Cardiac Involvement in ANCA (+) and ANCA (-) Churg-Strauss Syndrome Evaluated by Cardiovascular Magnetic Resonance

Abstract

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.

Prevalence and prognostic relevance of cardiac involvement in ANCA-associated vasculitis: Eosinophilic granulomatosis with polyangiitis and granulomatosis with polyangiitis

“…Moreover, previous cross-sectional studies predominantly used ECG and/or echocardiography only [5][6][7][8][10][11][12][13], whereas studies using cardiac magnetic resonance imaging (CMR) and invasive methods are scarce and the number of patients included low [9,14,15]. To date, two longitudinal studies suggested myocardial fibrosis was associated with adverse outcome in EGPA patients, but the small and descriptive nature prohibits any definitive conclusions [14,18]. Thus, there is lack of prospective studies with contemporary noninvasive and invasive imaging to define the true prevalence of cardiac involvement in EGPA and GPA and link this involvement to long-term prognosis.…”

The Critical Role of Cardiac Magnetic Resonance Imaging in Evaluating Patients With Eosinophilic Granulomatosis With Polyangiitis

“…The diagnosis of EGPA itself has varying challenges, one of which is the fact that a patient with cardiac involvement may present with anti-neutrophil cytoplasmic antibody (ANCA) positivity less frequently than a patient without cardiac involvement [16]. The current use of ECG for the detection of cardiovascular involvement is limited to evaluating left ventricular (LV) wall motion and functional changes [20]. The shortcomings of ECG include the inability to identify tissue damage and the detection of other types of damage before there are apparent LV abnormalities [20].…”

“…The current use of ECG for the detection of cardiovascular involvement is limited to evaluating left ventricular (LV) wall motion and functional changes [20]. The shortcomings of ECG include the inability to identify tissue damage and the detection of other types of damage before there are apparent LV abnormalities [20]. The absence of chest pain and ECG abnormalities can lead to a false sense of security, even in patients with increased troponin levels.…”

“…The EGPA Consensus Task Force has stated that the use of advanced imaging techniques showed more sensitivity in detecting cardiac involvement [24]. ECG has reliably shown its ability to detect the presence of heart failure in EGPA, with its main limitation being that it shows damage after it has already been inflicted [20]. CMR has consistently outperformed ECG in the early detection of cardiovascular system involvement in EGPA [25,26].…”

Ventricular Tachycardia and Sudden Cardiac Death in Connective Tissue Diseases: Can Cardiovascular Magnetic Resonance Play a Role?

“…The amount of fibrotic tissue, as assessed by LGE, is an independent predictor for future cardiac events [35].…”