title: Clinical and laboratory characteristics of 19 patients with Churg-Strauss syndrome from a single South Australian centre.
abstract: BACKGROUND
  Churg-Strauss syndrome (CSS) is a rare, idiopathic systemic vasculitis. There is emerging evidence of an association between the presence or absence of antineutrophil cytoplasmic antibodies (ANCA) and clinical phenotype. Thromboembolism is an increasingly recognised complication of the disease.
  
  
  OBJECTIVE
  Given the paucity of Australian data, the aim of this study was to examine the clinical and laboratory features of CSS in a single Australian centre.
  
  
  METHODS
  We performed a retrospective review of all patients who fulfilled the American College of Rheumatology classification criteria for CSS managed at the Department of Immunology, Royal Adelaide Hospital between 2002 and 2008.
  
  
  RESULTS
  Nineteen patients were included. All patients had asthma and most had upper airway involvement. Peripheral nerve, musculoskeletal, gastrointestinal and cutaneous involvement was common. Renal and cardiac involvement was uncommon in this series. Histological confirmation was obtained in 15 patients (78.9%). Ten patients (52.6%) were ANCA+, and these were more likely to have musculoskeletal involvement, such as arthralgia or myalgia (odds ratio 57, P = 0.005). Thrombosis was a feature at diagnosis in six patients (31.6%); two of these recurred with relapse. Sixteen patients (84.2%) were followed up; five died, and mean survival was 8.9 years.
  
  
  CONCLUSIONS
  This is the first Australian study to focus on CSS. Our results demonstrate similar presentation and prognosis of CSS to previous descriptions; however, we noted that musculoskeletal involvement was more common in ANCA+ patients. In our series, thrombosis was a significant complication and we suggest that thromboprophylaxis may be warranted.
authors.affiliation: Human Immunology, SA Pathology/IMVS, Adelaide, South Australia, Australia. andrew.whyte@health.sa.gov.au
authors.forename: A F
authors.initials: AF
authors.lastname: Whyte
authors.forename: W B
authors.initials: WB
authors.lastname: Smith
authors.forename: S N
authors.initials: SN
authors.lastname: Sinkar
authors.forename: F E
authors.initials: FE
authors.lastname: Kette
authors.forename: P
authors.initials: P
authors.lastname: Hissaria
mesh_terms: D000328:Adult; D000368:Aged; D015267:Churg-Strauss Syndrome / Q000175:diagnosis* / Q000453:epidemiology* / Q000503:physiopathology; D005260:Female; D005500:Follow-Up Studies; D006760:Hospitalization; D006801:Humans; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D013021:South Australia / Q000453:epidemiology
keywords: Churg-Strauss syndrome; antineutrophil cytoplasmic antibody; epidemiology; thrombosis; vasculitis