NELL1-Associated Membranous Nephropathy in a Patient with Papillary Thyroid Carcinoma: A Case Report and Literature Review.

Glomerular diseases Pub Date : 2025-11-12 eCollection Date: 2025-01-01 DOI:10.1159/000549351
Basheer Ahamad Kummangal, Oliver Padgett, Lisa Kumasaka, Nicole Andeen, Rupali S Avasare
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Abstract

Introduction: Membranous nephropathy (MN) is a leading cause of nephrotic syndrome in adults. NELL1 accounts for approximately 10% of MN cases and is emerging as a distinct subtype often linked to secondary conditions, such as malignancies and drugs. Here, we describe a case of MN in a patient with metastatic papillary thyroid carcinoma.

Case presentation: A 78-year-old man with metastatic papillary thyroid carcinoma and a distant history of treated syphilis presented with nephrotic syndrome. A renal biopsy revealed segmental MN with positive NELL1 staining, while the thyroid carcinoma tissue also demonstrated NELL1 staining at weak to moderate intensity. The patient was managed conservatively without immunosuppression due to the lack of oncologic treatment options and syphilis was treated with doxycycline due to positive serologies, though active infection was not suspected. Follow-up showed stabilization of renal function and marked reduction in proteinuria.

Conclusion: NELL1-positive MN is increasingly recognized for its association with malignancy. The patient achieved partial remission with supportive, non-immunosuppressive therapy. Clinicians should maintain a high index of suspicion for secondary conditions when NELL1-positive MN is encountered. This case report reviews secondary NELL1 associations and underscores the diagnostic challenges in MN when multiple potential secondary causes exist.

Abstract Image

Abstract Image

甲状腺乳头状癌患者nell1相关膜性肾病1例报告及文献复习。
膜性肾病(MN)是导致成人肾病综合征的主要原因。NELL1约占MN病例的10%,并且正在成为一种独特的亚型,通常与继发性疾病(如恶性肿瘤和药物)相关。在这里,我们描述一个转移性甲状腺乳头状癌患者的MN病例。病例介绍:一名78岁男性转移性甲状腺乳头状癌和长期治疗史梅毒呈现肾病综合征。肾活检显示部分MN有NELL1阳性染色,而甲状腺癌组织也显示弱至中等强度的NELL1染色。由于缺乏肿瘤治疗方案,患者接受了保守治疗,没有免疫抑制,由于血清学阳性,梅毒接受了强力霉素治疗,尽管没有怀疑活动性感染。随访显示肾功能稳定,蛋白尿明显减少。结论:nell1阳性MN越来越被认为与恶性肿瘤有关。患者通过支持性非免疫抑制治疗获得部分缓解。当遇到nell1阳性MN时,临床医生应保持对继发性疾病的高度怀疑。本病例报告回顾了继发性NELL1的关联,并强调了当存在多种潜在继发性原因时MN的诊断挑战。
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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