Strange C, Bolster M, Mazur J, Taylor M, Gossage J R, Silver R
Division of Pulmonary and Critical Care Medicine, Allergy and Clinical Immunology, Medical University of South Carolina, Charleston, USA.
Chest. 2000 Oct;118(4):1077-82. doi: 10.1378/chest.118.4.1077.
To determine the cause of pulmonary hypertension (PH) in systemic sclerosis (SSc) patients since PH can occur because of pulmonary arteriopathy, pulmonary parenchymal destruction, and left ventricular cardiac dysfunction.
Consecutive case series in a university hospital.
Nine SSc patients with PH (mean pulmonary artery pressure, 41 mm Hg), with (n = 6) or without (n = 3) concomitant interstitial lung disease (ILD).
Acute infusion of epoprostenol was begun at 2 ng/kg/min and was titrated upward at a rate of 2 ng/kg/min every 30 min until symptomatic complications developed or pulmonary artery vascular resistance (PVR) was reduced by 50%.
Eight of nine patients demonstrated a reduction of > or = 20% in PVR, suggesting that vasoreactivity is common despite the presence of significant ILD. A single patient had no response to infusion with unchanged hemodynamics and oxygenation. One patient developed hypoxemia as cardiac output increased, suggesting a worsening of ventilation/perfusion matching or the presence of an anatomic shunt. Acute pulmonary edema developed in one patient at an infusion rate of 6 ng/kg/min. The results of cardiac catheterization suggested that pulmonary edema was caused by SSc heart disease.
SSc patients with ILD have diverse and sometimes multiple causes of PH that can be determined by short-term epoprostenol infusion. Beneficial effects can be obtained from epoprostenol despite extensive ILD.
确定系统性硬化症（SSc）患者肺动脉高压（PH）的病因，因为PH可能由肺动脉病变、肺实质破坏和左心室心功能不全引起。
大学医院的连续病例系列研究。
9例患有PH的SSc患者（平均肺动脉压为41mmHg），其中6例伴有间质性肺疾病（ILD），3例不伴有ILD。
开始以2 ng/kg/min的速度急性输注依前列醇，每30分钟以2 ng/kg/min的速度递增，直至出现症状性并发症或肺动脉血管阻力（PVR）降低50%。
9例患者中有8例PVR降低≥20%，这表明尽管存在严重的ILD，血管反应性仍很常见。1例患者对输注无反应，血流动力学和氧合情况未改变。1例患者在心输出量增加时出现低氧血症，提示通气/灌注匹配恶化或存在解剖分流。1例患者在输注速度为6 ng/kg/min时发生急性肺水肿。心导管检查结果提示肺水肿由SSc心脏病引起。
患有ILD的SSc患者的PH病因多样，有时是多种原因，可通过短期输注依前列醇来确定。尽管存在广泛的ILD，但依前列醇仍可产生有益效果。
