Background/Purpose: Cardiovascular involvement in ANCA-associated vasculitis (AAV) including microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA) and granulomatosis with polyangiitis (GPA), has long been regarded as rare, yet a wide spectrum of abnormalities have been reported, with prevalence figures ranging from 5–60% of patients depending on the series and diagnostic methods applied. To investigate the prevalence and prognostic relevance of cardiovascular involvement in an AAV population of MPA, EGPA and GPA patients.

Methods: In a retrospective cohort study, 143 MPA, 46 GPA and 45 EGPA patients were analyzed. Birmingham vasculitis activity (BVA) score of all patients were calculated. Demographic and clinical characteristics of patients with and without AAV-related cardiovascular involvement were compared. Statistical analysis was done using SPSS version 22.0 for Windows.

Results: Within the cohort of 234 AAV patients, median follow-up times were 5.6 years for the MPA patients (age at diagnosis 55 ± 17, range 12–83years) and 5.2 years for the GPA patients (age 53± 16, range 12–82 years) and 4.8 years for the EGPA (age 48 ± 17, range 11–76 years). The percentages of cardiovascular involvement in patients with MPA, GPA and EGPA were 29.4%(42/143), 37.0%(17/46), and 35.6%(16/45), respectively. There is no significant difference among the three groups in terms of cardiovascular involvement. The common cardiovascular event in these 75 patients that can be observed is congestive heart failure (28/75), followed with valve disease (27/75), pericardial effusion (17/75), arrhythmia (17/75), cardiomyopathy (6/75) and angina (5/75). Univariate logistic regressions showed that age at diagnosis (P=0.002; 95% CI 2.922–12.221)), sex (P=0.03; 95% CI 0.959-0.998), higher BVA score (P<0.001; 95% CI, 3.063–6.429) and poor respiratory function at remission (P=0.013; 95% CI 1.282–8.595), respectively correlated with AAV patients with cardiovascular involvement. Mortality rate was 4.2% in AAV patients (10/243); 7 out of 10 death cases had cardiovascular involvement. There was a statistical significance of mortality between the patients with and without cardiovascular involvement.

Conclusion: In this retrospective study, the MPA, GPA and EGPA groups showed comparable mortality. AAV patients with cardiovascular involvement appeared at greater risk of premature death, with increased risk in males, elder age, higher BVA score and poor respiratory function at remission.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/prevalence-and-prognostic-relevance-of-cardiovascular-involvement-in-anca-associated-vasculitis-a-retrospective-cohort-study/