Background/Purpose: Eosinophilic granulomatosis with polyangiitis (EGPA) is a vasculitis characterized by eosinophilia and granuloma formation, affecting multiple organs. The Five-Factor Score (Medicine, 2011) identifies key prognostic factors, including severe cardiac or gastrointestinal involvement, renal failure, age ≥65, and absence of ENT symptoms. Among these, cardiac involvement is most closely linked to poor outcomes. Although CNS involvement is rare and not included in the score, it may still affect prognosis. Major causes of death in EGPA include cardiac and CNS complications, gastrointestinal perforation, respiratory failure, and infections. Treatment typically includes glucocorticoids and cyclophosphamide, with newer agents like anti–IL-5/IL-5R monoclonal antibodies and rituximab for refractory cases. This study evaluated treatment and outcomes in patients with organ involvement affecting survival.

Methods: We retrospectively analyzed 51 patients with EGPA treated at our hospital from 2000 to 2024. The mean age at onset was 60.1±12.1 years. We focused on cardiac and cerebrovascular involvement. CNS lesions were included only if they occurred during the active disease phase. We also assessed the presence of lifestyle-related diseases (e.g., smoking, hypertension, dyslipidemia, diabetes) and ANCA status. Treatments used for remission induction and maintenance were reviewed. Prognosis was analyzed according to presence of cardiac and CNS involvement. In fatal cases, we examined causes of death, treatment regimens, and glucocorticoid dosages. Relative risk with 95% confidence intervals was used for analysis.

Results: All patients received glucocorticoids. Cyclophosphamide was used in 9 cases, rituximab in 2, IVIG in 17, mepolizumab in 35, azathioprine in 24, mycophenolate mofetil in 7, and methotrexate in 7. Mepolizumab was effective in reducing prednisolone doses. Cardiac involvement was observed in 8 patients, all presenting during the initial phase. None relapsed after treatment. Cardiac function was evaluated over time using echocardiography, focusing on wall motion abnormalities and pericardial effusion. Two patients showed marked improvement with immunosuppressive therapy. One had pre-existing arteriosclerosis and responded poorly. However, all 8 survived beyond 12 months with no cardiac deterioration. Cerebrovascular events occurred in 11 cases—9 cases at initial presentation, 2 cases at 3- and 11-years post-onset. Lifestyle diseases were not significant risk factors for cardiac or CNS lesions. Four patients died (mean age 78±4 years). One died of liver cancer; three had refractory disease with persistent glucocorticoid dependency. Of these, two died from pneumonia and one from CNS involvement. Cardiac and CNS involvement were not significant prognostic factors. However, age ≥61 at onset and maintenance prednisolone doses ≥7.5 mg/day were significant risk factors.

Conclusion: Cardiac and cerebrovascular involvement in EGPA may be controlled long-term with appropriate early treatment. In contrast, older-onset patients with glucocorticoid-dependent disease require careful management to prevent fatal infections and poor outcomes.

Table. Baseline characteristics of the study population.Clinical manifestations, laboratory parameters, organ involvement, comorbid lifestyle-related diseases, and treatment modalities are presented for the entire cohort, and stratified by survival status (surviving and fetal cases). Proportions within each group are shown in parentheses.

Figure. Kaplan–Meier survival curves.

A: Comparison of overall survival between patients with and without cardiac involvement showed no significant difference.

B: No significant difference in survival was observed between patients with and without cerebrovascular involvement.

C: Patients receiving maintenance prednisolone doses ≥7.5 mg/day demonstrated significantly reduced survival compared to those receiving < 7.5 mg/day.

D: Patients with disease onset at age ≥61 years had significantly lower survival rates than those with onset at ≤60 years.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/clinical-outcomes-and-prognostic-impact-of-cardiac-and-cerebrovascular-involvement-in-eosinophilic-granulomatosis-with-polyangiitis-a-retrospective-study/