Malignancy-associated membranous nephropathy: focus on diagnosis and treatment
Malignancy-associated membranous nephropathy: focus on diagnosis and treatment
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DOI：
10.1007/s40620-023-01751-4
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发表时间：
2023-09-15
影响因子：
3.4
通讯作者：
Qin，Yan
中科院分区：
文献类型：
--
作者：
Ai，SanXi;Yan，XiWei;Qin，Yan
背景恶性相关膜性肾病的临床病理特征已有报道，但诊断和治疗信息有限。方法对2012年6月至2021年10月在中国三级医院就诊的恶性相关膜性肾病患者进行回顾性分析。与特发性膜性肾病相比，恶性膜性肾病患者年龄较大，较少出现肾小球磷脂酶A2受体阳性(37.9%比85.0%)和以IgG4为主的沉积(66.7%比95.0%)。在膜性肾病的诊断中，67%(28/42)的患者恶性肿瘤未知，只能通过肿瘤筛查才能发现。在19名同时被诊断为癌症和活检证实的膜性肾病的患者中，15名最初只接受了抗癌治疗。10例肿瘤缓解的患者中有6例膜性肾病得到缓解，而5例未缓解的患者中无一例缓解，提示两者之间存在因果关系。部分癌症缓解后持续或复发的膜性肾病患者，经免疫抑制治疗后，膜性肾病得到缓解。中位随访24个月，25%(10/40)的患者死亡，主要死于肿瘤。结论肿瘤筛查在膜性肾病患者中非常重要，特别是在老年患者和磷脂酶A2受体阴性或非IgG4沉积的患者中。在某些病例中，肿瘤缓解后可观察到膜性肾病的缓解。如果膜性肾病在癌症缓解后没有缓解，可以考虑免疫抑制治疗。
BackgroundThe clinicopathological features of malignancy-associated membranous nephropathy have been described previously, but information about diagnosis and treatment remains limited.MethodsPatients with malignancy-associated membranous nephropathy in a tertiary hospital in China between June 2012 and October 2021 were retrospectively reviewed.ResultsForty-two patients with malignancy-associated membranous nephropathy were identified. Compared to patients with idiopathic membranous nephropathy, patients with malignancy-associated membranous nephropathy were older and less frequently showed glomerular phospholipase A2 receptor staining (37.9% vs 85.0%) and IgG4 predominant deposition (66.7% vs 95.0%). At diagnosis of membranous nephropathy, the malignancy was unknown in 67% (28/42) of patients and was detected only by tumor screening. Among the 19 patients with concurrent diagnosis of cancer and biopsy-proven membranous nephropathy, 15 received anticancer treatment alone initially. Six of the 10 patients who attained cancer remission achieved remission of membranous nephropathy, while none of the 5 patients without remission of cancer did, suggesting a causal relationship between the two diseases. Some patients with persistent or relapsing membranous nephropathy following cancer remission achieved remission of membranous nephropathy after immunosuppressive therapy. Over a median follow-up of 24 months, 25% (10/40) of patients died, mainly due to neoplasia.ConclusionsTumor screening is important in patients with membranous nephropathy, especially in elderly patients and patients with negative phospholipase A2 receptor or non-IgG4 predominant deposition. Remission of membranous nephropathy can be observed following remission of cancer in some cases. Immunosuppressive therapy may be considered if membranous nephropathy does not remit after remission of cancer.Graphical abstract