系统性红斑狼疮合并肺动脉高压46例临床分析  

Clinical analysis of therapy of 46 cases systemic lupus erythematosus associated with pulmonary arterial hypertension

作　　者：王治国[1] 刘学明 石哲群[3] 佟胜全[1] 吴萍[1] 

机构地区：[1]河北省唐山市工人医院风湿免疫科,063000 [2]河北省唐山市工人医院神经内科,063000 [3]唐山工人医院康复医院风湿免疫科

出　　处：《中国综合临床》2016年第10期892-895,共4页Clinical Medicine of China

基　　金：河北省医学科学研究重点课题计划（20150948）

摘　　要：目的：研究环磷酰胺、糖皮质激素联合西地那非对系统性红斑狼疮合并肺动脉高压（ SLE-PAH）的治疗效果，并对其预后不良因素进行分析。方法选取唐山市工人医院2009年1月至2015年1月收治的46例SLE-PAH患者作为研究对象，均给予环磷酰胺、西地那非及糖皮质激素治疗，疗程为24周。观察其疗效，比较治疗前后系统性红斑狼疮疾病活动评分（ SLEDAI）、6 min步行距离、心功能改善情况；比较有效和无效患者的临床资料，分析影响预后的不良因素。结果24周后治疗总有效率为78．3％（36／46），肺动脉压力（PASP）从治疗前（59±16） mmHg下降至（45±15） mmHg，治疗前后比较差异有统计学意义（t＝4．539，P＜0．01）；SLEDAI从治疗前（6．6±2．3）分下降至（2．4±0．8）分，治疗前后比较差异有统计学意义（t＝11．680，P＜0．01）；6 min步行距离从治疗前（402±96） m上升至（465±97） m，治疗前后比较差异有统计学意义（ t＝－3．137，P＜0．01）；心功能治疗后较治疗前改善明显，差异有统计学意义（ P＜0．01）。无效及恶化患者10例，年龄为（42±6）岁；治疗有效患者36例，年龄为（33±4）岁，两者年龄比较差异有统计学意义（ t＝－5．423，P＜0．01）。无效和恶化患者抗RNP抗体阳性率（90．0％，9／10）明显高于有效患者（52．8％，19／36），差异有统计学意义（χ2＝4．552，P＜0．01）。结论环磷酰胺联合西地那非及糖皮质激素对SLE-PAH患者有明显疗效，PASP、心功能、6 min步行距离是影响预后的重要参考指标；抗RNP抗体、年龄是预后不良因素。Objective To study the theraputic effect of cyclophosphamide,glucocorticoid combined with sildenafil on pulmonary hypertension in systemic lupus erythematosus（SLE-PAH） patients,and to analyze the prognosis adverse factors. Methods Forty-six cases patients with SLE-PAH from Tangshan Gongren Hospital from January 2009 to January 2015 were selected as research subjects. All patients were treated with prednisone, cyclophosphamide and sildenafil,and the course of treatment was 24 weeks. The treatment effect was observed, the systemic lupus erythematosus disease activity score（SLEDAI）,6 minutes walk distance and heart function before and after the treatment were compared. The effective and uneffective patients＇clinical and test information were compared,and the prognosis adverse factors were analyzed. Results After 24 weeks treatment,the total ef-fective rate was 78. 3%（36/46）. Pulmonary artery systolic pressure（PASP） dropped from （59±16） mmHg be-fore treatment to （45±15） mmHg after treatment,there was significant statistical difference（t=-4. 539,P〈0. 01）. SLEDAI dropped from （6. 6±2. 3） points to （2. 4±0. 8） points,,there was significant statistical differ-ence（t =11. 680,P〈0. 01）. The 6 minutes walk distance rose form （402±96） minutes to （465±97） minutes, there was significant statistical difference（ t=-3. 137,P〈0. 01） . Cardiac function improved significantly,there was significant statistical difference（ P〈0. 01） . There were 10 cases uneffective and deterirated patients,with the age of （42±6） years,36 cases effective patients with the age of （33±4） years,the difference was statistically significant（ t=-5. 423, P〈0. 01 ） . Uneffective and deterirated patients＇ anti-RNP antibody positive rate was 90. 9%（ 9/10 ） , significantly higher than the effective patients （ 52. 8%（ 19/36 ） ,χ2 = 4. 552, P〈0. 01 ） . Conclusion Cyclophosphamide, glucocorticoid combined with sildenafil has obvious effect on patients with SLE-PA

关 键 词：系统性红斑狼疮 肺动脉高压 环磷酰胺 西地那非 预后 

分 类 号：R593.241[医药卫生—内科学]