Renal-limited vasculitis with elevated levels of multiple antibodies
Renal-limited vasculitis with elevated levels of multiple antibodies
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DOI：
10.1007/s13730-017-0248-3
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发表时间：
2017-05-01
期刊：
影响因子：
1
通讯作者：
Yanagita, Motoko
中科院分区：
文献类型：
--
作者：
Sato, Noriaki;Yokoi, Hideki;Yanagita, Motoko
肾局限性血管炎（RLV）是一种抗中性粒细胞胞浆抗体（ANCA）相关的血管炎，表现为新月体肾小球肾炎，无其他器官受累。尽管几项研究报道了蛋白酶3（PR 3）-ANCA和髓过氧化物酶（MPO）-ANCA或ANCA和抗肾小球基底膜（GBM）抗体双重阳性的新月体肾小球肾炎患者，但所有三种抗体阳性的患者，即，三重阳性患者，很少报告。我们在此报告一例男性少免疫型新月体肾小球肾炎阳性MPO-ANCA，PR 3-ANCA，抗GBM抗体。肾活检明确诊断为RLV伴少免疫型新月体肾炎。荧光免疫染色显示IgG在GBM上没有线性沉积，表明不涉及抗GBM相关疾病。强化治疗，包括泼尼松龙，血浆置换，静脉注射环磷酰胺，是有效的。我们报告的情况下，三阳性患者新月体肾炎，谁是成功地治疗糖皮质激素，血浆置换和环磷酰胺，这表明，治疗RLV的血清学三抗体阳性的患者在没有线性IgG沉积可以受益于血浆置换和主要诱导缓解微观多血管炎的联合治疗方案。
Renal-limited vasculitis (RLV) is a type of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis that presents with crescentic glomerulonephritis with no other organ involvement. Although several studies reported patients with crescentic glomerulonephritis who were dual positive for proteinase 3 (PR3)-ANCA and myeloperoxidase (MPO)-ANCA or ANCA and anti-glomerular basement membrane (GBM) antibody, patients positive for all three antibodies, i.e., triple-positive patients, were rarely reported. We herein report the case of a male with pauci-immune type crescentic glomerulonephritis positive for MPO-ANCA, PR3-ANCA, and anti-GBM antibody. Renal biopsy led to the definitive diagnosis of RLV with pauci-immune-type crescentic glomerulonephritis. Fluorescence immunostaining showed no linear deposition of IgG on GBM, indicating no involvement of anti-GBM associated diseases. Intensive therapy, including prednisolone, plasma exchange, and intravenous cyclophosphamide, was effective. We report the case of triple-positive patient with crescentic glomerulonephritis, who was successfully treated with glucocorticoid, plasma exchange, and cyclophosphamide, suggesting that treatment for RLV in the patient with serological triple antibodies positivity in the absence of linear IgG deposition could benefit from the combination therapy regimen for plasma exchange and primary induction of remission against microscopic polyangiitis.