title: Prevalence and prognostic relevance of cardiac involvement in ANCA-associated vasculitis: eosinophilic granulomatosis with polyangiitis and granulomatosis with polyangiitis.
abstract: BACKGROUND
  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.
  
  
  METHODS
  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.
  
  
  RESULTS
  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).
  
  
  CONCLUSIONS
  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.
authors.affiliation: Maastricht University Medical Centre, Department of Cardiology, Maastricht, The Netherlands.
authors.forename: M R
authors.initials: MR
authors.lastname: Hazebroek
authors.affiliation: CARIM School for Cardiovascular Diseases, Maastricht University, Maastricht, The Netherlands; Maastricht University Medical Centre, Department of Nephrology and Clinical Immunology, Internal Medicine, Maastricht, The Netherlands.
authors.forename: M J
authors.initials: MJ
authors.lastname: Kemna
authors.affiliation: Maastricht University Medical Centre, Department of Cardiology, Maastricht, The Netherlands.
authors.forename: S
authors.initials: S
authors.lastname: Schalla
authors.affiliation: Maastricht University Medical Centre, Department of Cardiology, Maastricht, The Netherlands.
authors.forename: S
authors.initials: S
authors.lastname: Sanders-van Wijk
authors.affiliation: Maastricht University Medical Centre, Department of Radiology, Maastricht, The Netherlands.
authors.forename: S C
authors.initials: SC
authors.lastname: Gerretsen
authors.affiliation: Maastricht University Medical Centre, Department of Cardiology, Maastricht, The Netherlands.
authors.forename: R
authors.initials: R
authors.lastname: Dennert
authors.affiliation: Maastricht University Medical Centre, Department of Cardiology, Maastricht, The Netherlands.
authors.forename: J
authors.initials: J
authors.lastname: Merken
authors.affiliation: Research Unit Hypertension and Cardiovascular Epidemiology, Department of Cardiovascular Sciences, University of Leuven, Leuven Belgium.
authors.forename: T
authors.initials: T
authors.lastname: Kuznetsova
authors.affiliation: Research Unit Hypertension and Cardiovascular Epidemiology, Department of Cardiovascular Sciences, University of Leuven, Leuven Belgium; Department of Epidemiology, Maastricht University, Maastricht, The Netherlands.
authors.forename: J A
authors.initials: JA
authors.lastname: Staessen
authors.affiliation: Maastricht University Medical Centre, Department of Cardiology, Maastricht, The Netherlands.
authors.forename: H P
authors.initials: HP
authors.lastname: Brunner-La Rocca
authors.affiliation: Maastricht University Medical Centre, Department of Nephrology and Clinical Immunology, Internal Medicine, Maastricht, The Netherlands.
authors.forename: P
authors.initials: P
authors.lastname: van Paassen
authors.affiliation: CARIM School for Cardiovascular Diseases, Maastricht University, Maastricht, The Netherlands.
authors.forename: J W
authors.initials: JW
authors.lastname: Cohen Tervaert
authors.affiliation: Maastricht University Medical Centre, Department of Cardiology, Maastricht, The Netherlands. Electronic address: s.heymans@maastrichtuniversity.nl.
authors.forename: S
authors.initials: S
authors.lastname: Heymans
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D000465:Algorithms; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000150:complications* / Q000188:drug therapy / Q000453:epidemiology* / Q000276:immunology; D001706:Biopsy; D057791:Cardiac Imaging Techniques / Q000379:methods*; D002318:Cardiovascular Diseases / Q000150:complications* / Q000401:mortality; D015267:Churg-Strauss Syndrome / Q000150:complications / Q000188:drug therapy / Q000453:epidemiology; D015331:Cohort Studies; D017023:Coronary Angiography; D004452:Echocardiography; D004562:Electrocardiography; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000150:complications* / Q000188:drug therapy / Q000453:epidemiology* / Q000276:immunology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009206:Myocardium / Q000473:pathology; D063868:Patient Outcome Assessment; D011237:Predictive Value of Tests; D015995:Prevalence; D011379:Prognosis; D011446:Prospective Studies; D012307:Risk Factors
keywords: Cardiovascular disease; Granulomatosis with polyangiitis; Prognosis; Systemic vasculitis