Cardiac involvement in Wegener's granulomatosis resistant to induction therapy

Abstract

Objectives: The aim of the study was to assess cardiac involvement in patients with Wegener's granulomatosis (WG), who failed to achieve remission following >6 months induction therapy for life or organ threatening disease.

Methods: Eleven WG patients (eight males, mean age 47 ± 13 years), who failed to achieve remission despite >6 months induction therapy, underwent transthoracic echocardiography (TTE) and cardiac magnetic resonance (CMR).

Results: Cardiac involvement was present in 9 (82%) patients. Regional wall motion abnormalities were found in two individuals, but none had left ventricular (LV) ejection fraction <50%. Nine patients had late gadolinium enhancement (LGE) lesions involving LV myocardium and right ventricle free wall was involved in four patients. LGE lesions were found in subepicardial, midwall and subendocardial LV myocardial layers. CMR revealed myocarditis in six patients. Patients with myocarditis had a higher number of LV segments with LGE (5.2 ± 3.4 vs 1.0 ± 1.2, p = 0.03) and more frequent diastolic dysfunction by TTE (5 vs 0, p = 0.02) than those without. Pericardial effusion was observed in five patients, while localized pericardial thickening in six patients.

Conclusions: In WG resistant to >6 months induction therapy cardiac involvement is frequent and is characterized by foci of LGE lesions and signs of myocardial inflammatory process.

Fig. 1

Late gadolinium enhancement (LGE) image (a) in four-chamber apical view as well as TSE T2-weighted (b), pre- (c) and post-contrast TSE T1-weighted images (d) in short axis view in 58-years old male with Wegener’s granulomatosis. LGE image shows subendocardial fibrosis in left and right ventricle (arrows) (a). Mid segments demonstrate increased T2 signal intensity (arrows) on TSE T2-weighted image (b) suggesting myocardial edema and early gadolinium enhancement (arrows) on post-contrast TSE T1-weighted image (d) reflecting increased myocardial hyperemia/capillary leak. Cardiac magnetic resonance revealed preserved global left ventricular systolic function (EF = 56%) with regional wall motion abnormalities (akinesis of the apex, hypokinesis of apical anterior, lateral, septal and mid anteroseptal segments)