Abstract: TH-PO0757
Investigation of Clinical Features of Eosinophilic Granulomatosis with Polyangiitis (EGPA) in 55 Myeloperoxidase (MPO)-ANCA-Positive and -Negative Patients in Kyoto University Hospital
Session Information
- Glomerular Histopathology: Evolving Insights
November 06, 2025 | Location: Exhibit Hall, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Authors
- Sugioka, Sayaka, Department of Nephrology, Graduate School of Medicine, Kyoto University, Kyoto, Japan
- Yamamoto, Shinya, Department of Nephrology, Graduate School of Medicine, Kyoto University, Kyoto, Japan
- Kosaka, Tatsuaki, Department of Nephrology, Graduate School of Medicine, Kyoto University, Kyoto, Japan
- Shoji, Mikihito, Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto, Japan
- Hiwa, Ryosuke, Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto, Japan
- Morinobu, Akio, Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto, Japan
- Yanagita, Motoko, Department of Nephrology, Graduate School of Medicine, Kyoto University, Kyoto, Japan
Background
EGPA is a necrotizing vasculitis involving small to medium-sized systemic blood vessels characterized by eosinophil infiltration and granuloma formation. The disease is classically divided into three phases: allergy, eosinophilic infiltration, and vasculitis and it progresses from one phase to next slowly typically. However, it is difficult to diagnose and treat because phases may be absent, overlapping, or extending and EGPA symptoms vary widely depending on the affected organ.
Methods
We retrospectively collected 55 patients diagnosed with EGPA who visited the Department of Immunology and Collagen Disease and the Department of Nephrology at Kyoto University between 2005 and 2024. First, we examined the characteristics of organ involvement (kidney, lung, paranasal sinus, heart, nerve, skin) in MPO-ANCA-positive and negative EGPA patients. Next, we examined the relationship between MPO-ANCA titer and the renal lesions (hematuria, proteinuria and AKI) and the number of years between initial symptoms and EGPA diagnosis. Third, we examined the renal prognosis of all EGPA patients.
Results
Renal lesions were significantly more common in MPO-ANCA-positive patients (52.4%) compared to MPO-ANCA-negative patients (14.7%) (p＜0.01). Ear and nasopharyngeal lesions tended to be more common in MPO-ANCA-positive patients whereas cardiac lesions tended to be more common in MPO-ANCA-negative patients. Among MPO-ANCA-positive patients, there was no association between MPO-ANCA titer and the presence of hematuria, proteinuria and AKI, and the duration between initial symptoms and EGPA diagnosis. After treatment, 83.3% of patients with hematuria and 66.7% of patients with proteinuria achieved complete remission, while AKI patients improved but not back to baseline renal function.
Conclusion
Clinical features may differ between MPO-ANCA-positive and negative EGPA cases. The rate of renal complications is higher in MPO-ANCA-positive EGPA cases than in MPO-ANCA-negative cases. The severity of renal lesions and when they appear cannot be predicted by MPO-ANCA titer. Generally, the renal prognosis of EGPA is good, however in some cases they can be severe and appear a long time after the initial lesions. Therefore, careful follow-up is required in EGPA cases.