Cardiac involvement in granulomatosis with polyangiitis: a magnetic resonance imaging study of 31 consecutive patients

• Published in: Rheumatology (Oxford, England) Vol. 56; no. 6; pp. 947 - 956
• Main Authors: Pugnet, Grégory, Gouya, Hervé, Puéchal, Xavier, Terrier, Benjamin, Kahan, André, Legmann, Paul, Guillevin, Loïc, Vignaux, Olivier
• Format: Journal Article
• Language: English
• Published: England 01-06-2017
• Subjects: Adult; Aged; Aged, 80 and over; Cardiomyopathies - diagnosis; Cross-Sectional Studies; Echocardiography; Female; Granulomatosis with Polyangiitis - diagnosis; Humans; Magnetic Resonance Angiography; Magnetic Resonance Imaging, Cine; Male; Middle Aged; Pericarditis - diagnosis; Recurrence; Retrospective Studies; Risk Factors; Ventricular Dysfunction, Left - diagnosis; Young Adult; cardiac manifestations; granulomatosis with polyangiitis; cardiac magnetic resonance imaging; ANCA-associated vasculitis
• ISSN: 1462-0324; 1462-0332
• DOI: 10.1093/rheumatology/kew490

Specific cardiac involvement in granulomatosis with polyangiitis (GPA) is probably underestimated since many of these conditions are subclinical. The objective of this study was to assess the prevalence and patterns of cardiac abnormalities detected by cardiac MRI (CMRI) in patients with GPA.
Thirty-one consecutive patients with newly diagnosed or relapsing GPA underwent CMRI to assess morphological, functional, perfusion at rest and delayed enhancement abnormalities.
At least one abnormality was observed on CMRI for 19 of 31 patients (61%). Four patients (13%) had an impaired left ventricle ejection fraction (LVEF). LV regional wall motion abnormalities were found in 11 patients (35%). Late gadolinium enhancement (LGE) was detected in 10 of 31 patients (32%). LGE was mostly nodular ( n = 9). Myocardial early contrast enhancement was detected in 5 of the 31 patients (16%), which was systematically associated with LGE in the same territory. CMRI detected pericarditis in eight patients (26%). GPA with <18 months duration was associated with a higher LVEF ( P = 0.03), fewer CMRI abnormalities ( P = 0.04) and less LV hypokinesia ( P = 0.04) than GPA with a longer duration. Patients with recent-onset GPA had a higher LVEF ( P = 0.01) and less LV hypokinesia ( P = 0.006) than patients experiencing a relapse ( P = 0.02).
CMR is an accurate technique for detecting heart involvement in GPA. This unique non-invasive technique may provide information with important clinical implications for the accurate early assessment of cardiac lesions in GPA patients and for detecting cumulative, irreversible damage. It may also have prognostic implications.