Classification and characteristics of Japanese patients with antineutrophil cytoplasmic antibody-associated vasculitis in a nationwide, prospective, inception cohort study.
Classification and characteristics of Japanese patients with antineutrophil cytoplasmic antibody-associated vasculitis in a nationwide, prospective, inception cohort study.
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DOI：
10.1186/ar4550
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发表时间：
2014-04-23
影响因子：
4.9
通讯作者：
Research Committee on Intractable Vasculitides, the Ministry of Health, Labour and Welfare of Japan
中科院分区：
文献类型：
--
作者：
Sada KE;Yamamura M;Harigai M;Fujii T;Dobashi H;Takasaki Y;Ito S;Yamada H;Wada T;Hirahashi J;Arimura Y;Makino H;Research Committee on Intractable Vasculitides, the Ministry of Health, Labour and Welfare of Japan
我们使用一项全国性、前瞻性、初始队列研究的数据，调查了日本抗神经细胞胞浆抗体（ANCA）相关性血管炎（AAV）患者的临床和血清学特征。根据欧洲药品管理局（EMEA）的算法，对156例新诊断的AAV日本患者进行了分类，其中AAV相关非肉芽肿性肺病变的探索性替代标志物预定义为肺泡出血和间质性肺病（ILD），并对其临床和血清学特征进行了评价。使用EMEA算法，我们确定了14例（9.0%）嗜酸性肉芽肿病伴多血管炎（EGPA），33例（21.2%）肉芽肿病伴多血管炎（GPA），78例（50.0%）显微镜下多血管炎和肾局限性血管炎（MPA/RLV），31例（19.9%）不可分类的血管炎。EGPA（男/女，5/9）、GPA（12/21）、MPA/RLV（35/43）和无法分类（9/22）患者的平均年龄分别为58.0、63.6、71.1和70.6岁。髓过氧化物酶（MPO）ANCA和蛋白酶3 ANCA的阳性率分别为EGPA 50.0%和0%、GPA 54.6%和45.5%、MPA/RLV 97.4%和2.6%、无法分类者93.5%和3.2%。根据伯明翰血管炎活动性评分（BVAS），EGPA以皮肤（71.4%）和神经系统（92.9%）表现突出，GPA以耳鼻咽喉（84.9%）和胸部（66.7%）表现突出。MPA/RLV组和GPA组肾脏损害发生率分别为91.0%和63.6%。EGPA的平均血清肌酐水平为0.71 mg/dL，GPA为1.51 mg/dL，MPA/RLV为2.46 mg/dL，无法分类为0.69 mg/dL。发生ILD的患者百分比分别为EGPA 14.3%、GPA 9.0%、MPA/RLV 47.4%和无法分类的61.3%。ILD患者（n = 61）的BVAS显著低于无ILD患者（n = 95）（P = 0.019），耳、鼻、喉和心血管表现较少。MPO-ANCA阳性MPA/RLV是日本患者中最常见的AAV形式，一半GPA患者的MPO-ANCA阳性。ILD是日本AAV患者的重要临床表现。MPO-ANCA阳性的不可分类的血管炎和ILD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：UMIN 000001648。2009年2月28日注册。
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.
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DOI：
10.1093/ndtplus/sfq050
发表时间：
2010-08
期刊：
NDT plus
影响因子：
--
作者：
Bhanji A;Karim M
通讯作者：
Karim M
影响因子：
19.6
作者：
Hauer, HA;Bajema, IM;Hagen, EC
通讯作者：
Hagen, EC
影响因子：
4.3
作者：
Foulon, Guillaume;Delaval, Philippe;Crestani, Bruno
通讯作者：
Crestani, Bruno
影响因子：
4.3
作者：
Ando, Masaru;Miyazaki, Eishi;Kumamoto, Toshihide
通讯作者：
Kumamoto, Toshihide
影响因子：
6.9
作者：
Homma, S;Matsushita, H;Nakata, K
通讯作者：
Nakata, K