Reports and analyses of 11 cases of connective tissue diseases related pulmonary arterial hypertension treated by mycophenolate mofetil / 中华风湿病学杂志

ABSTRACT

Objective To explore the efficacy of mycophenolate mofetil (MMF), which is a kind of immuno-suppressant drugs, on the treatment of Connective tissue diseases-pulmonary arterial hypertension (CTD-PAH). Methods Medical charts of eleven cases of hospitalized patients who were diagnosed as CTD-PAH and treated by MMF in Beijing Chaoyang Hospital affiliated to the Capital Medical University, from January 2014 to June 2016 were collected and analyzed. Results In the 11 cases of CTD-PAH, the systemic lupus erythematosus (SLE) related pulmonary hypertension (SLE-PAH) were 7, while the systemic sclero-derma associated pulmonary hypertension (SSc-PAH) were 2, and rheumatoid arthritis related pulmonary hy-pertension (RA-PAH) was 1, and the mixed connective tissue disease related pulmonary hypertension (MCTD-PAH) was 1. All patients were women, and the average age was (40 ±14) years, and the average duration of PAH was (34 ±35) months. The combination therapy of corticosteriods and MMF was applied to 7 cases, meanwhile the therapy of corticosteriods, MMF and bosentan was used in 1 case, corticosteriods, MMF and sildenafil was prescribed for 3 cases, and symptoms of the patients alleviated. Except for one case having been followed up for 7 months and one for 6 months, 9 patients completed the 1-year follow-up, and the survival rate was 100%(9/9). Notably, one patient, who had been alleviated for 111 months with therapy of corticosteriods and MMF, adopted the combination therapy of corticosteriods, MMF and bosentan for aggravated chest distress, and became stable eventually. Conclusion MMF may have therapeutic effects on inducing and even maintaining the stabilization of CTD-PAH.