Fusegawa S, Mikami T, Hashimoto M, Yamada Y, Kobayashi T, Yasuda H, Sagawa A, Fujisaku A
Department of Cardiovascular Medicine, Hokkaido University School of Medicine.
Kokyu To Junkan. 1991 Aug;39(8):819-23.
Recently pulmonary hypertension (PH) has been recognized as one of life threatening complications which determine the prognosis of patients with systemic lupus erythematosus (SLE). Clinical aspects and pathology in patients with SLE complicated by PH (SLE-PH) have been reported to have a close similarity to those in patients with primary pulmonary hypertension (PPH), the prognosis of which is very poor in general. However, the long-term prognosis for patients with SLE-PH is not clear yet. Mainly because of some technical limitations for determining the severity of the PH, long-term follow-up studies have been inconclusive. In this study, 7 patients with SLE-PH and 6 patients with PPH were studied by using two-dimensional echocardiography. The interventricular septal curvature (R) was measured from the parasternal short axis view and the reciprocal value (1/R) was applied as a severity index of the PH. In 4 patients with SLE-PH and 6 with PPH, pulmonary arterial pressure was measured by cardiac catheterization concomitantly. There were no significant differences in 1/R and pulmonary arterial pressure between the two groups when PH was diagnosed for the first time. In the PPH group, the mean period of follow-up study was 25.3 months and all of the patients died during that period. In SLE-PH group, the mean period of follow-up study was 50.6 months, and only 3 patients (43%) died during that period. No patients in the PPH group showed improvement in 1/R, but 3 patients with SLE-PH improved in 1/R during a long follow-up period (60, 96 and 103 months respectively). Thus, the prognosis for SLE-PH was better than that for PPH, and there were some differences in the course and worsening behavior of PH in SLE-PH compared with that in PPH during the long-term follow-up study with reference to the echocardiographic investigation using 1/R value.
最近，肺动脉高压（PH）已被公认为是决定系统性红斑狼疮（SLE）患者预后的危及生命的并发症之一。据报道，SLE合并PH（SLE-PH）患者的临床特征和病理与原发性肺动脉高压（PPH）患者极为相似，而PPH患者的总体预后通常很差。然而，SLE-PH患者的长期预后尚不清楚。主要由于确定PH严重程度存在一些技术限制，长期随访研究尚无定论。在本研究中，对7例SLE-PH患者和6例PPH患者进行了二维超声心动图检查。从胸骨旁短轴视图测量室间隔曲率（R），并将其倒数（1/R）用作PH的严重程度指标。在4例SLE-PH患者和6例PPH患者中，同时通过心导管检查测量肺动脉压。首次诊断为PH时，两组之间的1/R和肺动脉压无显著差异。在PPH组中，平均随访研究时间为25.3个月，在此期间所有患者均死亡。在SLE-PH组中，平均随访研究时间为50.6个月，在此期间只有3例患者（43%）死亡。PPH组中没有患者的1/R显示改善，但3例SLE-PH患者在长期随访期间（分别为60、96和103个月）的1/R有所改善。因此，SLE-PH的预后优于PPH，并且在使用1/R值进行超声心动图检查的长期随访研究中，SLE-PH与PPH相比，PH的病程和恶化行为存在一些差异。
