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Clinical features and outcome of microscopic polyangiitis under a new consensus algorithm of ANCA-associated
Joong Kyong Ahn1, Ji-Won Hwang, Jaejoon Lee
1Department of Internal Medicine, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, 108 Pyoung-Dong, Jongro-Ku, Seoul, Republic of Korea.
Abstract:
The classification system for antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis and polyarteritis nodosa had its limitations due to numerous overlapping features of these disease entities. The aim of this study is to investigate the clinical features and outcome of patients diagnosed with microscopic polyangiitis (MPA) according to the newly proposed consensus algorithm of ANCA-associated vasculitides and polyarteritis nodosa. Fifty-five cases of MPA, comprised of 33 men and 22 women, diagnosed according to a new consensus algorithm at a single tertiary hospital were identified for analysis. The main clinical features were constitutional symptoms (78.2%), followed by renal involvement (74.5%), musculoskeletal symptoms (67.3%), skin manifestations (50.9%), neurologic involvement (43.6%), and lung involvement (41.8%). P-ANCA and/or anti-myeloperoxidase antibody were present in 69.1%. Five Factor Score and Birmingham Vasculitis Activity Score (BVAS) at diagnosis were 1.1 ± 0.9 and 10.9 ± 4.9, respectively. Forty-four patients were available for a long-term follow-up, and six patients (13.6%) resulted in death. Mortality was associated with BVAS > 9 at the time of diagnosis, age > 60 years, and presence of cardiomyopathy and interstitial lung disease. The survival rate at 1 and 3 years was 93.9 and 89.2%, respectively. Eight patients (14.5%) required dialysis at the time of diagnosis. This is the first study to demonstrate the clinical features in patients with MPA using a new consensus algorithm. Survival rate was higher than previously reported, and interstitial lung disease was a new risk factor for death in patients with MPA.
Insights
This study analyzed microscopic polyangiitis (MPA) using a new consensus algorithm, finding higher survival rates than previously reported. Interstitial lung disease emerged as a new risk factor for mortality in MPA patients.
Area of Science:
- Rheumatology
- Nephrology
- Immunology
Background:
- The classification of antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis and polyarteritis nodosa faced challenges due to overlapping clinical features.
- A new consensus algorithm was proposed to better differentiate these conditions.
Purpose of the Study:
- To investigate the clinical features and outcomes of patients diagnosed with microscopic polyangiitis (MPA) using the newly proposed consensus algorithm.
- To identify risk factors for mortality in MPA patients.
Main Methods:
- Retrospective analysis of 55 MPA cases diagnosed via the new consensus algorithm at a tertiary hospital.
- Evaluation of clinical manifestations, serological markers (P-ANCA, anti-MPO), and disease activity scores (Five Factor Score, BVAS).
- Long-term follow-up of 44 patients to assess survival rates and identify mortality predictors.
Main Results:
- Common clinical features included constitutional symptoms (78.2%), renal (74.5%), and musculoskeletal (67.3%) involvement.
- P-ANCA and/or anti-MPO antibodies were detected in 69.1% of patients.
- The 1- and 3-year survival rates were 93.9% and 89.2%, respectively, with mortality linked to BVAS > 9, age > 60, cardiomyopathy, and interstitial lung disease.
Conclusions:
- The new consensus algorithm aids in diagnosing MPA, revealing distinct clinical features and improved survival rates compared to prior reports.
- Interstitial lung disease is identified as a novel risk factor for mortality in MPA.
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