Differences in the Clinicopathological Features of Membranous Nephropathy Associated with NELL-1 Versus PLA2R -A Single Center Experience

Abstract

Background: The present study discusses changing clinicopathological profile in Membranous Nephropathy(MN). MN is a disease classically presenting with nephrotic range proteinuria along with the presence of microscopic sub-epithelial deposits in glomeruli [1]. With the discovery of novel antigens like THSD7A (thrombospondin type 1 domain containing 7A), NELL-1 (neural epidermal growth factor-like 1 protein) classification of patients into primary (associated with phospholipase A-2 receptor antibody) and secondary membranous nephropathy has become difficult [2]. This study aims to throw light on the clinicopathological profile of patients of MN associated with NELL-1 deposits. Methodology: Data of 52 patients with MN was reviewed and patients were followed up for 1-year post-diagnosis for the remission and development of signs or symptoms suggestive of malignancy. All the patients were given standard care as per KDIGO. Results: 7 patients came out to be MN with NELL-1 deposition. However, a raised association of malignancy among NELL-1 positive patients was not found. Conclusion: We conclude that NELL-1 positivity is frequently seen in the elderly age group. Patients with NELL-1 deposition have similar clinical profiles as compared to patients with PLA2R-positive patients. Moreover, the patients with NELL-1 deposits are treatment-resistant as compared to patients with PLA2R deposits. No increased incidence of malignancy was found amongst patients with NELL-1 deposits.