结缔组织病相关肺动脉压的治疗.pptVIP

西地那非联合波生坦治疗（n=35） PAP mPAP CI PVR (mmHg) (mmHg) (L/min/m2) (dyn) 西地那非 10±4 63±22 2.2±0.9 1360±1440 西地那非 11±5 60±20 2.6±0.6 1120±1280 ＋波生坦 P 0.7 0.001 0.001 0.001 结 论 结缔组织病是导致PAH的重要常见原因 结缔组织病相关PAH的预后很差 治疗方法 传统治疗：钙离子拮抗剂疗效有限 针对原发病的治疗 部分结缔组织病相关PAH对免疫抑制治疗有效 靶向治疗：波生坦、前列环素类似物、西地那非 治疗步骤（建议方案） PAH-SLE/MCTD 传统治疗 (必要时利尿、吸氧，抗凝治疗，INR 2.0-3.0) NYHA I/II IV III(CI3.3L/min/m2) III(CI3.3L/min/m2) 单纯免疫抑制治疗 PAH特异性治疗联合免疫抑制治疗 4-6月后评价临床及血流动力学指标 4-6月后评价临床及血流动力学指标 有效 维持治疗(硫唑嘌呤/骁悉) 停止免疫抑制治疗，考虑联合治疗 随访临床和血流动力学改变 停止免疫抑制治疗，考虑PAH特异性治疗 无效 有效 无效 Arthritis Rheum 2008;58(2):521-31 * * * * * * A significant increase from baseline in mean walk distance was observed in bosentan-treated patients as early as Week?4 (at the 62.5?mg?b.i.d. dose). A further increase was obtained by Week?8 (with the target dose), which was maintained at the Week-16 assessment. In contrast, the initial non-significant mean increase from baseline with placebo was lost with a subsequent continual decrease over time. * 44 patients with PAH secondary to CTD who were treated with bosentan were stable in 6‐min walk distance at the end of the study (+19.5 m, 95% confidence interval (CI) ?3.2 to 42.2), whereas patients treated with placebo deteriorated (?2.6 m, 95% CI ?54.0 to 48.7). * The Kaplan–Meier estimates display the observed survival of the 64 patients with PAH secondary to CTD who were included in the two double‐blind studies and followed up in the open‐label study extensions. Survival on bosentan was 85.9% after 1 year and 73.4% after 2 years. Patients are considered from the 双盲、随机研究，their open‐label follow‐up studies. Kaplan–Meier survival estimates with 95% confidence limits for patients with PAH related to CTD, given bosentan as the preferred treatment. As the number of patients was decreasing over time, the cut‐off date was chosen as month 24. * * * Here are the results for the change in 6-minute walk distance versus baseline. As you can see, pati