[Systemic lupus erythematosus associated pulmonary arterial hypertension: clinical analysis of 91 cases].

OBJECTIVE

To explore the clinical characteristics and therapeutic efficacy of systemic lupus erythematosus (SLE) associated pulmonary arterial hypertension (PAH).

METHODS

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.

RESULTS

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.

CONCLUSION

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.