系统性红斑狼疮合并肺动脉高压38例临床分析

目的 分析系统性红斑狼疮(SLE)合并肺动脉高压(PHT)的发病机制、临床特点、治疗及预后.方法 对38例诊断明确、资料完整的SLE合并PHT患者进行回顾性分析.结果 38例患者中出现肺动脉高压距SLE确诊的平均间隔期为2.3年;其中3例为严重PHT;伴明显的心力衰竭;雷诺现象27例;38例患者抗核抗体(ANA)均为阳性,抗dsDNA抗体阳性21例,抗Sm抗体阳性16例,抗SSA、SSB抗体阳性8例,抗磷脂抗体升高4例,类风湿因子(RF)升高13例,抗U1RNP抗体阳性15例;合并肺纤维化者7例.所有患者经激素及免疫抑制剂等治疗后,3例死亡,余35例病情稳定.结论 PHT是SLE的一个严重并发症,预后不良,雷诺现象是其早期的临床表现.早期诊断、早期联合治疗PHT是控制病情的关键.

Clinical analysis of 38 systemic lupus erythematosus patients complicated with pulmonary hypertension

Objective To analyze the pathogenesis, clinical features, treatment and prognosis of pulmonary hypertension (PHT) in systemic lupus erythematosus (SLE) patients. Methods Thirty-eight patients admitted during Jan 2001 to Aug 2008 were diagnosed as SLE associated with PHT and were analyzed retrospectively. Results All cases were female with the mean age of 35.7 yds. The mean period from PHT to the diagnosis of SLE was 2.3 years. Three cases were severe PHT with remarkable heart failure. Twenty-seven eases demonstrated Raynaund's phenomenon. Antinuclear antibody (ANA) was positive in all 38 cases and the titer was 1:320. Anti-DNA antibody, anti-Sin antibody, anti-U1RNP antibody and anti-SSA, anti-SSB were positive in 21 cases, 16 cases, 15 cases, 8 cases respectively. Antiphospholipid antibody and rheumatoid factor (RF) were elevated in 4 cases and 13 cases respectively. Seven cases were involved in lung fibrosis and pulmonary function test showed mild to moderate restrictive abnormality in these 7 cases. After treated with the combination of steroid and immunosuppressive agents, one case died on the 10th day and 2 cases died in 2 to 3 years after the diagnosis was established. The other 35 cases stayed stable. Conclusion PHT is one of the severe complications of SLE and often accompanied by poor outcome. Therefore early diagnosis and early combined therapy is very important.