Anti-LDL Receptor-Related Protein 2 Nephropathy with Synchronous Primary Kidney Extranodal Marginal Zone Lymphoma

INTRODUCTION: Anti-LDL receptor-related protein 2 (anti-LRP2) nephropathy is a rare but progressive form of autoimmune-mediated tubulointerstitial nephritis and glomerular disease, characterized by a classic pattern of immune complex deposition in the kidney. A theoretic link between autoimmune disease and lymphoproliferative diseases exists, and therefore, in some cases autoimmune-mediated inflammation and lymphoproliferative neoplasm can co-exist in the same site. CASE PRESENTATION: An elderly man presented with 6 months of weight loss and fatigue. Subsequent workup showed an elevated serum creatinine and subnephrotic range proteinuria. Kidney biopsy was performed which revealed anti-LRP2 nephropathy with concurrent primary kidney extranodal marginal zone lymphoma. He was subsequently treated with rituximab but remains dialysis-dependent (12 months after his initial diagnosis, at time of publication of this report). CONCLUSION: We discuss the bidirectional relationship between autoimmune disease and lymphoma in the kidney, along with a brief review of the literature pertaining to these rare lesions. Our case report highlights the diagnostic difficulties faced by pathologists when encountering this entity. We also attempt to spread awareness about the co-existence of tubulointerstitial inflammation and lymphoproliferative disorder, which may be under-recognized.