Department of Internal Medicine, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, 108 Pyoung-Dong, Jongro-Ku, Seoul, Republic of Korea.
Rheumatol Int. 2012 Oct;32(10):2979-86. doi: 10.1007/s00296-011-2079-4. Epub 2011 Sep 7.
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.
抗中性粒细胞胞浆抗体（ANCA）相关性血管炎和多动脉炎的分类系统由于这些疾病实体存在许多重叠的特征，因此存在局限性。本研究旨在根据新提出的 ANCA 相关性血管炎和多动脉炎的共识算法，研究诊断为显微镜下多血管炎（MPA）的患者的临床特征和结局。在一家三级医院，根据新的共识算法诊断为 MPA 的 55 例患者被确定为分析对象，其中男性 33 例，女性 22 例。主要临床特征为全身症状（78.2%），其次为肾脏受累（74.5%）、肌肉骨骼症状（67.3%）、皮肤表现（50.9%）、神经受累（43.6%）和肺部受累（41.8%）。p-ANCA 和/或抗髓过氧化物酶抗体阳性率为 69.1%。诊断时的 5 因素评分和伯明翰血管炎活动评分（BVAS）分别为 1.1±0.9 和 10.9±4.9。44 例患者可进行长期随访，6 例患者（13.6%）死亡。死亡与诊断时的 BVAS>9、年龄>60 岁、心肌病和间质性肺病有关。1 年和 3 年的生存率分别为 93.9%和 89.2%。8 例（14.5%）患者在诊断时需要透析。这是第一项使用新共识算法研究 MPA 患者临床特征的研究。生存率高于以往报道，间质性肺病是 MPA 患者死亡的新危险因素。
