Background/Purpose Cardiac manifestations in granulomatosis with polyangiitis (GPA) patients are usually considered to be rare but may be life-threatening. However specific cardiac involvement in GPA is probably underestimated during life because many of these cardiac disturbances are subclinical. Contrast-enhanced cardiac magnetic resonance imaging (CMRI) may be a sensitive tool to detect and analyze cardiac involvement in ANCA associated vasculitis. The objective of our study was to assess the prevalence and patterns of cardiac abnormalities detected by cardiac magnetic resonance imaging in patients with GPA.
Methods
Thirty-one consecutive patients with either new or relapsing GPA underwent CMRI to determine morphological, functional, perfusion at rest and delayed enhancement abnormalities.
Results
At least one abnormality on CMRI was observed in 19/31 patients (61.3%). Four patients (14.8%) had an impaired left ventricle (LV) ejection fraction with evidence of clinically cardiac failure in two of them. LV kinetic abnormalities were found in 11 patients (36.7%). Myocardial delayed contrast enhancement (DCE) was detected in 9/31 patients (29%), 6 of whom with cardiac manifestations. DCE was mainly nodular (n = 7/9). Myocardial early contrast enhancement was detected in 5 of the 31 patients (16.1%) and was always associated with DCE in the same territory. CMRI detected pericarditis in 7 patients (22.6%). GPA of less than 18 months duration as compared with GPA of longer duration had greater LV ejection fraction (P=0.008) and less CMRI abnormalities (P=0.04), DCE (P=0.19) or LV hypokinesia (P=0.04). Patients who presented with new-onset GPA had less CMRI abnormalities than patients who experienced a relapse (P= 0.02).
Conclusion CMRI is an accurate technique for diagnosing heart involvement in GPA and for analyzing precisely its mechanisms including inflammatory, microvascular and fibrotic components. This unique noninvasive information may have important clinical implications in early and accurate assessment of cardiac lesions in GPA patients but also to detect cumulative non reversible damage. Moreover, it may have prognostic implications.
Disclosure:
G. Pugnet,
None;
X. Puéchal,
None;
B. Terrier,
None;
H. Gouya,
None;
A. Kahan,
None;
P. Legmann,
None;
L. Guillevin,
None;
O. Vignaux,
None.
ACR Meeting Abstracts - https://acrabstracts.org/abstract/cardiac-involvement-in-granulomatosis-with-polyangiitis-a-magnetic-resonance-imaging-study-of-31-consecutive-patients/