Sada Ken-ei, Yamamura Masahiro, Harigai Masayoshi, Fujii Takao, Dobashi Hiroaki, Takasaki Yoshinari, Ito Satoshi, Yamada Hidehiro, Wada Takashi, Hirahashi Junichi, Arimura Yoshihiro, Makino Hirofumi
Arthritis Res Ther. 2014 Apr 23;16(2):R101. doi: 10.1186/ar4550.
We investigated the clinical and serological features of patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in Japan using data from a nationwide, prospective, inception cohort study.
In total, 156 Japanese patients with newly diagnosed AAV were classified according to the European Medicines Agency (EMEA) algorithm with exploratory surrogate markers for AAV-related non-granulomatous pulmonary lesions, predefined as alveolar haemorrhage and interstitial lung disease (ILD), and their clinical and serological features were evaluated.
Using the EMEA algorithm, we identified 14 patients (9.0%) with eosinophilic granulomatosis with polyangiitis (EGPA), 33 (21.2%) with granulomatosis with polyangiitis (GPA), 78 (50.0%) with microscopic polyangiitis and renal-limited vasculitis (MPA/RLV), and 31 (19.9%) with unclassifiable vasculitis. The average ages of patients with EGPA (male/female, 5/9), GPA (12/21), and MPA/RLV (35/43) and unclassifiable (9/22) were 58.0, 63.6, 71.1, and 70.6 years, respectively. Myeloperoxidase (MPO)-ANCA and proteinase-3 ANCA positivity was 50.0% and 0% for EGPA, 54.6% and 45.5% for GPA, 97.4% and 2.6% for MPA/RLV, and 93.5% and 3.2% for unclassifiable, respectively. According to the Birmingham Vasculitis Activity Score (BVAS), cutaneous (71.4%) and nervous system (92.9%) manifestations were prominent in EGPA and ear, nose, and throat manifestations (84.9%) and chest manifestations (66.7%) in GPA. Renal manifestations developed frequently in MPA/RLV (91.0%) and GPA (63.6%). The average serum creatinine levels were 0.71 mg/dL for EGPA, 1.51 mg/dL for GPA, 2.46 mg/dL for MPA/RLV, and 0.69 mg/dL for unclassifiable. The percentages of patients with ILD were 14.3% for EGPA, 9.0% for GPA, 47.4% for MPA/RLV, and 61.3% for unclassifiable. Patients with ILD (n = 61) had significantly lower BVAS (P = 0.019) with fewer ear, nose, and throat and cardiovascular manifestations than patients without ILD (n = 95).
MPO-ANCA-positive MPA/RLV is the most common form of AAV in Japanese patients, and one-half of patients with GPA were positive for MPO-ANCA. ILD is an important clinical manifestation in Japanese patients with AAV. Unclassifiable vasculitis with MPO-ANCA positivity and ILD may represent a novel variant of MPA.
The University Hospital Medical Information Network Clinical Trials Registry: UMIN000001648. Registered 28 February 2009.
我们利用一项全国性、前瞻性、起始队列研究的数据，调查了日本抗中性粒细胞胞浆抗体（ANCA）相关血管炎（AAV）患者的临床和血清学特征。
总共156例新诊断的日本AAV患者根据欧洲药品管理局（EMEA）算法进行分类，使用AAV相关非肉芽肿性肺部病变的探索性替代标志物，将其预先定义为肺泡出血和间质性肺病（ILD），并评估他们的临床和血清学特征。
使用EMEA算法，我们识别出14例（9.0%）嗜酸性肉芽肿性多血管炎（EGPA）患者、33例（21.2%）肉芽肿性多血管炎（GPA）患者、78例（50.0%）显微镜下多血管炎和肾局限性血管炎（MPA/RLV）患者以及31例（19.9%）无法分类的血管炎患者。EGPA患者（男/女，5/9）、GPA患者（12/21）、MPA/RLV患者（35/43）和无法分类的患者（9/22）的平均年龄分别为58.0岁、63.6岁、71.1岁和70.6岁。EGPA患者中髓过氧化物酶（MPO）-ANCA和蛋白酶3-ANCA阳性率分别为50.0%和0%，GPA患者中分别为54.6%和45.5%，MPA/RLV患者中分别为97.4%和2.6%，无法分类的患者中分别为93.5%和3.2%。根据伯明翰血管炎活动评分（BVAS），皮肤表现（71.4%）和神经系统表现（92.9%）在EGPA中较为突出，耳、鼻、喉表现（84.9%）和胸部表现（66.7%）在GPA中较为突出。肾脏表现在MPA/RLV（91.0%）和GPA（63.6%）中经常出现。EGPA患者的平均血清肌酐水平为0.71mg/dL，GPA患者为1.51mg/dL，MPA/RLV患者为2.46mg/dL，无法分类的患者为0.69mg/dL。EGPA患者中ILD的比例为14.3%，GPA患者中为9.0%，MPA/RLV患者中为47.4%，无法分类的患者中为61.3%。与无ILD的患者（n = 95）相比，有ILD的患者（n = 61）的BVAS显著更低（P = 0.019），耳、鼻、喉和心血管表现更少。
MPO-ANCA阳性的MPA/RLV是日本AAV患者中最常见的形式，GPA患者中有一半MPO-ANCA呈阳性。ILD是日本AAV患者的重要临床表现。MPO-ANCA阳性且伴有ILD的无法分类的血管炎可能代表MPA的一种新变体。
大学医院医学信息网络临床试验注册中心：UMIN000001648。2009年2月28日注册。
