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Clinical features and outcomes of microscopic polyangiitis in Korea
Ji Seon Oh1, Chang-Keun Lee, Yong Gil Kim
1Division of Allergy and Rheumatology, Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea.
Abstract:
Microscopic polyangiitis (MPA) is a systemic vasculitis affecting small vessels. To determine the clinical features and outcomes of MPA in Korean patients, we retrospectively reviewed the medical records of patients diagnosed with MPA at a single medical center in Korea between 1989 and 2006. The 18 patients who met the Chapel Hill criteria for MPA had a mean (+/-SD) age at the time of diagnosis of 62.4+/-12.7 yr. Renal manifestations and general symptoms were the most common features of MPA, with lung involvement also very common. Antineutrophil cytoplasmic antibodies (ANCA) were present in 17 of the 18 patients (94%). Of 17 patients treated with steroids and cyclophosphamide, 11 (65%) had stable or improved course. One patient treated with steroids without cyclophosphamide showed disease progression. Ten of the 18 patients (56%) died at a median follow-up of 8 months. MPA in Korean patients was distinguished by a higher rate of lung involvement, especially alveolar hemorrhage, which was the leading cause of death in our patients. Korean patients were also older at MPA onset and were more likely positive for ANCA. Other overall clinical manifestations did not differ significantly.
Insights
Microscopic polyangiitis (MPA) in Korean patients often presents with kidney and lung issues, particularly alveolar hemorrhage. While ANCA positivity is common, treatment with steroids and cyclophosphamide showed some success, though mortality remains high.
Area of Science:
- Nephrology
- Rheumatology
- Pulmonology
Background:
- Microscopic polyangiitis (MPA) is a rare systemic vasculitis impacting small blood vessels.
- Understanding MPA's clinical profile in diverse populations is crucial for effective management.
Purpose of the Study:
- To delineate the clinical characteristics and outcomes of MPA in Korean patients.
- To identify specific features distinguishing Korean MPA patients from other ethnic groups.
Main Methods:
- Retrospective review of medical records for 18 patients diagnosed with MPA between 1989 and 2006.
- Patients met the established Chapel Hill criteria for MPA diagnosis.
Main Results:
- The average age at diagnosis was 62.4 years, with renal and general symptoms being most frequent.
- Lung involvement, especially alveolar hemorrhage, was notably common and a primary cause of death.
- Antineutrophil cytoplasmic antibodies (ANCA) were detected in 94% of patients.
- Treatment with steroids and cyclophosphamide led to improvement in 65% of patients.
Conclusions:
- MPA in Korean patients exhibits a higher incidence of lung involvement and alveolar hemorrhage compared to other populations.
- Korean MPA patients tend to be older at onset and have a higher prevalence of ANCA positivity.
- Despite treatment, MPA carries a significant mortality risk, underscoring the need for further research and improved therapeutic strategies.
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