Systemic lupus erythematosus associated pulmonary arterial hypertension: results from 10-year inpatient cases analysis in a single center / 中华风湿病学杂志

ABSTRACT

Objective To investigate the clinical characteristics and current condition of treatment for systemic lupus erythematosus (SLE) associated pulmonary arterial hypertension (PAH).Methods 10-year inpatients cases were reviewed and followed up.Cases were divided into 2 groupsgroup Apatients with baseline pulmonary arterial systolic pressure (PASP) lower than 70 mmHg； group Bpatients with baseline PASP higher than 70 mm Hg.Pearson's correlation analysis,Chi-square test,Logistic regression,Cox-Mantel and Wilcoxon test were used for statistical analysis.Results There were totally 155 cases with 184 records of admission which accounted for 4.16％ among total lupus cases.The main clinical characteristics included Raynaud's phenomenon (47.3％),pericardial effusion (41.9％) and high titer of anti-RNP antibody (55.4％).There were 132 cases enrolled for prognostic statistical analysis.There were 47 cases of death (35.6％) in total,among which 9 cases (19.1％) were in group A and 38 cases (80.9％) were in group B.In group A,there was a positive correlation between PASP and lupus disease activity index score.Single therapy analysis by Chi-square test showed that cyclophosphamide (CTX) (P＜0.05) and PAH targeted drugs (P＜0.01) were significantly associated with favorable outcome but logistic regressive analysis only confirmed the efficacy of target drugs (P＜0.01).PAH target drugs significantly improved the one year survival rate of the severe cases.Conclusion The main clinical characteristics of SLE associated PAH include Raynand's phenomenon,pericardial effusion and positive anti-RNP antibody.The severity of PAH may not be related to lupus disease activity.PAH targeted drugs are effective in SLE-PAH.CTX may be effective in some cases.For severe cases,the combination therapy of CTX and PAH targeted drugs could significantly improve the prognosis.