Clinical analysis of 38 systemic lupus erythematosus patients complicated with pulmonary hypertension / 中华风湿病学杂志

ABSTRACT

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 1320. 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.