NIV 2014

Introduction: Cardiac involvement in ANCA-associated vasculitides (AAV), i.e. eosinophilic granulomatosis with polyangiitis (EGPA; Churg-Strauss syndrome) and granulomatosis with polyangiitis (GPA; Wegener’s granulomatosis) patients is an important predictor of mortality, but its prevalence remains unclear.

Aim of the study: To investigate the prevalence of cardiac involvement in a large population of ambulatory EGPA and GPA patients in sustained remission.

Material and Methods: To address the cardiac involvement in a phenotypical well characterized and large prospective cohort study of EGPA and GPA patients, we included 50 consecutive EGPA patients (aged 59±11 years) and 41 consecutive GPA patients (aged 60±11 years) in sustained remission and without previous in-depth cardiac screening. The latter comprised clinical evaluation, electrocardiography (ECG), 24-hour Holter registration, echocardiography and cardiac magnetic resonance imaging (CMR). Control subjects included fifty age- and sex-matched subjects, randomly selected from a population study undergoing ECG and echocardiography. Cardiac involvement characterized by major ECG abnormalities, pericardial effusion, (peri)myocarditis, focal or diffuse myocardial fibrosis and/or edema, wall motion abnormalities, valvular regurgitation ≥grade 3, pulmonary hypertension (sPAP>45 mmHg), diastolic dysfunction grade≥2, or significant coronary stenosi(e)s ≥70%.

Results: Age, sex and cardiovascular risk factors were similar between the EGPA, GPA and control group. ECG and echocardiography demonstrated cardiac involvement in 54% EGPA and 34% GPA patients as compared to 8% in controls (both P <0.002). Adding CMR as diagnostic modality increased the prevalence of cardiac involvement to 66% in EGPA and 61% in GPA patients. CMR detected cardiac involvement in all AAV patients demonstrating ECG and/or echocardiographic involvement. In patients without such abnormalities, CMR additionally demonstrated cardiac involvement in over 30% of EGPA and 41% of GPA. In 52% EGPA and 44% GPA patients without cardiac symptoms and with normal ECG, cardiac involvement was present. With respect to ANCA detection, cardiac involvement was equally frequent in ANCA negative versus ANCA positive patients (71% (24/34) ANCA- versus 56% (9/16) ANCA+ EGPA patients, P=0.53; 0% (0/1) ANCA- versus 63% (25/40) ANCA+ GPA patients). Endomyocardial biopsy performed in 11 EGPA and 2 GPA patients demonstrated chronic or acute myocarditis in all but one patient.

Conclusion: This large prospective and well characterized cohort reveals an up to 66% cardiac involvement in AAV patients in remission, even in the absence of cardiac symptoms or ECG abnormalities. Therefore, the use of imaging techniques, especially CMR, is recommended for cardiac evaluation of EGPA and GPA patients.