Bonham Catherine A, Oldham Justin M, Gomberg-Maitland Mardi, Vij Rekha
Section of Pulmonary and Critical Care Medicine, University of Chicago, Chicago, IL.
Section of Pulmonary and Critical Care Medicine, University of Chicago, Chicago, IL.
Chest. 2015 Oct;148(4):1055-1062. doi: 10.1378/chest.14-2546.
It is unclear whether recent advances in pulmonary arterial hypertension therapy can be safely applied to sarcoidosis-associated pulmonary hypertension (SAPH). Evidence for prostacyclin (PG) therapy in SAPH is limited.
We conducted a single-center, retrospective review of 46 patients with sarcoidosis, 26 of whom had SAPH. Thirteen received PG as monotherapy or in combination with oral vasodilators.
Follow-up right-sided heart catheterization at a mean of 12.7 months revealed improved cardiac output, cardiac index, and pulmonary vascular resistance. Functional class and N-terminal pro-brain natriuretic peptide levels also improved in patients treated with PG. No significant change in oxygen requirement was seen with vasodilator therapy initiation. At 2 years, 15 patients with SAPH survived, including eight on PG, and at 5 years, seven survived, including five on PG. Survival was significantly reduced in patients with SAPH compared with patients who had sarcoidosis without pulmonary hypertension. Multivariate analysis demonstrated that the use of PG therapy in SAPH is not associated with increased mortality.
Many patients with severe SAPH showed significant hemodynamic and clinical improvement on long-term IV or subcutaneous PG therapy and had survival outcomes similar to patients with moderate SAPH on oral vasodilator therapy.
尚不清楚肺动脉高压治疗方面的最新进展能否安全应用于结节病相关肺动脉高压（SAPH）。关于前列环素（PG）治疗SAPH的证据有限。
我们对46例结节病患者进行了单中心回顾性研究，其中26例患有SAPH。13例接受了PG单药治疗或与口服血管扩张剂联合治疗。
平均12.7个月时的随访右心导管检查显示心输出量、心脏指数和肺血管阻力有所改善。接受PG治疗的患者功能分级和N末端脑钠肽前体水平也有所改善。开始使用血管扩张剂治疗后，氧需求未见明显变化。2年时，15例SAPH患者存活，其中8例接受PG治疗；5年时，7例存活，其中5例接受PG治疗。与无肺动脉高压的结节病患者相比，SAPH患者的生存率显著降低。多变量分析表明，SAPH患者使用PG治疗与死亡率增加无关。
许多重度SAPH患者在长期静脉或皮下PG治疗后血流动力学和临床症状有显著改善，生存结果与接受口服血管扩张剂治疗的中度SAPH患者相似。
