title: Evans syndrome and systemic lupus erythematosus: clinical presentation and outcome.
abstract: OBJECTIVE
  To review the clinical, laboratory and outcome features of Evans syndrome (ES) in systemic lupus erythematosus (SLE) patients.
  
  
  METHODS
  We reviewed the charts of 953 SLE patients followed up regularly at our service. ES was defined as the presence of hemolytic anemia and thrombocytopenia concomitantly or sequentially. Clinical and laboratory manifestations occurring during the disease course, as well as concomitant diseases and survival was carefully reviewed.
  
  
  RESULTS
  We identified ES in 26 of 953 (2.7%) SLE patients. Twenty-three were women with mean age at SLE diagnosis of 25.7 years. Four (15%) patients had disease onset before the age of 16. In the majority of patients (92%), immune thrombocytopenia and AIHA appeared simultaneously at the beginning of SLE. Active features of SLE were a frequent finding concomitant to ES, especially arthritis (77%), malar rash (61.5%), photosensitivity (57.6%), oral ulcers (34.6%), nephritis (73%), serositis (54%), neuropsychiatric (19%) and pulmonary (15%) manifestations. In addition to this multisystemic disease, 34.6% of our patients had an association with another autoimmune disease such as antiphospholipid syndrome. Recurrence of ES was observed in only four (15%) patients. After follow-up time of 8.72 years, 19 patients (73%) were in remission and seven (27%) patients died.
  
  
  CONCLUSIONS
  ES is a rare manifestation in SLE, occurring in patients with severe multisystemic SLE manifestations. Treatment strategies frequently used in SLE contribute to longer disease remission and less frequent exacerbation than observed in the general population with ES.
authors.affiliation: Medical School of Sorocaba, Pontifical Catholic University of São Paulo, Brazil.
authors.forename: Guilherme Lavras
authors.initials: GL
authors.lastname: Costallat
authors.forename: Simone
authors.initials: S
authors.lastname: Appenzeller
authors.forename: Lilian Tereza Lavras
authors.initials: LT
authors.lastname: Costallat
mesh_terms: D000293:Adolescent; D000328:Adult; D000744:Anemia, Hemolytic, Autoimmune / Q000150:complications / Q000175:diagnosis* / Q000453:epidemiology*; D016736:Antiphospholipid Syndrome / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology; D001938:Brazil / Q000453:epidemiology; D015897:Comorbidity; D005260:Female; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications / Q000175:diagnosis* / Q000453:epidemiology*; D008297:Male; D008875:Middle Aged; D012008:Recurrence; D015996:Survival Rate; D013921:Thrombocytopenia / Q000150:complications / Q000175:diagnosis* / Q000453:epidemiology*; D055815:Young Adult