Department of Clinical Medicine, Internal Medicine, Zhejiang Chinese Medical University, Hangzhou, Zhejiang, China.
Department of Renal Medicine, Tiantai County People's Hospital, Taizhou, Zhejiang, China.
Br J Hosp Med (Lond). 2024 Oct 30;85(10):1-13. doi: 10.12968/hmed.2024.0338. Epub 2024 Oct 29.
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. 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. 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. 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.
膜性肾病 (MN) 和免疫球蛋白 A 肾病 (IgAN) 均为免疫复合物介导的肾小球疾病,但同一患者同时发生这两种疾病并不常见,目前尚无临床数据支持这两种疾病在治疗和预后方面的同时存在。本研究旨在探讨 MN 合并 IgAN 患者的临床和病理特征,以及治疗结局。 回顾性分析了 2015 年 12 月至 2022 年 12 月期间收治的 7 例 MN 合并 IgAN 患者的临床资料、病理特征、诊断和治疗信息。 7 例患者中,男 2 例,女 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±1519.4mg/24h。7 例患者均检测到磷脂酶 A2 受体(PLA2R)表达,5 例患者临床诊断为肾病综合征。此外,7 例患者均有镜下血尿,2 例患者有间歇性肉眼血尿。所有纳入研究的 7 例患者均行肾活检。疾病分期后,患者的 MN 分期为 I-III 期,IgAN 分期为 II-III 期。病理检查显示肾小球基底膜(GBM)异常和上皮下空间弥漫性 IgG 沉积,以 IgG4 亚型为主。同时,IgA 不同程度弥漫性系膜区沉积,补体成分 C3 与 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)联合激素或他克莫司、环磷酰胺、利妥昔单抗等免疫抑制剂治疗。治疗 2-6 个月后,所有患者均完全缓解,预后良好。 MN 合并 IgAN 多见于中老年人,发病率相对较低。合并症的潜在特征表现为 IgAN 叠加在 MN 背景下的一种形式。ACEI 或 ARB 联合激素或各种免疫抑制剂治疗可能是一种有效的治疗策略。