title: Severe pulmonary arterial hypertension secondary to lupus in the emergency department: proactive intense care associated with a better short-term survival.
abstract: 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.
authors.affiliation: Emergency Department, Renji Hospital, Shanghai JiaoTong University School of Medicine, Shanghai, China.
authors.forename: Yi
authors.initials: Y
authors.lastname: Chen
authors.forename: Li
authors.initials: L
authors.lastname: Guo
authors.forename: Yuzhen
authors.initials: Y
authors.lastname: Li
authors.forename: Guang-Liang
authors.initials: GL
authors.lastname: Chen
authors.forename: Xiao-Xiang
authors.initials: XX
authors.lastname: Chen
authors.forename: Shuang
authors.initials: S
authors.lastname: Ye
mesh_terms: D000328:Adult; D000959:Antihypertensive Agents / Q000627:therapeutic use*; D016022:Case-Control Studies; D003422:Critical Care; D004359:Drug Therapy, Combination; D004502:Education, Medical, Continuing; D004636:Emergency Service, Hospital; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology / Q000401:mortality; D007318:Inservice Training; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000175:diagnosis / Q000401:mortality; D008297:Male; D011446:Prospective Studies; D012720:Severity of Illness Index; D016019:Survival Analysis; D013997:Time Factors; D016896:Treatment Outcome; D014218:Triage
chemical_list: D000959:Antihypertensive Agents
keywords: PAH-targeted combination therapy; pulmonary arterial hypertension; systemic lupus erythematosus