title: Cardiac involvement in Churg-Strauss syndrome.
abstract: OBJECTIVE
  Churg-Strauss syndrome (CSS) is a rare form of systemic vasculitis. Previous studies showing cardiac involvement in CSS patients were limited in the number of patients and were often based solely on clinical manifestations. The aim of the present study was to determine in detail the incidence of cardiac involvement in a large population of ambulatory CSS patients.
  
  
  METHODS
  Thirty-two consecutive patients with CSS in remission (mean +/- SD duration of disease between diagnosis and enrollment 6.1 +/- 5.8 years, mean +/- SD age 61 +/- 10 years) who were previously unaware of cardiac involvement were compared with 32 randomly selected age- and sex-matched control subjects, using clinical evaluation, electrocardiography (EKG), echocardiography, and cardiac magnetic resonance imaging (MRI).
  
  
  RESULTS
  Detailed cardiac evaluation revealed a 62% prevalence of cardiac involvement in CSS patients compared with 3% in controls (P < 0.001), with clinical symptoms in 26% and 3%, respectively (P = 0.009), EKG abnormalities in 66% and 3%, respectively (P < 0.001), and echocardiographic defects in 50% and 3%, respectively (P < 0.001). Cardiac MRI detected cardiac manifestations in 62% of CSS patients. In the presence of cardiac MRI abnormalities, echocardiography could detect cardiac involvement with a sensitivity of 83% and a specificity of 80%. The absence of symptoms or EKG abnormalities did not exclude cardiac involvement, because abnormalities could still be detected in 38% of these patients at the time of echocardiography or cardiac MRI.
  
  
  CONCLUSIONS
  These results demonstrate a high incidence of cardiac involvement in CSS patients. Systematic cardiac evaluation including detailed imaging is required to properly identify CSS patients with cardiac involvement.
authors.affiliation: Maastricht University, Maastricht, The Netherlands.
authors.forename: Robert M
authors.initials: RM
authors.lastname: Dennert
authors.forename: Pieter
authors.initials: P
authors.lastname: van Paassen
authors.forename: Simon
authors.initials: S
authors.lastname: Schalla
authors.forename: Tatiana
authors.initials: T
authors.lastname: Kuznetsova
authors.forename: Becker S
authors.initials: BS
authors.lastname: Alzand
authors.forename: Jan A
authors.initials: JA
authors.lastname: Staessen
authors.forename: Sebastiaan
authors.initials: S
authors.lastname: Velthuis
authors.forename: Harry J
authors.initials: HJ
authors.lastname: Crijns
authors.forename: Jan Willem Cohen
authors.initials: JW
authors.lastname: Tervaert
authors.forename: Stephane
authors.initials: S
authors.lastname: Heymans
mesh_terms: D000368:Aged; D015267:Churg-Strauss Syndrome / Q000453:epidemiology*; D003920:Diabetes Mellitus / Q000453:epidemiology; D004452:Echocardiography; D004562:Electrocardiography; D005260:Female; D006331:Heart Diseases / Q000175:diagnosis* / Q000453:epidemiology*; D006801:Humans; D015994:Incidence; D008137:Longitudinal Studies; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D015995:Prevalence