title: Cardiac involvement in ANCA (+) and ANCA (-) Churg-Strauss syndrome evaluated by cardiovascular magnetic resonance.
abstract: BACKGROUND
  The cardiovascular magnetic resonance (CMR) pattern of Churg-Strauss syndrome (CSS) includes myopericarditis, diffuse subendocardial vasculitis or myocardial infarction with or without cardiac symptoms and is usually associated with lack of antineutrophil cytoplasmic antibodies (ANCA).
  
  
  OBJECTIVE
  To correlate the CMR pattern with ANCA in CSS, compare it with healthy controls and systemic lupus erythematosus (SLE) patients and re-evaluate 2 yrs after the first CMR.
  
  
  METHODS
  28 consecutive CSS, aged 42±7 yrs, were referred for CMR and 2 yrs re-evaluation. The CMR included left ventricular ejection fraction (LVEF), T2-weighted (T2-W), early (EGE) and late gadolinium enhanced (LGE) imaging. Their results were compared with 28 systemic lupus erythematosus (SLE) under remission and 28 controls with normal myocardial perfusion, assessed by scintigraphy.
  
  
  RESULTS
  CMR revealed acute cardiac lesions in all ANCA (-) CSS with active disease and acute cardiac symptoms and only in one asymptomatic ANCA (+) CSS, with active disease. Diffuse subendocardial fibrosis (DSF) or past myocarditis was identified in both ANCA(+) and ANCA (-) CSS, but with higher incidence and fibrosis amount in ANCA (-) CSS (p<0.05). In comparison to SLE, both ANCA (+) and ANCA (-) CSS had higher incidence of DSF, lower incidence of myocarditis and no evidence of myocardial infarction, due to coronary artery disease (p<0.05). In 2 yrs CMR follow up, 1/3 of CSS with DSF presented LV function deterioration and one died, although immunosuppressive treatment was given early after CSS diagnosis.
  
  
  CONCLUSIONS
  Cardiac involvement either as DSF or myocarditis, can be detected in both ANCA (+) and ANCA (-) CSS, although more clinically overt in ANCA (-). DSF carries an ominous prognosis for LV function. CMR, due to its capability to detect disease severity, before cardiac dysfunction takes place, is an excellent tool for CSS risk stratification and treatment individualization.
authors.affiliation: 50 Esperou Street, 175-61 P. Faliro, Athens, Greece. soma13@otenet.gr.
authors.forename: Sophie
authors.initials: S
authors.lastname: Mavrogeni
authors.forename: Georgia
authors.initials: G
authors.lastname: Karabela
authors.forename: Elias
authors.initials: E
authors.lastname: Gialafos
authors.forename: Efthymios
authors.initials: E
authors.lastname: Stavropoulos
authors.forename: George
authors.initials: G
authors.lastname: Spiliotis
authors.forename: Gikas
authors.initials: G
authors.lastname: Katsifis
authors.forename: Genovefa
authors.initials: G
authors.lastname: Kolovou
mesh_terms: D000328:Adult; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000378:metabolism; D015267:Churg-Strauss Syndrome / Q000150:complications / Q000175:diagnosis*; D003331:Coronary Vessels / Q000000981:diagnostic imaging / Q000473:pathology; D004562:Electrocardiography; D004719:Endomyocardial Fibrosis / Q000209:etiology; D005260:Female; D006321:Heart / Q000000981:diagnostic imaging*; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications / Q000175:diagnosis*; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009205:Myocarditis / Q000209:etiology; D009206:Myocardium / Q000276:immunology / Q000473:pathology*; D011379:Prognosis; D011877:Radionuclide Imaging; D012306:Risk; D016277:Ventricular Function, Left
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic