Successful treatment of MPO-ANCA positive crescentic IgA nephropathy/IgA vasculitis with nephritis potentially triggered by a COVID-19 vaccine in a young adult female using corticosteroids, rituximab, and avacopan.

IF 1 Q4 UROLOGY & NEPHROLOGY
Ken Kaseda, Ryou Terakawa, Rena Matsui, Minoru Yasukawa, Shinichiro Asakawa, Shigeyuki Arai, Osamu Yamazaki, Yoshifuru Tamura, Ryuji Ohashi, Shigeru Shibata, Yoshihide Fujigaki
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引用次数: 0

Abstract

An 18-year-old female presented with palpable purpura nine months before her hospital admission, which first appeared 1 month after receiving a COVID-19 vaccine and recurred intermittently. One month prior to admission, she developed macrohematuria, abdominal pain, and a loss of appetite. Occult blood in urine had been noted during high school health check-ups. Upon admission, she continued to have macrohematuria, along with renal dysfunction and a nephritic urinalysis, serum myeloperoxidase-anti-neutrophil cytoplasmic antibodies (MPO-ANCA) positivity. A renal biopsy revealed crescentic glomerulonephritis with mesangial and endocapillary hypercellularity, and dominant IgA deposition and electron-dense deposits in the mesangial regions. The diagnosis was IgA nephropathy (IgAN) or IgA vasculitis with nephritis (IgAVN), with a possible overlap of MPO-ANCA-associated glomerulonephritis. Treatment began with methylprednisolone pulse therapy and prednisolone. After the diagnosis, rituximab (RTX) and avacopan were added to the regimen. Within two months, renal function, hematuria, and MPO-ANCA levels had normalized, and proteinuria was almost fully resolved by 13 months. If IgAN/IgAVN and ANCA-associated vasculitis were indeed triggered by the COVID-19 vaccination in this case, it is plausible that both conditions share similar pathologic mechanisms. This case emphasizes the need for a reliable laboratory method to detect pathogenic ANCA to guide both induction and maintenance therapy. Further investigation into the effectiveness of the ANCA-associated glomerulonephritis treatment protocol including corticosteroids, RTX, and avacopan in managing crescentic IgAN/IgAVN could offer valuable insights into improving patient care.

使用皮质类固醇、利妥昔单抗和阿瓦库潘成功治疗一名年轻成年女性MPO-ANCA阳性月牙IgA肾病/IgA血管炎伴可能由COVID-19疫苗引发的肾炎。
一名18岁女性在入院前9个月出现可触及的紫癜,首次出现在接种COVID-19疫苗1个月后,间歇性复发。入院前一个月,患者出现大量血尿、腹痛和食欲减退。在高中的健康检查中发现了尿液中的隐血。入院时,患者仍有大量血尿,并伴有肾功能不全和肾病性尿分析,血清髓过氧化物酶-抗中性粒细胞胞浆抗体(MPO-ANCA)阳性。肾活检显示月牙状肾小球肾炎伴系膜和毛细血管内细胞增多,系膜区主要有IgA沉积和电子致密沉积。诊断为IgA肾病(IgAN)或IgA血管炎合并肾炎(IgAVN),可能与mpo - anca相关的肾小球肾炎重叠。治疗开始使用甲基强的松龙脉冲疗法和强的松龙。诊断后,在治疗方案中加入利妥昔单抗(RTX)和阿瓦库潘。2个月内,肾功能、血尿和MPO-ANCA水平恢复正常,13个月时蛋白尿几乎完全消除。如果在这种情况下IgAN/IgAVN和anca相关的血管炎确实是由COVID-19疫苗接种引发的,那么这两种情况似乎具有相似的病理机制。本病例强调需要可靠的实验室方法来检测致病性ANCA,以指导诱导和维持治疗。进一步研究anca相关肾小球肾炎治疗方案的有效性,包括皮质类固醇、RTX和avacopan在管理新月形IgAN/IgAVN方面的有效性,可以为改善患者护理提供有价值的见解。
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来源期刊
CEN Case Reports
CEN Case Reports UROLOGY & NEPHROLOGY-
CiteScore
1.90
自引率
0.00%
发文量
80
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.
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