Anti-GBM/ANCA double-positive crescentic nephritis in an elderly patient presenting with heart failure-like symptoms: A case report.

IF 2 4区 医学 Q2 MEDICINE, GENERAL & INTERNAL
Meijuan Shi, Guoqing Shi, Hongmei Liu, Yao Huang, Nan Luo, Yumei Ge, Jie Zhu
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引用次数: 0

Abstract

Rationale: Anti-glomerular basement membrane (anti-GBM) antibody disease and antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis represent the 2 leading causes of rapidly progressive glomerulonephritis (RPGN). The coexistence of these conditions is rare, typically progresses rapidly, and has a poor prognosis. Elderly patients often present with nonspecific systemic symptoms, increasing the likelihood of misdiagnosis and treatment delay.

Patient concerns: A 74-year-old woman presented with a one-month history of intermittent shortness of breath, worsening progressively over ten days, accompanied by nausea and vomiting. She denied hemoptysis, gross hematuria, rash, or arthralgia.

Diagnoses: Initial assessment suggested heart failure. However, laboratory results revealed severe renal impairment (creatinine: 1025.99 μmol/L). Renal biopsy demonstrated crescent formation in over 80% of glomeruli, with immunofluorescence showing linear immunoglobulin G deposits along glomerular capillary loops. Serological testing revealed markedly elevated anti-GBM antibody (>400 RU/mL) and MPO-ANCA (108.4 RU/mL) levels, together with a perinuclear ANCA (P-ANCA) pattern on indirect immunofluorescence, supporting the diagnosis of anti-GBM glomerulonephritis with concomitant MPO-ANCA positivity.

Interventions: Treatment included methylprednisolone pulse therapy, cyclophosphamide, and 3 plasma exchange sessions. Additional supportive measures comprised hemodialysis, antihypertensive therapy, and management of renal anemia.

Outcomes: Although the patient's uremic symptoms improved markedly, renal function did not recover. She was discharged on hemodialysis and was subsequently transitioned to maintenance peritoneal dialysis. By January 2026, the anti-GBM antibody level had decreased markedly to 4.27 RU/mL, below the positive cutoff, and MPO-ANCA had also become negative. At the last follow-up in April 2026, the patient remained dialysis-dependent and experienced recurrent hospitalizations for pneumonia, heart failure, and acute pancreatitis.

Lessons: This case emphasizes that elderly patients with anti-GBM/ANCA double-positive crescentic glomerulonephritis may initially present with nonspecific symptoms, increasing the risk of incorrect diagnosis. Although combination therapy resulted in symptomatic improvement and serological remission, renal function failed to recover and dialysis dependence persisted. Screening for anti-GBM antibody and ANCA should therefore be considered in patients with unexplained rapidly progressive deterioration of kidney function or active urinary sediment, and renal biopsy should be performed promptly when clinically indicated.

抗gbm /ANCA双阳性月牙性肾炎1例,表现为心力衰竭样症状。
理由:抗肾小球基底膜(抗gbm)抗体疾病和抗中性粒细胞胞浆抗体(ANCA)相关血管炎是快速进展性肾小球肾炎(RPGN)的两个主要原因。这些情况的共存是罕见的,通常进展迅速,预后差。老年患者经常出现非特异性全身症状,增加了误诊和治疗延误的可能性。患者关注:74岁女性,有1个月间断性呼吸短促病史,10天内逐渐加重,伴有恶心和呕吐。她否认咯血、肉眼血尿、皮疹或关节痛。诊断:初步评估提示心力衰竭。然而,实验室结果显示严重的肾功能损害(肌酐:1025.99 μmol/L)。肾活检显示超过80%的肾小球呈新月形,免疫荧光显示沿肾小球毛细血管袢呈线性免疫球蛋白G沉积。血清学检测显示抗gbm抗体(400ru /mL)和MPO-ANCA (108.4 RU/mL)水平明显升高,间接免疫荧光显示核周ANCA (P-ANCA)模式,支持抗gbm肾小球肾炎伴MPO-ANCA阳性的诊断。干预措施:治疗包括甲基强的松龙脉冲治疗、环磷酰胺和3次血浆交换。额外的支持措施包括血液透析、降压治疗和肾性贫血的管理。结果:尽管患者尿毒症症状明显改善,但肾功能未恢复。她出院进行血液透析,随后转为维持腹膜透析。到2026年1月,抗gbm抗体水平明显下降至4.27 RU/mL,低于阳性临界值,MPO-ANCA也变为阴性。在2026年4月的最后一次随访中,患者仍然依赖透析,并因肺炎、心力衰竭和急性胰腺炎反复住院。经验教训:本病例强调老年抗gbm /ANCA双阳性月牙状肾小球肾炎患者最初可能表现为非特异性症状,增加了误诊的风险。虽然联合治疗导致症状改善和血清学缓解,但肾功能未能恢复,透析依赖持续存在。因此,对于不明原因的肾功能快速进行性恶化或活动性尿沉渣患者,应考虑筛查抗gbm抗体和ANCA,并在临床指征时及时行肾活检。
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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来源期刊
Medicine
Medicine 医学-医学:内科
CiteScore
2.80
自引率
0.00%
发文量
4342
审稿时长
>12 weeks
期刊介绍: Medicine is now a fully open access journal, providing authors with a distinctive new service offering continuous publication of original research across a broad spectrum of medical scientific disciplines and sub-specialties. As an open access title, Medicine will continue to provide authors with an established, trusted platform for the publication of their work. To ensure the ongoing quality of Medicine’s content, the peer-review process will only accept content that is scientifically, technically and ethically sound, and in compliance with standard reporting guidelines.
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