Background/Purpose: Eosinophilic granulomatosis with polyangiitis (EGPA) cardiomyopathy carries a poor prognosis, and is the main cause of 1st-year and long-term mortality. Morbidity due to chronic cardiac insufficiency is also a major concern. The need for complementary investigations, which may detect cardiac involvement earlier, is crucial to effectively treat these patients early, before the advent of more severe clinical manifestations and cardiac damage. Cardiac magnetic resonance imaging (CMRI) seems to be a sensitive tool to detect EGPA cardiac lesions (defined as late myocardial gadolinium enhancement) in symptomatic but also asymptomatic patients, thereby raising the questions of CMRI specificity and the type(s) of anomalies (active inflammatory and/or fixed fibrotic) detected. This study was undertaken to determine retrospectively the diagnostic and prognostic impacts of such lesions in a cohort of EGPA patients with or without signs of cardiomyopathy who had undergone systematic CMRI screening.
Methods: This retrospective analysis concerned a monocentric cohort of 42 EGPA patients: 25/42 male (59.5%); mean age at diagnosis: 46.5 years; 11/42 (26.2%) ANCA-positive, with a median EGPA duration of 0.42 years before the 1st CMRI screening.
Results: Among the 42 patients, 17 (40.5%), 15 (88.2%) of whom were ANCA-negative, were diagnosed with cardiomyopathy, independently of CMRI findings. CMRI revealed late myocardial gadolinium enhancement in 14/17 (82.4%) patients with cardiomyopathy vs. 11/25 (44%) without cardiomyopathy (p=0.024). Using the sole criterion of late myocardial gadolinium enhancement, CMRI sensitivity and specificity for diagnosing cardiomyopathy were, respectively, 82.4% (95% CI: 0.59–0.93) and 56% (95% CI: 0.37–0.73). Among patients with cardiomyopathy who underwent additional CMRI during follow-up (median follow-up: 4.6 years), 6/14 patients’ CMRI-detected cardiac lesions had regressed under maintenance therapy, while those of 8/14 patients worsened or stabilized, despite treatment. No differences were found between non-cardiomyopathic patients with or without CMRI anomalies concerning subsequent EGPA cardiac manifestations and outcomes.
Conclusion: CMRI is a sensitive but non-specific method for detecting potential cardiomyopathic EGPA lesions. The diagnostic significance of late myocardial gadolinium enhancement in EGPA patients remains uncertain and should not be the only criterion for cardiomyopathy diagnosis. Furthermore, for patients with no other signs of cardiomyopathy, the CMRI-detected anomalies do not seem to adversely affect prognosis or outcome. However, longer follow-up is needed to assess this apparent absence of impact of CMRI lesions on cardiac outcome in asymptomatic patients.
Disclosure:
B. Dunogué,
None;
P. Cohen,
None;
B. Terrier,
None;
J. Marmursztejn,
None;
D. Duboc,
None;
O. Vignaux,
None;
L. Guillevin,
None.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/impact-of-cardiac-magnetic-resonance-imaging-on-eosinophilic-granulomatosis-with-polyangiitis-outcomes-a-long-term-retrospective-study-on-42-patients/