Severe pulmonary arterial hypertension secondary to lupus in the emergency department: proactive intense care associated with a better short-term survival.

Objective: Pulmonary arterial hypertension (PAH) is a severe complication of systemic lupus erythematosus (SLE) and could be an acute critical condition presenting to the emergency department (ED). Our previous retrospective study revealed that the ED-related mortality of such patients was over 50%. The aim of the current prospective study is to initiate a proactive intense care strategy on severe SLE-PAH patients in the emergency setting and evaluate its impact on the short-term survival.

Methods: The proactive intense care strategy was applied, which includes: (i) an education and training course on the topic of SLE-PAH for ED physicians; (ii) a SLE-PAH patient triage protocol with prompt specialist consultation and admission; and (iii) intensive care with prompt initiation of combination PAH-targeted therapy, that is, at least two drugs from the three categories as represented by iloprost, bosentan and sildenafil. Consecutive SLE-PAH patients with WHO functional class III or IV who attended the ED were enrolled following the aforementioned protocol. A historical group of SLE-PAH patients in the ED (n = 11) was set up as a comparison, and 3-month short-term survival was calculated.

Results: During October 2010 to December 2012, a total of 11 consecutive severe SLE-PAH patients were included in the present study. Compared with the historical group, an improved short-term survival can be appreciated over time (historical group vs. proactive group, 27.3% vs. 72.7%, P = 0.033). The application of PAH-targeted combination therapy apparently contributed to the better outcome (P = 0.0099).

Conclusions: Proactive care and combination PAH-targeted treatment can improve short-term survival of severe SLE-PAH in the emergency setting.