NELL-1 is a novel target antigen in Chinese LMN patients

Abstract

Abstract Introduction: Neural Epidermal Growth Factor Like 1 (NELL-1) has been found in some secondary membranous nephropathy (sMN) patients. The aim of this study is to describe the clinical and pathological features of Chinese patients with NELL-1-associated sMN. Methods We conducted a cohort study at two Chinese medical centers and 101 sMN patients were enrolled. Our study re-stained immunofluorescent(IF) PLA2R, THSD7A, and NELL-1 podocyte antigens in their kidney tissue paraffin samples. The clinicopathological features of NELL-1 associated sMN in our cohort were described and analyzed in detail. Result 14 (13.8%) were PLA2R-MN, 5 (4.9%) were NELL-1-MN, and 82 (81.8%) tested negative for all 3 target antigens, with no cases of THSD7A-related MN found in this sMN cohort. Notably, all 5 patients with NELL1-related MN had a history of systemic lupus erythematosus (SLE), and 4 of them exhibited renal biopsy pathology consistent with lupus IV + V membranous nephropathy (LMN). 80% of patients with NELL-1 associated sMN were female, with a median age of 41 years. During the follow-up period, two NELL-1 LMN with baseline renal function in CKD4 entered ESRD within 1 year. Combined with the data from Anzhen Hospital NELL-1 MN study we supposed that NELL-1 might be the second most prevalent target antigen for membranous nephropathy in Chinese patients. Conclusion NELL-1 maybe a novel target antigen in Chinese LMN patients. Young female LMN patients suggested to be screened for the target antigen of NELL1,which may facilitating early diagnosis and efficacy monitoring of sMN.