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

Int J Cardiol. 2015 Nov 15:199:170-9. doi: 10.1016/j.ijcard.2015.06.087. Epub 2015 Jul 15.


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).

Conclusion: 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.

Keywords: Cardiovascular disease; Granulomatosis with polyangiitis; Prognosis; Systemic vasculitis.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • 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 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 / 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