Yoo Juyoung, Kim Ho Jae, Ahn Sung Soo, Jung Seung Min, Song Jason Jungsik, Park Yong-Beom, Lee Sang-Won
Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Republic of Korea.
Clin Exp Rheumatol. 2017 Mar-Apr;35 Suppl 103(1):111-118. Epub 2017 Mar 23.
We reclassified Korean patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) into 3 categories of AAV including MPO-ANCA, PR3-ANCA and ANCA-negative vasculitis, and investigated clinical and prognostic features.
We reviewed the medical records of 133 patients with microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic GPA (EGPA), who had either myeloperoxidase (MPO)-ANCA, proteinase 3 (PR3)-ANCA or no ANCA, and who had ever achieved the first remission. We compared clinical manifestations, initial Birmingham vasculitis activity score (BVAS) and five factor score (FFS), and relapse rates.
Patients with ANCA-negative vasculitis had the youngest mean age at diagnosis (50.0 years old) among AAV categories. General, cutaneous and renal manifestations were commonly observed in MPO-ANCA vasculitis, while mucous membrane, eye, ear nose throat (ENT) and renal manifestations were often documented in PR3-ANCA vasculitis. ENT manifestation was also frequently observed in ANCA-negative vasculitis. However, there were no significant differences in pulmonary and nervous system manifestations among 3 AAV categories. There were no significant differences in cumulative relapse free survival according to the presence of MPO-ANCA or PR3-ANCA or no ANCA. Meanwhile, initial BVAS or BVAS for GPA ≥13.5 in MPO-ANCA vasculitis and initial FFS (1996) ≥1 in MPO-ANCA and ANCA-negative vasculitis were significant predictors of relapse of each AAV category.
Clinical manifestations varied AAV categories, and neither MPO-ANCA nor PR3-ANCA significantly affected relapse of AAV. Initial BVAS or BVAS for GPA and FFS (1996) helped to predict relapse of specified AAV categories.
我们将韩国抗中性粒细胞胞浆抗体（ANCA）相关血管炎（AAV）患者重新分类为3类AAV，包括髓过氧化物酶（MPO）-ANCA、蛋白酶3（PR3）-ANCA和ANCA阴性血管炎，并研究其临床和预后特征。
我们回顾了133例显微镜下多血管炎（MPA）、肉芽肿性多血管炎（GPA）和嗜酸性GPA（EGPA）患者的病历，这些患者分别具有髓过氧化物酶（MPO）-ANCA、蛋白酶3（PR3）-ANCA或无ANCA，且均曾达到首次缓解。我们比较了临床表现、初始伯明翰血管炎活动评分（BVAS）和五因素评分（FFS）以及复发率。
在AAV各类型中，ANCA阴性血管炎患者诊断时的平均年龄最小（50.0岁）。MPO-ANCA血管炎常见全身、皮肤和肾脏表现，而PR3-ANCA血管炎常记录有黏膜、眼、耳、鼻、喉（ENT）和肾脏表现。ENT表现在ANCA阴性血管炎中也经常观察到。然而，3种AAV类型在肺部和神经系统表现方面无显著差异。根据是否存在MPO-ANCA、PR3-ANCA或无ANCA，累积无复发生存率无显著差异。同时，MPO-ANCA血管炎中初始BVAS或GPA的BVAS≥13.5以及MPO-ANCA和ANCA阴性血管炎中初始FFS（1996）≥1是各AAV类型复发的重要预测因素。
AAV各类型临床表现各异，MPO-ANCA和PR3-ANCA均未显著影响AAV的复发。初始BVAS或GPA的BVAS以及FFS（1996）有助于预测特定AAV类型的复发。
