Clinical analysis of therapy of 46 cases systemic lupus erythematosus associated with pulmonary arterial hypertension / 中国综合临床

ABSTRACT

Objective To study the theraputic effect of cyclophosphamide,glucocorticoid combined with sildenafil on pulmonary hypertension in systemic lupus erythematosus(SLE?PAH) patients,and to analyze the prognosis adverse factors. Methods Forty?six cases patients with SLE?PAH from Tangshan Gongren Hospital from January 2009 to January 2015 were selected as research subjects. All patients were treated with prednisone, cyclophosphamide and sildenafil,and the course of treatment was 24 weeks. The treatment effect was observed, the systemic lupus erythematosus disease activity score(SLEDAI),6 minutes walk distance and heart function before and after the treatment were compared. The effective and uneffective patients'clinical and test information were compared,and the prognosis adverse factors were analyzed. Results After 24 weeks treatment,the total ef?fective rate was 78. 3%(36/46). Pulmonary artery systolic pressure(PASP) dropped from (59±16) mmHg be?fore treatment to (45±15) mmHg after treatment,there was significant statistical difference(t=-4. 539,P<0. 01). SLEDAI dropped from (6. 6±2. 3) points to (2. 4±0. 8) points,,there was significant statistical differ?ence(t =11. 680,P<0. 01). The 6 minutes walk distance rose form (402±96) minutes to (465±97) minutes, there was significant statistical difference( t=-3. 137,P<0. 01) . Cardiac function improved significantly,there was significant statistical difference( P<0. 01) . There were 10 cases uneffective and deterirated patients,with the age of (42±6) years,36 cases effective patients with the age of (33±4) years,the difference was statistically significant( t=-5. 423, P<0. 01 ) . Uneffective and deterirated patients' anti?RNP antibody positive rate was 90. 9%( 9/10 ) , significantly higher than the effective patients ( 52. 8%( 19/36 ) ,χ2 = 4. 552, P<0. 01 ) . Conclusion Cyclophosphamide, glucocorticoid combined with sildenafil has obvious effect on patients with SLE?PAH. PASP,heart function and 6 minutes walk distance are the important indicators of prognosis. Anti?RNP antibody and age are the adverse prognostic factors.