Clinical and pathological characteristics of NELL-1-positive membranous nephropathy: a case series study
Year: 2025
Type: article
Study design: Observational Study
Abstract: Objective: This study aimed to comprehensively characterize the clinical and pathological features, treatment strategies, and outcomes of patients with neural epidermal growth factor-like 1 protein (NELL-1)-positive membranous nephropathy (MN). Methods: We retrospectively analyzed non-systemic lupus erythematosus (SLE) MN patients diagnosed between January 2010 and August 2021 at Cangzhou Central Hospital, China. Inclusion required negative PLA2R and THSD7A staining and available paraffin-embedded renal tissue. Clinical, laboratory, pathological, treatment, and outcome data were collected and analyzed descriptively. Results: Among 531 non-SLE MN cases screened, 23 patients (13 males, 10 females; mean age 49.4 ± 12.0 years) were identified as NELL-1(+), PLA2R(-), and THSD7A(-). Hypertension was present in 26.1%, and nephrotic syndrome in 60.9% of patients. Bright granular NELL-1 deposits along glomerular capillary loops were observed in all cases, with segmental or incomplete global distribution seen in 65.2%. IgG4 and IgG1 were the predominant immunoglobulin subclasses (82.6 and 78.2%, respectively). Over a median follow-up of 56 months, 73.9% received immunosuppressive therapy, while 26.1% were managed with supportive treatment alone. The overall remission rate was 87.0% (73.9% complete, 13.0% partial), with only two patients experiencing transient renal function decline and none progressing to end-stage renal disease. Notably, 26.1% of patients developed malignancies, most commonly lung cancer. In patients with malignancy, tumor resection and supportive therapy alone frequently led to remission of MN. Conclusion: NELL-1-positive MN is characterized by high rates of IgG1 and IgG4 deposition and generally favorable responses to therapy. A substantial proportion of patients were diagnosed with malignancy, highlighting the importance of cancer screening in this population. Timely identification and management of underlying malignancy may contribute to improved renal outcomes.
Source: Frontiers in Medicine
Authors: Kang Li, Zheng Liang, Xiaojing Liu, Shuyi Cao
Institution: Cangzhou Central Hospital
Language: English
Subfield: Nephrology
Field: Medicine
Domain: Health Sciences
Open Access status: gold
Estimated APC paid: $3,801
OpenAlex ID: W4413998684