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ANCA-associated vasculitis: report from Korea
Hye Won Kim1, Yeong Wook Song2,3
1Division of Rheumatology, Department of Internal Medicine, Seoul National University Hospital, 101 Daehak-ro, Jongno-gu, Seoul, 110-744, Korea.
Clinical and Experimental Nephrology
|January 8, 2013
Summary
This study reviews Korean patients with anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV). Korean AAV patients show distinct features, including lower ANCA positivity in GPA and EGPA, and reduced renal involvement compared to Caucasians.
Area of Science:
- Rheumatology
- Immunology
- Internal Medicine
Background:
- Anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) encompasses several autoimmune diseases characterized by inflammation of small blood vessels.
- Understanding the specific clinical manifestations and serological profiles of AAV in different ethnic populations is crucial for accurate diagnosis and management.
Purpose of the Study:
- To investigate and summarize the distinct clinical features of Korean patients diagnosed with ANCA-associated vasculitis (AAV) based on a comprehensive literature review.
- To compare the characteristics of AAV in Korean patients with those reported in Caucasian populations, focusing on disease prevalence, ANCA positivity, and organ involvement.
Main Methods:
- A systematic literature review was conducted to identify studies detailing the clinical characteristics of Korean patients with AAV.
- Data extraction focused on disease subtypes (GPA, MPA, EGPA), ANCA serotype and positivity rates, and the extent of organ involvement, particularly renal disease.
Main Results:
- Granulomatosis with polyangiitis (GPA) in Korean patients often presents as limited or granulomatous disease.
- ANCA positivity rates were notably lower in Korean GPA (56.6-68.9%) and EGPA (5.9-8.3%) compared to Microscopic Polyangiitis (MPA) (69-94%).
- Korean patients with GPA and EGPA exhibited lower rates of renal involvement and progression to end-stage renal disease compared to Caucasian counterparts.
Conclusions:
- Korean patients with AAV display unique clinical characteristics that differ from those observed in Caucasian populations.
- The prevalence of specific ANCA subtypes and the extent of renal disease in Korean AAV patients warrant further investigation in large-scale studies.

