Teng Jialin, Zhang Wei
Unit of Rheumatology, Renji Hospital, Shanghai Jiaotong University, School of Medicine, Shanghai 200001, China.
Unit of Rheumatology, Renji Hospital, Shanghai Jiaotong University, School of Medicine, Shanghai 200001, China. Email:
Zhonghua Yi Xue Za Zhi. 2014 Apr 8;94(13):969-72.
To explore the clinical characteristics and therapeutic efficacy of systemic lupus erythematosus (SLE) associated pulmonary arterial hypertension (PAH).
A total of 91 cases of SLE-PAH from 2007 to 2011 were reviewed and followed up. They were divided into 2 groups: group A: New York Heart Association (NYHA) functional class 1 and 2; group B: NYHA functional class 3 and 4.
There were 2 males and 89 females with a mean age of 37 ± 11 years. The mean duration of SLE disease process was 7 ± 6 years. PAH was the primary symptom of SLE onset in 10 cases. Pulmonary arterial systolic pressure (PASP) as measured by ultrasonic cardiography (UCG) were between 40 to 128 mmHg. Eighteen cases (19.78%) underwent right heart catheterization (RHC). There were good parallels of PASP value between RHC and UCG. The main characteristics included Raynaud's phenomenon (53.8%), pericardial effusion (51.6%) and a high titer of anti-RNP antibody (57.1%). PASP was positively associated with SLE disease activity in mild and moderate cases. Among 27 mortality cases, there were 4 in group A (14.8%) and 23 in group B (85.2%) . And the causes of immortality were mostly non-cardiac in group A and right heart failure in group B. Cyclophosphamide was effective in mild and moderate cases. Forty-four cases received PAH target treatment and it could decrease the PASP in mild and moderate cases and significantly prolong the survival for severe cases.
The major clinical characteristics of SLE-PAH patients include Raynaud's phenomenon, pericardial effusion and positivity of anti-RNP antibody. PASP is positively associated with SLE disease activity in mild and moderate cases for whom intravenous pulse cyclophosphamide therapy may be effective. For severe cases, concomitant PAH target therapy may significantly improve the prognosis.
探讨系统性红斑狼疮（SLE）相关肺动脉高压（PAH）的临床特征及治疗效果。
回顾性分析2007年至2011年共91例SLE-PAH患者并进行随访。将其分为两组：A组：纽约心脏协会（NYHA）心功能1级和2级；B组：NYHA心功能3级和4级。
91例患者中男性2例，女性89例，平均年龄37±11岁。SLE病程平均7±6年。10例患者以PAH为SLE首发症状。超声心动图（UCG）测量的肺动脉收缩压（PASP）为40至128 mmHg。18例（19.78%）患者接受了右心导管检查（RHC）。RHC与UCG测量的PASP值具有良好的一致性。主要特征包括雷诺现象（53.8%）、心包积液（51.6%）和抗RNP抗体高滴度阳性（57.1%）。在轻、中度病例中，PASP与SLE疾病活动度呈正相关。27例死亡病例中，A组4例（14.8%），B组23例（85.2%）。A组死亡原因多为非心脏性，B组多为右心衰竭。环磷酰胺对轻、中度病例有效。4例患者接受了PAH靶向治疗，该治疗可降低轻、中度病例的PASP，并显著延长重度病例的生存期。
SLE-PAH患者的主要临床特征包括雷诺现象、心包积液和抗RNP抗体阳性。在轻、中度病例中，PASP与SLE疾病活动度呈正相关，静脉脉冲环磷酰胺治疗可能有效。对于重度病例，联合PAH靶向治疗可显著改善预后。
