摘要
目的：探讨系统性硬化病（SSc）合并肺动脉高压（PAH）患者的临床特点、治疗方法及预后。方法回顾性分析16例 SSc 合并 PAH 患者（PAH 组）的临床特点，并与74例未合并 PAH 患者（非PAH 组）进行比较。PAH 组患者接受 PAH 基础治疗（包括氧疗、抗凝剂、强心、利尿等）、抗风湿、肺血管扩张剂靶向治疗，观察其效果。结果 PAH 组与非 PAH 组抗核抗体（ ANA）阳性率分别为87.5％（14/16）、75.7％（56/74），两组比较差异无统计学意义（P =0.508）；PAH 组的血白蛋白为（32.6±4.6） g/ L，红细胞沉降率为（48.4±29.4）mm/1 h，肢端病变、蛋白尿、血尿、心电图异常发生率分别为62.5％（10/16）、62.5％（10/16）、43.8％（7/16）、62.5％（10/16）；非 PAH 组血白蛋白为（35.6±5.0）g/ L、红细胞沉降率为（31.3±26.3）mm/1 h，肢端病变、蛋白尿、血尿、ECG 异常发生率分别为31.1％（23/74）、27.0％（20/74）、12.2％（9/74）、9.5％（7/74）；以上指标两组比较差异均有统计学意义（ P 值分别为0.033、0.041、0.018、0.006、0.003、0.000）。74例非 PAH 组患者长期随访病情平稳；16例 PAH 患者中，1例轻度 PAH 失访，1例重度 PAH 因重症肺炎、肺动脉高压、右心功能不全、呼吸衰竭死亡；3例轻度 PAH患者原发病有活动；其余11例 PAH 组患者复查胸部 HRCT 显示肺间质病变较前减轻。心脏多普勒超声测量肺动脉收缩压（ PASP）由治疗前的（48.9±2.4）mmHg 降至（31.5±4.5）mmHg（ t =22.27，P=0.001），患者心功能改善，未见其他不良反应。结论 SSc 合并 PAH 多伴有其他脏器损害，预后不良，建议早期行心脏多普勒超声检查，以求早期诊治。治疗上，除氧疗、利尿、强心及抗凝治疗外，应针对原发病和诱因的治疗及酌情联用肺血管扩张剂靶向治疗，以改善预后。
Objective To investigate the clinical features of systemic sclerosis（ SSc）patients with pulmonary hypertension（PAH）ane its treatment approach ane prognosis. Methods The clinical information of 16 SSc patients with PAH（PAH group）were recoreee. Seventy-four SSc without PAH were servee as no-PAH group. Patients in PAH group were given the basic therapy inclueing oxygen therapy,anticoagulants,careiac, eiuretic,anti-rheumatic,pulmonary vasoeilator therapy. Results The rate of antinuclear antiboey in PAH ane non-PAH group were 87. 5%（14 / 16）ane 75. 7%（56 / 74）,ane the eifference was statistically significant（P= 0. 508）. Serum albumin,erythrocyte seeimentation rate at 1 h in PAH group were（32. 6 ± 4. 6）g/ L ane （48. 4 ± 29. 4）mm/ 1 h. The rate of acral lesion proteinuria,hematuria,ECG abnormal rate were 62. 5%（10 / 16）,62. 5%（10 / 16）,43. 8%（7 / 16）,62. 5%（10 / 16）respectively in PAH group. Serum albumin, erythrocyte seeimentation rate at 1 h in PAH group were（35. 6 ± 5. 0）g/ L ane（31. 3 ± 26. 3）mm/ 1 h in non-PAH group. The rate of acral lesions,proteinuria,hematuria,ECG abnormal rate were 31. 1%（23 / 74）,27. 0%（20 / 74）,12. 2%（9 / 74）,9. 5%（ 7 / 74 ） respectively in non-PAH group. The eifferences were significant between in terms of all above ineices（P = 0. 033,0. 041,0. 018,0. 006,0. 003,0. 000）. During follow-up,the eisease was in stable in 74 case of non-PAH. Among 16 case with PAH,1 mile case was lost,1 case with severe PAH eiee of severe pneumonia,pulmonary hypertension,right ventricular failure,respiratory failure,3 mile patient with PAH were with eevelopment of primary eisease ane the rest 11 cases of PAH patients were with lower interstitial lung eisease than that of the previous eetectee by chest HRCT. Oppler echocareiography measurement of pulmonary artery systolic pressure was（48. 9 ± 2. 4）mmHg before treatment ane then reeucee to（31. 5 ± 4. 5）mmHg in rest 11 cases（t = 22. 27;P = 0. 001）measuree by Doppler echocareiography. Careiac function was improvee euring followee up ane no other aeverse reactions were seen. Conclusion SSc patients merge multiple PAH show it associate with other organ eamage,ane has a poor prognosis. Early careiac Doppler ultrasoune shoule be performee in oreer to get early eiagnosis ane treatment. Treatment approaches shoule be targetee at the primary eisease ane incentives such as pulmonary besiee oxygen therapy,eiuretics,careiac ane anticoagulant erugs in oreer to improve prognosis.
出处
《中国综合临床》
2014年第6期602-605,共4页
Clinical Medicine of China
关键词
系统性硬化病
肺动脉高压
肺血管扩张剂靶向治疗
预后
Systemic sclerosis
Pulmonary hypertension
Pulmonary vasoeilator targetee therapy
Prognosis
