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抗中性粒细胞胞浆抗体相关性血管炎与肾小球免疫沉积的病例报告

A case report of antineutrophil cytoplasmic antibody-associated vasculitis and glomerular immune depositions.

作者信息

Kaynar K, Güvercin B, Demir S, Mungan S, Çifçi E T

机构信息

Department of Nephrology, Faculty of Medicine, Karadeniz Technical University, Trabzon, Turkey.

Department of Pathology, Faculty of Medicine, Karadeniz Technical University, Trabzon, Turkey.

出版信息

Hippokratia. 2022 Apr-Jun;26(2):86-88.

Abstract

BACKGROUND

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a systemic disease leading to renal complications of pauci-immune focal and segmental necrotizing crescentic glomerulonephritis (PI-NCGN).

CASE DESCRIPTION

We present a 57-year-old female patient with rapidly progressive glomerulonephritis, multiple systemic infections [candidiasis and severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2)], severe weight loss, arthralgia, positive myeloperoxidase ANCA, acute deterioration of cardiac function and NCGN with heavy deposition of immunoglobulin (Ig) A and complement 3 (C3) in kidney biopsy. After two months of follow-up and appropriate treatments [methylprednisolone (60 mg/day), cyclophosphamide (15 mg/kg)], our patient recovered from multiple life-threatening infections, including candidiasis treated by fluconazole and SARS-CoV-2 treated by methylprednisolone and acute cardiac failure. In addition, she was saved from dialysis despite all poor prognostic factors.

CONCLUSION

AAV might lead to immune complex deposition in kidneys due to different pathogenetic mechanisms like complement activation and immune complex formation, apart from losing tolerance to neutrophil proteins. HIPPOKRATIA 2022, 26 (2):86-88.

摘要

背景

抗中性粒细胞胞浆抗体(ANCA)相关性血管炎(AAV)是一种全身性疾病,可导致寡免疫性局灶节段性坏死性新月体性肾小球肾炎(PI-NCGN)的肾脏并发症。

病例描述

我们报告一名57岁女性患者,患有快速进展性肾小球肾炎、多种全身感染(念珠菌病和严重急性呼吸综合征冠状病毒2 [SARS-CoV-2])、严重体重减轻、关节痛、髓过氧化物酶ANCA阳性、心功能急性恶化以及肾活检显示免疫球蛋白(Ig)A和补体3(C3)大量沉积的NCGN。经过两个月的随访和适当治疗[甲泼尼龙(60毫克/天)、环磷酰胺(15毫克/千克)],我们的患者从多种危及生命的感染中康复,包括用氟康唑治疗的念珠菌病和用甲泼尼龙治疗的SARS-CoV-2以及急性心力衰竭。此外,尽管存在所有不良预后因素,她仍避免了透析。

结论

除了对中性粒细胞蛋白失去耐受性外,AAV可能由于补体激活和免疫复合物形成等不同致病机制导致免疫复合物在肾脏中沉积。《希波克拉底》2022年,26(2):86 - 88。

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