title: Clinical features and outcome of microscopic polyangiitis under a new consensus algorithm of ANCA-associated vasculitides in 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.
authors: Department of Internal Medicine, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, 108 Pyoung-Dong, Jongro-Ku, Seoul, Republic of Korea.
authors: Joong Kyong
authors: JK
authors: Ahn
authors: Ji-Won
authors: JW
authors: Hwang
authors: Jaejoon
authors: J
authors: Lee
authors: Chan Hong
authors: CH
authors: Jeon
authors: Hoon-Suk
authors: HS
authors: Cha
authors: Eun-Mi
authors: EM
authors: Koh
mesh_terms: D000368:Aged; D000465:Algorithms; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000145:classification / Q000175:diagnosis* / Q000208:ethnology / Q000401:mortality / Q000628:therapy; D044466:Asian People; D002423:Cause of Death; D016009:Chi-Square Distribution; D032921:Consensus; D018450:Disease Progression; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D016015:Logistic Models; D008297:Male; D055953:Microscopic Polyangiitis / Q000145:classification / Q000175:diagnosis* / Q000208:ethnology / Q000401:mortality / Q000628:therapy; D008875:Middle Aged; D015999:Multivariate Analysis; D011237:Predictive Value of Tests; D011379:Prognosis; D006435:Renal Dialysis; D056910:Republic of Korea / Q000453:epidemiology; D018570:Risk Assessment; D012307:Risk Factors; D015996:Survival Rate; D013997:Time Factors