英国和日本队列中肉芽肿性多血管炎的表型与结局比较。
Comparison of the Phenotype and Outcome of Granulomatosis with Polyangiitis Between UK and Japanese Cohorts.
作者信息
Furuta Shunsuke, Chaudhry Afzal N, Arimura Yoshihiro, Dobashi Hiroaki, Fujimoto Shouichi, Homma Sakae, Rasmussen Niels, Jayne David R
机构信息
From Department of Allergy and Clinical Immunology, Chiba University Hospital, Chiba, Japan; Lupus and Vasculitis Clinic, Addenbrooke's Hospital, Cambridge University Hospitals, Cambridge, UK; Department of Nephrology and Rheumatology, Kyorin University School of Medicine, Tokyo; Department of Internal Medicine, Division of Hematology, Rheumatology and Respiratory Medicine, Faculty of Medicine, Kagawa University, Kagawa; Department of Hemovascular Medicine and Artificial Organs, Faculty of Medicine, University of Miyazaki, Miyazaki; Department of Respiratory Medicine, Toho University Omori Medical Center, Tokyo, Japan; Department of Autoimmune Serology, Statens Seruminstitut, Copenhagen, Denmark.
S. Furuta, MD, PhD, Chiba University Hospital; A.N. Chaudhry, MD, PhD, Addenbrooke's Hospital; Y. Arimura, MD, PhD, Professor, Kyorin University School of Medicine; H. Dobashi, MD, PhD, Kagawa University; S. Fujimoto, MD, PhD, Professor, University of Miyazaki; S. Homma, MD, PhD, Professor, Toho University Omori Medical Center; N. Rasmussen, MD, Statens Seruminstitut; D.R. Jayne, MD, FRCP, FMedSci, Department of Medicine, University of Cambridge.
出版信息
J Rheumatol. 2017 Feb;44(2):216-222. doi: 10.3899/jrheum.160005. Epub 2016 Nov 1.
OBJECTIVE
There are differences in the frequencies of antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis subgroups between different geographic regions, and we have reported differences in the phenotype of microscopic polyangiitis between Europe and Japan. In this retrospective observational study, we compared phenotypes and outcomes of granulomatosis with polyangiitis (GPA) between the United Kingdom and Japan.
METHODS
We identified 128 UK and 82 Japanese patients with GPA diagnosed between 2000 and 2012. We evaluated baseline characteristics including ANCA status and organ involvement, treatment, patient and renal survival, and time to first relapse.
RESULTS
Median age at onset was higher in Japan than in the UK (62.2 yrs vs 57.5 yrs, p < 0.01). The proportion of patients with proteinase 3 (PR3)-ANCA was lower in Japan than in the UK (61.0% vs 85.2%, p < 0.01), while the proportion of myeloperoxidase-ANCA was higher in Japan than the UK (34.1% vs 8.6%, p < 0.01). Serum creatinine at diagnosis was lower in Japan than the UK (68.1 μmol/l vs 101.0 μmol/l, p < 0.01). Respiratory involvement was more frequent in Japan than the UK (69.5% vs 40.6%, p < 0.01). In both countries, most patients received both glucocorticoids and cyclophosphamide. At 60 months the cumulative survival rates were 87.6% and 94.3% in Japan and the UK, respectively (p = 0.03). At 60 months the cumulative relapse rates were 37.1% and 68.1% in Japan and the UK, respectively (p < 0.01).
CONCLUSION
Japanese patients with GPA were older at disease onset, with less PR3-ANCA positivity, milder renal dysfunction, and more frequent respiratory involvement than UK patients. The relapse-free survival rate was higher in Japan than the United Kingdom.
目的
不同地理区域抗中性粒细胞胞浆抗体（ANCA）相关血管炎亚组的发病频率存在差异，并且我们已经报道了欧洲和日本显微镜下多血管炎的表型差异。在这项回顾性观察研究中，我们比较了英国和日本肉芽肿性多血管炎（GPA）的表型和结局。
方法
我们确定了2000年至2012年间诊断为GPA的128例英国患者和82例日本患者。我们评估了基线特征，包括ANCA状态和器官受累情况、治疗、患者及肾脏生存率以及首次复发时间。
结果
日本患者的发病年龄中位数高于英国（62.2岁对57.5岁，p<0.01）。日本蛋白酶3（PR3）-ANCA阳性患者的比例低于英国（61.0%对85.2%，p<0.01），而髓过氧化物酶-ANCA阳性患者的比例日本高于英国（34.1%对8.6%，p<0.01）。日本诊断时的血清肌酐低于英国（68.1μmol/L对101.0μmol/L，p<0.01）。日本呼吸道受累比英国更常见（69.5%对40.6%，p<0.01）。在两个国家，大多数患者都接受了糖皮质激素和环磷酰胺治疗。60个月时，日本和英国的累积生存率分别为87.6%和94.3%（p=0.03）。60个月时，日本和英国的累积复发率分别为37.1%和68.1%（p<0.01）。
结论
与英国患者相比，日本GPA患者发病年龄更大，PR3-ANCA阳性率更低，肾功能不全更轻，呼吸道受累更频繁。日本的无复发生存率高于英国。
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