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

• Published in: International journal of cardiology Vol. 199; pp. 170 - 179
• Main Authors: Hazebroek, M.R., Kemna, M.J., Schalla, S., Sanders-van Wijk, S., Gerretsen, S.C., Dennert, R., Merken, J., Kuznetsova, T., Staessen, J.A., Brunner-La Rocca, H.P., van Paassen, P., Cohen Tervaert, J.W., Heymans, S.
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier Ireland Ltd 15.11.2015
• Subjects: Aged; Aged, 80 and over; Algorithms; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis - complications; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis - drug therapy; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis - epidemiology; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis - immunology; Biopsy; Cardiac Imaging Techniques - methods; Cardiovascular; Cardiovascular disease; Cardiovascular Diseases - complications; Cardiovascular Diseases - mortality; Churg-Strauss Syndrome - complications; Churg-Strauss Syndrome - drug therapy; Churg-Strauss Syndrome - epidemiology; Cohort Studies; Coronary Angiography; Echocardiography; Electrocardiography; Female; Granulomatosis with polyangiitis; Granulomatosis with Polyangiitis - complications; Granulomatosis with Polyangiitis - drug therapy; Granulomatosis with Polyangiitis - epidemiology; Granulomatosis with Polyangiitis - immunology; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Myocardium - pathology; Patient Outcome Assessment; Predictive Value of Tests; Prevalence; Prognosis; Prospective Studies; Risk Factors; Systemic vasculitis; Granulomatosis with polyangiitis; Cardiovascular disease; Prognosis; Systemic vasculitis
• ISSN: 0167-5273; 1874-1754
• DOI: 10.1016/j.ijcard.2015.06.087

To investigate the prevalence and prognostic relevance of cardiac involvement in an ANCA-associated vasculitis (AAV) population of eosinophilic granulomatosis with polyangiitis (EGPA) and granulomatosis with polyangiitis (GPA) patients. Prospective cohort study of fifty EGPA and forty-one GPA patients in sustained remission without previous in-depth cardiac screening attending our clinical immunology outpatient department. Cardiac screening included clinical evaluation, ECG, 24-hour Holter registration, echocardiography and cardiac magnetic resonance imaging (CMR) with coronary angiography and endomyocardial biopsy upon indication. Fifty age-, sex- and cardiovascular risk factor-matched control subjects were randomly selected from a population study. Long-term outcome was assessed using all-cause and cardiovascular mortality. A total of 91 AAV-patients (age 60±11, range 63–87years) were compared to 50-matched control subjects (age 60±9years, range 46–78years). ECG and echocardiography demonstrated cardiac abnormalities in 62% EGPA and 46% GPA patients vs 20% controls (P<0.001 and P=0.014, respectively). A total of 69 AAV-patients underwent additional CMR, slightly increasing the prevalence of cardiac involvement to 66% in EGPA and 61% in GPA patients. After a mean follow-up of 53±18months, presence of cardiac involvement using ECG and echocardiography in AAV-patients showed increased all-cause and cardiovascular mortality (Log-rank P=0.015 and Log-rank P=0.021, respectively). Cardiac involvement in EGPA and GPA patients with sustained remission is high, even if symptoms are absent and ECG is normal. Moreover, cardiac involvement is a strong predictor of (cardiovascular) mortality. Therefore, risk stratification using cardiac imaging is recommended in all AAV-patients, irrespective of symptoms or ECG abnormalities.