糖皮质激素加霉酚酸酯治疗系统性硬化症合并肺动脉高压5例

目的探讨大剂量糖皮质激素加霉酚酸酯治疗系统性硬化症(SSc)合并肺动脉高压(PHT)的疗效。方法5例SSc合并PHT患者口服大剂量强的松(1mg·kg-1·d-1)及霉酚酸酯(1.5g·d-1)治疗,观察治疗前后临床症状和肺动脉高压的变化。结果5例患者在治疗后3个月内症状明显改善,3个月后肺动脉段突出明显减轻(P<0.01),肺动脉收缩压明显降低(P<0.01),1例于2年后死于严重肺纤维化。结论大剂量强的松加霉酚酸酯是治疗SSc合并PHT一种安全有效的方法。

Glucocorticoid combined with mycophenolate mofetil for treatment of 5 patients with systemic sclerosis accompanied by pulmonary hypertension

Objective To investigate the efficacy in large dose of glucocorticoid combined with mycophenolate mofetil for treatment of systemic sclerosis (SSc)accompanied by pulmonary hypertension(PHT). Methods Five cases of SSc accompanied by PHT were administered orally with large does of predmisone(1 mg·kg~ -1 ·d~ -1 ) and mycophenolate mofetil(1.5 g·d~ -1 ).Changes of clinical symptoms and pulmonary hypertension.Results The clinical symptoms in all cases improved obviously within3 months. The value of bulge of pulmonary artery segment was decreased obviously after3 months (P<0.01).Pumonary artery systolic pressure was obviously decreased (P<0.01).One case died of serious pulmonary fibrosis after two years. Conclusion Large dose of prednisone and mycophenolate mofetil therapy for SSc accompanied by PHT is a safe and effective method.