Clinicopathological Features of Membranous Nephropathy Complicated by IgA Nephropathy: A Retrospective Analysis of Seven Cases.

IF 1 4区 医学 Q3 MEDICINE, GENERAL & INTERNAL British journal of hospital medicine Pub Date : 2024-10-30 Epub Date: 2024-10-29 DOI:10.12968/hmed.2024.0338
Beilei Xu, Qiuhong Bao, Wei Shen, Quxia Hong
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Abstract

Aims/Background Both membranous nephropathy (MN) and immunoglobulin A nephropathy (IgAN) are immune complex-mediated glomerular diseases, but the concurrent occurrence of these two conditions in the same patient is not common, a phenomenon that is currently not supported by clinical data in terms of treatment and prognosis. This study explores the clinical and pathological characteristics, as well as the treatment outcomes, of patients affected by MN and IgAN simultaneously. Methods The clinical data, pathological features, and diagnostic and therapeutic information of seven cases of MN complicated by IgAN, treated between December 2015 and December 2022, were retrospectively analyzed. Results Among the seven cases, there were two male and five female patients, with an average age of 57.3 ± 9.2 years. All patients presented with clinical manifestations of proteinuria and edema upon admission, with an average 24-hour urine protein of 3716.6 ± 1519.4 mg/24 h. Phospholipase A2 receptor (PLA2R) expression was detected in all seven cases, and nephrotic syndrome was clinically diagnosed in five cases. Additionally, all seven cases showed microscopic hematuria, with intermittent gross hematuria in two cases. All seven patients included in this study underwent renal biopsy. After disease staging, the patients had MN stages I-III and IgAN stages II-III. Pathological findings revealed abnormal glomerular basement membrane (GBM) and diffuse immunoglobulin G (IgG) deposition in the subepithelial space, predominantly of the IgG4 subtype. Simultaneously, there was diffuse mesangial zone deposition of immunoglobulin A (IgA) to varying degrees, co-localization of complement component C3 and IgA, and mesangial cell proliferation. Treatment strategies included angiotensin-converting enzyme inhibitors (ACEI) or angiotensin receptor blockers (ARB) in combination with steroids or immunosuppressive therapies such as tacrolimus, cyclophosphamide, and rituximab. After 2-6 months of treatment, all patients achieved complete remission with a favourable prognosis. Conclusion MN accompanied by IgAN tends to occur more frequently in middle-aged and elderly individuals, with a relatively low incidence. The latent feature of the comorbidities manifests as a form of IgAN superimposed on the background of MN. Utilizing ACEI or ARB in combination with steroids or various immunosuppressive therapies represents a potentially effective treatment strategy.

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膜性肾病并发 IgA 肾病的临床病理特征:七例病例的回顾性分析。
目的/背景 膜性肾病(MN)和免疫球蛋白 A 肾病(IgAN)都是免疫复合物介导的肾小球疾病,但这两种疾病同时出现在同一患者身上的情况并不常见,目前在治疗和预后方面还没有临床数据支持这一现象。本研究探讨了同时受 MN 和 IgAN 影响的患者的临床和病理特征以及治疗效果。方法 回顾性分析2015年12月至2022年12月期间收治的7例MN并发IgAN患者的临床资料、病理特征以及诊断和治疗信息。结果 7例患者中,男性2例,女性5例,平均年龄(57.3±9.2)岁。所有患者入院时均有蛋白尿和水肿的临床表现,平均 24 小时尿蛋白为 3716.6 ± 1519.4 mg/24 h。所有 7 例患者均检测到磷脂酶 A2 受体(PLA2R)表达,其中 5 例患者临床诊断为肾病综合征。此外,所有七例患者均出现镜下血尿,其中两例出现间歇性毛细血尿。本研究中的七名患者均接受了肾活检。经过疾病分期,患者均为 MN I-III 期和 IgAN II-III 期。病理结果显示肾小球基底膜(GBM)异常,上皮下间隙有弥漫性免疫球蛋白 G(IgG)沉积,主要为 IgG4 亚型。与此同时,免疫球蛋白 A(IgA)在不同程度上弥漫性地沉积在系膜区,补体成分 C3 和 IgA 共定位,系膜细胞增生。治疗策略包括血管紧张素转换酶抑制剂(ACEI)或血管紧张素受体阻滞剂(ARB)与类固醇或免疫抑制疗法(如他克莫司、环磷酰胺和利妥昔单抗)联合应用。经过 2-6 个月的治疗,所有患者的病情都得到了完全缓解,预后良好。结论 伴有 IgAN 的 MN 多见于中老年人,发病率相对较低。合并症的潜伏特征表现为在 MN 背景上叠加一种 IgAN。将 ACEI 或 ARB 与类固醇或各种免疫抑制剂联合使用是一种潜在的有效治疗策略。
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来源期刊
British journal of hospital medicine
British journal of hospital medicine 医学-医学:内科
CiteScore
1.50
自引率
0.00%
发文量
176
审稿时长
4-8 weeks
期刊介绍: British Journal of Hospital Medicine was established in 1966, and is still true to its origins: a monthly, peer-reviewed, multidisciplinary review journal for hospital doctors and doctors in training. The journal publishes an authoritative mix of clinical reviews, education and training updates, quality improvement projects and case reports, and book reviews from recognized leaders in the profession. The Core Training for Doctors section provides clinical information in an easily accessible format for doctors in training. British Journal of Hospital Medicine is an invaluable resource for hospital doctors at all stages of their career. The journal is indexed on Medline, CINAHL, the Sociedad Iberoamericana de Información Científica and Scopus.
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