积极治疗以改善其预后.

Risk factor of mortality in systemic sclerosis of Han nationality

Objective To determine the prognostic factors in systemic sclerosis.Methods Clinical data of definite systemic sclerosis patients were collected,including disease onset age,sex,disease course,Raynaud's phenomenon,skin involvement,gastroesophageal reflux,interstitial pneumonia,cardiac lesions,kidney lesions and scleroderma renal crisis.serum antibodies to scl-70.RNP and anti-centromere antibody were detected.Pulmonary artery pressure was measured by ultrasound cardiography.Cox hazard ratio model was employed to assess the mortality risk of systemic sclerosis patients.Results No difference in Raynaud's phenomenon,gastroesophageal reflux,anti-nuclear antybody,anti-sol-70 antibody,anti-centromere antibody,interstitial pneumonia,diffusion capacity (DLco),coronary artery disease,and peripheral artery atherosclerotic disease could be found between the dead and alive systemic sclerosis patients(P>0.05).Dead systemic sclerosis patients had later disease,onset(older than 60 years old)(P=0.002).Male gender(P=0.023),more diffuse skin involvement(P=0.000),more positive anti-RNP antibody(P=0.014),more pulmonary artery hypertension(P=0.000).more cardiac lesions(P=0.000),more cerebral infarets (P=0.035),more kidney lesions(P=0.000),and more scleroderma renal crisis(P=0.000) could be found jn dead sclerosis patients.Cox regression analysis showed that,onset later than 60 years old(OR=5.441.95% CI 2.126～13.926,P=0.000),male sex (OR=5.531,95%CI 2.014～15.190,P=0.001),anti-RNP antibedy positivity (OR=2.664,95%CI 1.016～6.592,P=0.034),diffuse skin involvement(OR=3.432,95%CI 1.400～8.411,P=0.007),pulmonary artery hypertension (OR=25.718,95% CI 5.954～111.085,P=0.000),cardiac lesions (OR=4.141.95%CI 1.685～10.159,P=0.002),kidney lesions(OR=4.214,95%CI 1.654～10.737,P=0.003) and scleroderma renal crisis (OR=20.677,95% CI 4.161～102.764.P=0.000)were risk factors for mortality in systemic sclerosis.Severe pulmonary hypertension was the most strong predictive factor for mortality in systemic sclerosis (OR=55.809,95% CI 12.879～241.832.P=0.000).Conclusion Aggressive therapy should be given to those systemic sclerosis patients with onset later than 60 years old,male sex,diffuse skin involvement,anti-RNP antibody positivity,cardiac involvement,kidney lesions,scleroderma renal crisis and pulmonary artery hypertension,especially seevere pulmonary hypertension.