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.