Concurrent presentation of IgG4-related tubulointerstitial nephritis and ANCA MPO crescentic glomerulonephritis.

Clinical Nephrology. Case Studies Pub Date : 2022-07-04 eCollection Date: 2022-01-01 DOI:10.5414/CNCS110852
Henry H L Wu, Claire C Y Wang, Alexander Woywodt, Arvind Ponnusamy
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引用次数: 2

Abstract

Concurrent IgG4-related tubulointerstitial nephritis and anti-neutrophil cytoplasmic antibodies (ANCA) myeloperoxidase (MPO) crescentic glomerulonephritis is an uncommon scenario, and the link between the two conditions, if any, is incompletely understood. We report the case of a 58-year-old woman who presented with a 2-month history of malaise and joint pain and was found to have acute kidney injury and hemato-proteinuria. Initial immunological tests revealed positive anti-neutrophil cytoplasmic antibodies with a peri-nuclear pattern (pANCA). An enzyme-linked immunoassay (ELISA) for anti-MPO antibodies was also positive, leading to a tentative diagnosis of ANCA-associated small vessel vasculitis with renal involvement. Steroid treatment was commenced, and an urgent kidney biopsy was performed. This showed crescentic glomerulonephritis, but also demonstrated concurrent tubulointerstitial nephritis with a dominance of IgG4-producing plasma cells. Serum IgG4 levels were also elevated. The patient was initially treated with intravenous cyclophosphamide and steroids and then switched to rituximab. When last seen, she was well after 1 dose of rituximab, with kidney function, inflammatory parameters, and serum IgG4 levels returning to normal levels. The concurrent presentation of ANCA-associated vasculitis and IgG4 renal disease is rare with only few cases reported in the literature. More work is needed to understand pathophysiology, outcomes, and management options for this complex scenario.

Abstract Image

Abstract Image

同时出现igg4相关的小管间质性肾炎和ANCA MPO月牙状肾小球肾炎。
同时发生igg4相关的小管间质性肾炎和抗中性粒细胞胞浆抗体(ANCA)髓过氧化物酶(MPO)月牙状肾小球肾炎是一种罕见的情况,如果有的话,这两种情况之间的联系尚不完全清楚。我们报告一个58岁的妇女谁提出了2个月的病史不适和关节疼痛,被发现有急性肾损伤和血蛋白尿。最初的免疫学试验显示阳性的抗中性粒细胞细胞质抗体与核周围模式(pANCA)。抗mpo抗体的酶联免疫测定(ELISA)也呈阳性,初步诊断为anca相关的小血管炎伴肾脏受累。开始类固醇治疗,并进行了紧急肾活检。这表现为新月形肾小球肾炎,但也表现为并发的小管间质肾炎,并以产生igg4的浆细胞为主。血清IgG4水平也升高。患者最初接受静脉注射环磷酰胺和类固醇治疗,然后改用利妥昔单抗。最后一次就诊时,患者在服用1剂利妥昔单抗后情况良好,肾功能、炎症参数和血清IgG4水平恢复正常。anca相关的血管炎和IgG4肾病同时出现是罕见的,文献中只有少数病例报道。需要更多的工作来了解这种复杂情况的病理生理学、结果和管理选择。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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