Clinical features and management of pulmonary hypertension secondary to systemic lupus erythematosus / 中华风湿病学杂志

ABSTRACT

Objective To investigate the incidence,pathogenesis,clinical features,principle in treatment and prognosis of pulmonary hypertension (PHT) secondary to systemic lupus erythematosus (SLE).Methods The clinical features,management and outcome of 6 patients with PHT secondary to SLE were reported and literature was reviewed.Results Six cases of PHT related to SLE,mean age 39 5 year,mean period of SLE 3 2 year,mean pulmonary artery pressure 61 8 mm Hg,3 were severe PHT.Organ involvements were seen in all cases6 with cardial involvement,of which 2 with heart failure;5 with renal impairment;3 with pulmonary lesions other than PHT,2 with hematological involvement;2 with thrombosis and 2 with Raynaund′s phenomenon (RP).Remarkable decrease of complement C3 was seen in all cases,anti Scl 70 and antiphospholipid antibody (ACL) positive were seen in 2 cases as well.No neurological involvement was found.After early comprehensive and intensive treatment,normalized PHT was seen in all but one case,concomitant with decrease in SLE activity.After 9 months′ follow up,1 case died from dysrhythmia,the rest kept stable,except RP.Conclusion PHT is a severe complication of SLE,often accompanied by multi organ involvement and poor outcome;Intensive and comprehensive treatment should be made early,especially for primary affection and its cause.Vasodialator (usually calcium channel blocker) and anticoagulant is a principle medication and prostacyclin should be considered for refractory cases.Better outcome can be achieved for those who are responsive to the treatment.