Ungerer R G, Tashkin D P, Furst D, Clements P J, Gong H, Bein M, Smith J W, Roberts N, Cabeen W
Am J Med. 1983 Jul;75(1):65-74. doi: 10.1016/0002-9343(83)91169-5.
Forty-nine patients with progressive systemic sclerosis who had undergone extensive studies including pulmonary artery catheterization as part of an ongoing prospective study of the natural course of progressive systemic sclerosis were evaluated. The overall prevalence of pulmonary arterial hypertension in this population of patients with progressive systemic sclerosis was 33 percent, and among 10 subjects with the CREST syndrome the prevalence of pulmonary hypertension was 50 percent. The relation between pulmonary arterial hypertension documented at catheterization and abnormal results of noninvasive studies suggesting pulmonary hypertension, including physical examination, chest x-ray, electrocardiography, echocardiography, single-breath diffusing capacity, and vital capacity, was studied. Diffusing capacity was significantly lower in those patients with definite pulmonary hypertension (mean pulmonary artery pressure of 22 mg Hg or more) compared with those with a normal mean pulmonary artery pressure, and a diffusing capacity below 43 percent of predicted showed the greatest sensitivity (67 percent) of any single diagnostic test in detecting definite pulmonary hypertension. Chest x-ray suggesting pulmonary hypertension was the least sensitive of the tests evaluated, but showed the greatest specificity (100 percent) in identifying patients with pulmonary hypertension. A classification matrix based on discriminant function analysis utilizing the combination of diffusing capacity below 43 percent of predicted and chest x-ray and electrocardiographic findings correctly identified 75 percent of patients with definite pulmonary hypertension and 97 percent of patients with a normal pulmonary artery pressure, but failed to identify correctly patients with mild pulmonary hypertension (mean pulmonary artery pressure of 20 mm Hg). These findings indicate that specific noninvasive studies are helpful in assessing the likelihood of normal or definitely elevated pulmonary artery pressures in patients with progressive systemic sclerosis, but patients with mild pulmonary hypertension are not likely to be identified by these noninvasive studies.
对49例进行性系统性硬化症患者进行了评估，这些患者作为正在进行的关于进行性系统性硬化症自然病程的前瞻性研究的一部分，接受了包括肺动脉导管插入术在内的广泛检查。在这组进行性系统性硬化症患者中，肺动脉高压的总体患病率为33%，在10例患有CREST综合征的患者中，肺动脉高压的患病率为50%。研究了导管插入术记录的肺动脉高压与提示肺动脉高压的非侵入性检查异常结果之间的关系，这些非侵入性检查包括体格检查、胸部X线、心电图、超声心动图、单次呼吸弥散能力和肺活量。与平均肺动脉压正常的患者相比，明确患有肺动脉高压（平均肺动脉压为22 mmHg或更高）的患者的弥散能力显著降低，在检测明确的肺动脉高压方面，预测值低于43%的弥散能力在所有单项诊断测试中显示出最高的敏感性（67%）。提示肺动脉高压的胸部X线检查在评估的测试中敏感性最低，但在识别肺动脉高压患者方面显示出最高的特异性（100%）。基于判别函数分析的分类矩阵，利用预测值低于43%的弥散能力、胸部X线和心电图结果的组合，正确识别了75%的明确患有肺动脉高压的患者和97%的肺动脉压正常的患者，但未能正确识别轻度肺动脉高压（平均肺动脉压为20 mmHg）的患者。这些发现表明，特定的非侵入性检查有助于评估进行性系统性硬化症患者肺动脉压正常或明显升高的可能性，但这些非侵入性检查不太可能识别出轻度肺动脉高压患者。
