Title:Cardiac Involvement in ANCA (+) and ANCA (-) Churg-Strauss Syndrome Evaluated by Cardiovascular Magnetic Resonance
Volume: 12
Issue: 5
Author(s): Sophie Mavrogeni, Georgia Karabela, Elias Gialafos, Efthymios Stavropoulos, George Spiliotis, Gikas Katsifis and Genovefa Kolovou
Affiliation:
Keywords:
ANCA, cardiac magnetic resonance, CSS, heart disease, SLE.
Abstract: Introduction: 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).
Aim: 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.
Patients-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.
