{"title":"一例肾局限性髓过氧化物酶抗中性粒细胞胞浆抗体阳性血管炎患者接受阿伐潘单药维持治疗。","authors":"Yoshifumi Ubara, Yuki Oba, Shigekazu Kurihara, Akinari Sekine, Masayuki Yamanouchi, Eiko Hasegawa, Tatsuya Suwabe, Kei Kono, Kenichi Ohashi, Takehiko Wada, Naoki Sawa","doi":"10.1007/s13730-024-00910-1","DOIUrl":null,"url":null,"abstract":"<p><p>A 76-year-old woman was admitted with progressive renal function decline. A kidney biopsy was performed because of myeloperoxidase anti-neutrophil cytoplasmic antibody (ANCA; 333 IU/mL), proteinuria (1.21 g/d), and urinary erythrocyte sediment (10-19/high-power field). Renal-limited ANCA-positive vasculitis with pauci-immune necrotizing crescentic glomerulonephritis (ANCA-associated vasculitis, AAV) was diagnosed. Glucocorticoid therapy was started, and the patient responded well. About 1 year later, avacopan treatment was started and glucocorticoid therapy was discontinued. Avacopan did not normalize ANCA levels and did not make urinary findings negative. However, further progression of renal function decline is prevented. Factors attributed to the development of AAV in this case were investigated; AAV developed after the second dose of the COVID-19 vaccine and ANCA levels re-elevated after the fifth dose. This suggests that the COVID-19 vaccine may have contributed to the development of AAV in this elderly patient. Avacopan monotherapy has been shown to be effective as maintenance therapy to control the progression of renal failure although not sufficient for complete remission of AAV.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":null,"pages":null},"PeriodicalIF":1.0000,"publicationDate":"2024-07-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"A case of renal limited myeloperoxidase anti-neutrophil cytoplasmic antibody-positive vasculitis treated with maintenance avacopan monotherapy.\",\"authors\":\"Yoshifumi Ubara, Yuki Oba, Shigekazu Kurihara, Akinari Sekine, Masayuki Yamanouchi, Eiko Hasegawa, Tatsuya Suwabe, Kei Kono, Kenichi Ohashi, Takehiko Wada, Naoki Sawa\",\"doi\":\"10.1007/s13730-024-00910-1\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>A 76-year-old woman was admitted with progressive renal function decline. A kidney biopsy was performed because of myeloperoxidase anti-neutrophil cytoplasmic antibody (ANCA; 333 IU/mL), proteinuria (1.21 g/d), and urinary erythrocyte sediment (10-19/high-power field). Renal-limited ANCA-positive vasculitis with pauci-immune necrotizing crescentic glomerulonephritis (ANCA-associated vasculitis, AAV) was diagnosed. Glucocorticoid therapy was started, and the patient responded well. About 1 year later, avacopan treatment was started and glucocorticoid therapy was discontinued. Avacopan did not normalize ANCA levels and did not make urinary findings negative. However, further progression of renal function decline is prevented. Factors attributed to the development of AAV in this case were investigated; AAV developed after the second dose of the COVID-19 vaccine and ANCA levels re-elevated after the fifth dose. This suggests that the COVID-19 vaccine may have contributed to the development of AAV in this elderly patient. Avacopan monotherapy has been shown to be effective as maintenance therapy to control the progression of renal failure although not sufficient for complete remission of AAV.</p>\",\"PeriodicalId\":9697,\"journal\":{\"name\":\"CEN Case Reports\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":1.0000,\"publicationDate\":\"2024-07-02\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"CEN Case Reports\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.1007/s13730-024-00910-1\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q4\",\"JCRName\":\"UROLOGY & NEPHROLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"CEN Case Reports","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1007/s13730-024-00910-1","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"UROLOGY & NEPHROLOGY","Score":null,"Total":0}
引用次数: 0
摘要
一名 76 岁的妇女因肾功能逐渐衰退而入院。由于出现髓过氧化物酶抗中性粒细胞胞浆抗体(ANCA;333 IU/mL)、蛋白尿(1.21 g/d)和尿红细胞沉淀(10-19/高倍视野),患者接受了肾活检。诊断为肾局限性 ANCA 阳性血管炎伴贫免疫坏死性新月体肾小球肾炎(ANCA 相关性血管炎,AAV)。开始使用糖皮质激素治疗,患者反应良好。约 1 年后,患者开始接受阿瓦柯潘治疗,糖皮质激素治疗停止。阿伐潘并没有使 ANCA 水平恢复正常,也没有使尿检结果呈阴性。但是,肾功能衰退的进一步发展得到了阻止。对该病例中出现 AAV 的因素进行了调查;COVID-19 疫苗第二剂后出现 AAV,第五剂后 ANCA 水平再次升高。这表明,COVID-19 疫苗可能是导致这名老年患者出现 AAV 的原因之一。阿伐潘单药治疗已被证明可有效控制肾功能衰竭的进展,但不足以使 AAV 完全缓解。
A case of renal limited myeloperoxidase anti-neutrophil cytoplasmic antibody-positive vasculitis treated with maintenance avacopan monotherapy.
A 76-year-old woman was admitted with progressive renal function decline. A kidney biopsy was performed because of myeloperoxidase anti-neutrophil cytoplasmic antibody (ANCA; 333 IU/mL), proteinuria (1.21 g/d), and urinary erythrocyte sediment (10-19/high-power field). Renal-limited ANCA-positive vasculitis with pauci-immune necrotizing crescentic glomerulonephritis (ANCA-associated vasculitis, AAV) was diagnosed. Glucocorticoid therapy was started, and the patient responded well. About 1 year later, avacopan treatment was started and glucocorticoid therapy was discontinued. Avacopan did not normalize ANCA levels and did not make urinary findings negative. However, further progression of renal function decline is prevented. Factors attributed to the development of AAV in this case were investigated; AAV developed after the second dose of the COVID-19 vaccine and ANCA levels re-elevated after the fifth dose. This suggests that the COVID-19 vaccine may have contributed to the development of AAV in this elderly patient. Avacopan monotherapy has been shown to be effective as maintenance therapy to control the progression of renal failure although not sufficient for complete remission of AAV.
期刊介绍:
Clinical and Experimental Nephrology (CEN) Case Reports is a peer-reviewed online-only journal, officially published biannually by the Japanese Society of Nephrology (JSN). The journal publishes original case reports in nephrology and related areas. The purpose of CEN Case Reports is to provide clinicians and researchers with a forum in which to disseminate their personal experience to a wide readership and to review interesting cases encountered by colleagues all over the world, from whom contributions are welcomed.