Relevance of Combined Clinicopathologic Phenotype and Antineutrophil Cytoplasmic Autoantibody Serotype in the Diagnosis of Antineutrophil Cytoplasmic Autoantibody Vasculitis.
Relevance of Combined Clinicopathologic Phenotype and Antineutrophil Cytoplasmic Autoantibody Serotype in the Diagnosis of Antineutrophil Cytoplasmic Autoantibody Vasculitis.
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DOI：
10.1016/j.ekir.2022.09.011
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发表时间：
2022-12
影响因子：
6
通讯作者：
Hogan, Susan L.
中科院分区：
文献类型：
--
作者：
Alba, Marco A.;Jennette, J. Charles;Hu, Yichun;Poulton, Caroline J.;Blazek, Lauren;Derebail, Vimal K.;Falk, Ronald J.;Hogan, Susan L.
肉芽肿性多血管炎 (GPA) 和显微镜下多血管炎 (MPA) 是抗中性粒细胞胞浆自身抗体 (ANCA) 血管炎的两种主要临床病理变异，主要分别与蛋白酶 3 (PR3)-ANCA 和髓过氧化物酶 (MPO)-ANCA 相关。关于 ANCA 血管炎、PR3-ANCA MPA 和 MPO-ANCA GPA 的罕见形式，人们知之甚少。在这项队列研究中，我们详细介绍了肾小球疾病合作网络 (GDCN) 初始队列中 PR3-ANCA MPA 和 MPO-ANCA GPA 患者的临床表现和结果。通过 PR3-ANCA (n = 116) 与 MPO-ANCA (n = 173) 比较 MPA 病例中的基线临床表现、复发、终末期肾病 (ESKD) 和生存率，并通过 PR3-ANCA (n = 108) 与 MPO-ANCA (n = 43) 比较 GPA 病例中的基线临床表现、复发、终末期肾病 (ESKD) 和生存率。采用 Fisher 精确检验和 Wilcoxon 两样本检验进行比较。使用比例风险模型来评估复发、ESKD 和死亡的发展。与 MPO-ANCA MPA 患者相比，PR3-ANCA MPA 患者更年轻（53 岁 vs. 62 岁，P = 0.0007），关节受累率（56% vs. 40%，P = 0.0115）和耳鼻喉 (ENT) 受累率（44% vs. 26%，P = 0.002）更高。两个 MPA 亚群的复发、ESKD 和生存率相似。在 GPA 组中，与 PR3-ANCA GPA 患者相比，MPO-ANCA GPA 患者年龄更大（61 岁 vs. 46 岁，P = 0.0007），并且更有可能是女性（56% vs. 35%，P = 0.027）。 MPO-ANCA GPA 的另一个特点是耳鼻喉科表现（58% vs. 77%，P = 0.028）和神经系统表现（5% vs. 25%，P = 0.0029）较少发生，并且 ESKD 和死亡率增加。 PR3-ANCA MPA 和 MPO-ANCA GPA 是临床病理学上不同的 ANCA 血管炎子集，不同于 MPO-ANCA MPA 和 PR3-ANCA GPA。尽管这些差异对临床管理和结果的影响值得进一步评估，但这些结果支持在 ANCA 血管炎的诊断中包括表型诊断和 ANCA 血清型的建议。
Granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), 2 major clinicopathologic variants of antineutrophil cytoplasmic autoantibody (ANCA) vasculitides, are mostly associated with proteinase 3 (PR3)-ANCA and myeloperoxidase (MPO)-ANCA, respectively. Less is known regarding the uncommon forms of ANCA vasculitis, PR3-ANCA MPA and MPO-ANCA GPA. In this cohort study we detailed the clinical presentation and outcome of patients with PR3-ANCA MPA and MPO-ANCA GPA from the Glomerular Disease Collaborative Network (GDCN) inception cohort. Baseline clinical manifestations, relapses, end-stage kidney disease (ESKD), and survival were compared within MPA cases by PR3-ANCA (n = 116) versus MPO-ANCA (n = 173) and within GPA cases by PR3-ANCA (n = 108) versus MPO-ANCA (n = 43). Fisher’s exact test and Wilcoxon two sample test were used for comparisons. Proportional hazards models were used to evaluate the development of relapses, ESKD, and death. Patients with PR3-ANCA MPA were younger (53 years vs. 62 years, P = 0.0007) and had increased prevalence of joint involvement (56% vs. 40%, P = 0.0115) and ear, nose, and throat (ENT) involvement (44% vs. 26%, P = 0.002) than MPO-ANCA MPA. Relapses, ESKD, and survival were similar between both MPA subsets. Within the GPA group, patients with MPO-ANCA GPA were older (61 years vs. 46 years, P = 0.0007) and more likely female (56% vs. 35%, P = 0.027) than PR3-ANCA GPA patients. MPO-ANCA GPA was also characterized by less prevalent ENT manifestations (58% vs. 77%, P = 0.028) and neurologic manifestations (5% vs. 25%, P = 0.0029), and increased ESKD and mortality. PR3-ANCA MPA and MPO-ANCA GPA are clinicopathologically distinct subsets of ANCA vasculitis that differ from MPO-ANCA MPA and PR3-ANCA GPA. Although the impact of these differences on the clinical management and outcome warrants further evaluation, these results support the recommendation of including both the phenotypic diagnosis and ANCA serotype in the diagnosis of ANCA vasculitis.
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影响因子：
19.6
作者：
Hauer, HA;Bajema, IM;Hagen, EC
通讯作者：
Hagen, EC
影响因子：
27.4
作者：
Flossmann, Oliver;Berden, Annelies;Westman, Kerstin
通讯作者：
Westman, Kerstin
影响因子：
--
作者：
Holle, Julia U.;Gross, Wolfgang L.;Reinhold-Keller, Eva
通讯作者：
Reinhold-Keller, Eva
影响因子：
39.2
作者：
Hogan, SL;Falk, RJ;Nachman, PH
通讯作者：
Nachman, PH
影响因子：
13.6
作者：
Alba MA;Flores-Suárez LF;Henderson AG;Xiao H;Hu P;Nachman PH;Falk RJ;Charles Jennette J
通讯作者：
Charles Jennette J