Background: Paraneoplastic glomerular diseases are triggered by substances secreted by tumor cells, such as tumor antigens, rather than direct tumor invasion. Summary: These conditions frequently manifest as glomerular disorders, particularly in the elderly, with membranous nephropathy being the most observed lesion. They often present with proteinuria, hematuria, and/or varying levels of kidney dysfunction. In some cases, the initial presentation may precede the diagnosis of malignancy and can be indistinguishable from the idiopathic glomerulopathies, requiring a high level of clinical suspicion to accurately identify a paraneoplastic origin. Although the exact pathophysiologic mechanisms underlying paraneoplastic glomerulopathy are not fully understood, they are thought to involve an immune-mediated response to tumor antigens in most cases. Key Message: Recognizing paraneoplastic glomerulopathies is of significant clinical importance as their management is distinct and has substantial implications for the treatment of the associated malignancy.

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