系统性硬化病合并肺动脉高压临床特点和治疗

目的探讨系统性硬化病(SSc)合并肺动脉高压(PHT)的临床特点、治疗方法和预后。方法回顾性研究28例SSc合并PHT患者的临床特点,实验室检查以及治疗后病情变化,并与73例未合并PHT的SSc患者进行比较。结果28例SSc合并PHT患者中,21例为血管源性PHT,7例为严重肺纤维化导致的肺源性PHT。与未合并PHT患者血清中白蛋白、γ-球蛋白、IgA、IgG以及C反应蛋白(CRP)水平间差别均有显著性意义(P<0.05)。肢端溃疡/指端凹陷性瘢痕、肌酶升高、肾脏受累、食管钡餐及ECG异常的发生率及ANA、抗RNP抗体阳性率间差别均有显著性意义(P<0.05)。结论上述指标有助于发现合并PHT的可能性;PHT是SSc的一个严重并发症,多伴有其他脏器损害,预后不良,应尽早采取综合治疗,尤其是针对原发病和诱因的强化治疗,血管扩张剂(主要是钙离子阻滞剂)和抗凝剂是治疗PHT的基本药物,病情顽固患者用前列环素制剂可改善预后。

The Clinical Features and Therapy of Pulmonary Hypertension Secondary to Systemic Selerosis

Objective To investigate the clinical features,therapy methods and prognosis of pulmonary hypertension (PHT) secondary to systemic sclerosis (SSc).Methods The clinical features,lab examination and outcome of 28 patients with PHT secondary to SSc were reported and compared with 73 SSC cases without PHT.Results PHT(systolic pressure>30 mm Hg) was diagnosed with Doppler echocardiography in 28 patients with SSc.Twenty-one patients have isolated PHT,while 7 patients were of secondary PHT which was due to severe pulmonary fibrosis.The levels of albumin,r-globulin,lgA,lgG and CRP in serum of patients with PHT were significantly higher than those without PHT(P<0.05).There were significantly differences in the incidences of pitting scars/ulcers,elevated muscle enzymes,barium swallow test,renal and ECG abnormalities and the positive rate of ANA,anti-RNP antibody in the patients with PHT and without PHT (P<0.05)Conclusion The above index can help find the possibility with PHT.PHT is a severe complication of SSc,often accompanied by multiorgan involvement and poor outcome;the comprehensive treatment should be adopted early,especially the intensive treatment for the primary affection and its cause.Vasodialator (usually caleium channel blocker) and anticoagulant are the principle medicine in treating PHT,and prostacyclin can be used to improve prognosis of the severe patients.