title: Clinical features and outcomes of posterior reversible encephalopathy syndrome in patients with systemic lupus erythematosus.
abstract: OBJECTIVE
  To analyze the clinical features and outcomes of patients with posterior reversible encephalopathy syndrome(PRES), the risk factors of PRES-related intracranial hemorrhage (ICH), and all-cause mortality in patients with systemic lupus erythematosus (SLE).
  
  
  METHODS
  Twenty-six episodes of PRES were identified in 23 SLE patients, using an electronic medical records database of 3,746 SLE patients.
  
  
  RESULTS
  The prevalence of PRES was 0.69% among SLE patients. The scores of the SLE Disease Activity Index without neurologic descriptors (SLEDAI-N) were significantly elevated from baseline for a mean of 3.3 during PRES (P = 0.009). Rapidly deteriorating renal function, pulmonary hemorrhage, thrombotic microangiopathy, macrophage activation syndrome, or multiple organ dysfunction syndrome appeared during 65.4% of episodes. In 16 episodes, patients completely recovered from PRES-related symptoms within a median of 7 days. Visual impairment was reversed within 2 days in 8 of 15 patients, but impairment in other patients was protracted for up to 4 months, especially when ICH was present. Hypoalbuminemia (<20 gm/liter; odds ratio [OR] 30, 95% confidence interval [95% CI] 2.04–441.84) and thrombocytopenia (<30,000/mm(3); OR 21, 95% CI 1.27–346.93) were risk factors for PRES-related ICH. Patients with SLEDAI-N scores >18 during a PRES attack had significantly higher mortality rates than did patients with SLEDAI-N scores ≤18 (P = 0.009 by log rank test).
  
  
  CONCLUSIONS
  PRES frequently occurs during active SLE with multiple complications. Hypoalbuminemia and thrombocytopenia may contribute to PRES-related ICH. The extra neurologic disease activity of lupus during PRES may influence the mortality rate of SLE patients.
authors.forename: Chien-Chih
authors.initials: CC
authors.lastname: Lai
authors.forename: Wei-Sheng
authors.initials: WS
authors.lastname: Chen
authors.forename: Yu-Sheng
authors.initials: YS
authors.lastname: Chang
authors.forename: Shu-Hung
authors.initials: SH
authors.lastname: Wang
authors.forename: Chun-Jui
authors.initials: CJ
authors.lastname: Huang
authors.forename: Wan-Yuo
authors.initials: WY
authors.lastname: Guo
authors.forename: Wu-Chang
authors.initials: WC
authors.lastname: Yang
authors.forename: De-Feng
authors.initials: DF
authors.lastname: Huang
mesh_terms: D000293:Adolescent; D000328:Adult; D002423:Cause of Death / Q000639:trends; D002648:Child; D003952:Diagnostic Imaging / Q000379:methods*; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000401:mortality; D008297:Male; D008875:Middle Aged; D054038:Posterior Leukoencephalopathy Syndrome / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology*; D015995:Prevalence; D011379:Prognosis; D012189:Retrospective Studies; D012307:Risk Factors; D015996:Survival Rate / Q000639:trends; D013624:Taiwan / Q000453:epidemiology; D055815:Young Adult