title: Thrombotic microangiopathy in systemic lupus erythematosus: a cohort study in North Taiwan.
abstract: OBJECTIVE
  Thrombotic microangiopathy (TMA) co-existing with SLE is rarely reported. This study aimed to investigate the triggering factors, clinical features and outcomes of SLE patients with TMA in Northern Taiwan.
  
  
  METHODS
  Twenty-five TMA cases out of 2461 SLE patients admitted to Taipei Veterans General Hospital, between 2000 and 2010, were enrolled.
  
  
  RESULTS
  When TMA occurred, 16 (64.0%) patients had infection; 22 (88.0%) were in an active disease state with a SLEDAI score >10. Among the infection group, 13 (81.3%) had an increase in the SLEDAI score of ≥ 4. We found that older age (≥ 50 years), low platelets (≤ 20,000/nm(3)), presence of infection, acute renal failure (ARF) or four or more TMA features were independent risk factors for persistent haematological abnormalities (P < 0.05); older age (≥ 50 years) and a high reticulocyte index (>2%) were the risk factors for persistent renal function impairment (P < 0.05). The overall mortality rate was 52.0% (13 out of 25); older age (≥ 40 years), low complement value, presence of infection (P < 0.001), two or more infection sources, ARF and four or more TMA features were the statistically significant factors contributing to a higher mortality rate. Patients receiving plasma exchange seven times or more had a significantly higher rate of improvement in renal function and haematological abnormalities.
  
  
  CONCLUSIONS
  Our study showed that infection was one of the major triggers for the flare-up of SLE disease activity and occurrence of TMA in SLE. Infection is also a strong risk factor for outcome in SLE patients with TMA. Plasma exchange can be considered as an adjuvant treatment modality.
authors.affiliation: Department of Medicine, Taipei Veterans General Hospital, Taipei 11217, Taiwan.
authors.forename: Ming-Han
authors.initials: MH
authors.lastname: Chen
authors.forename: Ming-Huang
authors.initials: MH
authors.lastname: Chen
authors.forename: Wei-Sheng
authors.initials: WS
authors.lastname: Chen
authors.forename: Peter
authors.initials: P
authors.lastname: Mu-Hsin Chang
authors.forename: Hui-Ting
authors.initials: HT
authors.lastname: Lee
authors.forename: Hsiao-Yi
authors.initials: HY
authors.lastname: Lin
authors.forename: De-Feng
authors.initials: DF
authors.lastname: Huang
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D015331:Cohort Studies; D015897:Comorbidity; D005260:Female; D006801:Humans; D007239:Infections / Q000150:complications; D008180:Lupus Erythematosus, Systemic / Q000453:epidemiology* / Q000208:ethnology* / Q000628:therapy; D008297:Male; D008875:Middle Aged; D010951:Plasma Exchange; D012189:Retrospective Studies; D012307:Risk Factors; D013624:Taiwan; D057049:Thrombotic Microangiopathies / Q000453:epidemiology* / Q000208:ethnology* / Q000628:therapy; D055815:Young Adult