Cozzo Domenico, Orlando Francesca, Bruno Mariolina, Ogna Adam, Forni Ogna Valentina
Servizio di nefrologia, EOC Ospedale "La Carità", Locarno, Switzerland.
Servizio di medicina interna, EOC Ospedale "La Carità", Locarno, Switzerland.
J Nephrol. 2025 Mar;38(2):343-352. doi: 10.1007/s40620-024-02084-6. Epub 2024 Oct 1.
Paraneoplastic minimal change disease (MCD) has been associated with hematological malignancies, whereas solid malignancies are commonly associated with membranous glomerulonephritis. In this systematic review of the literature, we describe the clinical features, treatment and outcome of MCD associated with solid neoplasms.
We performed a systematic review of the MEDLINE, COCHRANE, EMBASE and SCOPUS databases, including case reports of adult patients with biopsy-proven MCD and solid malignancy, without language or time restrictions.
Sixty-seven papers were included, presenting 86 cases with a mean age of 57.8 ± 14.7 years; 41.0% were women. Nephrotic syndrome was the initial presentation in 96.2% of patients; 67.2% had kidney function impairment, and 21.2% required kidney replacement therapy. The most frequent malignancies were malignant thymoma (34.9%), kidney (14.0%), lung (12.8%), and gastrointestinal tumors (12.8%). In 40.7% of cases, the neoplasm diagnosis preceded MCD by 33.8 ± 46.1 months, while in 31.4%, it followed diagnosis of MCD by 12.4 ± 22.6 months. In 27.9%, the neoplasm and kidney disease were diagnosed simultaneously. Immunosuppressive therapy was started in 79.1% of cases and tumor-specific treatment in 83.7%. Remission of MCD was achieved in 80.2% of patients: 38.2% responded to immunosuppressive treatment alone and 29.6% to oncological treatment alone.
The association between MCD and solid neoplasms is well-documented. Immunosuppressive therapy alone induced nephrotic syndrome remission in over one-third of cases; most others responded to tumor-specific treatment. Solid tumor screening should be considered in MCD independently of the steroid response, though more data on solid tumor-associated MCD prevalence are needed for a definitive statement.
CRD42024521854.
副肿瘤性微小病变病（MCD）与血液系统恶性肿瘤有关，而实体恶性肿瘤通常与膜性肾小球肾炎有关。在本系统文献综述中，我们描述了与实体肿瘤相关的MCD的临床特征、治疗及预后。
我们对MEDLINE、COCHRANE、EMBASE和SCOPUS数据库进行了系统综述，纳入经活检证实为MCD且患有实体恶性肿瘤的成年患者的病例报告，无语言或时间限制。
纳入67篇论文，共86例病例，平均年龄57.8±14.7岁；41.0%为女性。96.2%的患者最初表现为肾病综合征；67.2%有肾功能损害，21.2%需要肾脏替代治疗。最常见的恶性肿瘤是恶性胸腺瘤（34.9%）、肾脏肿瘤（14.0%）、肺癌（12.8%）和胃肠道肿瘤（12.8%）。40.7%的病例中，肿瘤诊断先于MCD 33.8±46.1个月，而31.4%的病例中，肿瘤诊断在MCD诊断后12.4±22.6个月。27.9%的病例中，肿瘤和肾病同时被诊断。79.1%的病例开始了免疫抑制治疗，83.7%开始了肿瘤特异性治疗。80.2%的患者实现了MCD缓解：38.2%仅对免疫抑制治疗有反应，29.6%仅对肿瘤治疗有反应。
MCD与实体肿瘤之间的关联有充分文献记载。仅免疫抑制治疗在超过三分之一的病例中诱导了肾病综合征缓解；大多数其他病例对肿瘤特异性治疗有反应。对于MCD患者，无论类固醇反应如何，都应考虑进行实体肿瘤筛查，不过需要更多关于实体肿瘤相关MCD患病率的数据才能得出明确结论。
PROSPERO试验注册号：CRD42024521854。
