Characteristics and Outcomes of NELL1 Membranous Nephropathy in Lipoic Acid Users and Nonusers

Rupali S. Avasare, Samantha Clark, Rebecca I. Spain, Raghav Wusirika, Robert Rope, Susan Gurley, Madison Stanaway, Miroslav Sekulic, Dominick Santoriello, Andrew S. Bomback, Pietro Canetta, Sitalakshmi J. Iyer, Vanderlene Kung, Vivek Charu, Megan L. Troxell, Satoru Kudose, Nicole K. Andeen

Research output: Contribution to journalArticlepeer-review

4 Scopus citations

Abstract

Introduction: Neural epidermal growth factor like 1 membranous nephropathy (NELL1 MN) is associated with various secondary etiologies. However, previous studies on the frequency of these associations and their impact on outcomes are limited. We report a large multiinstitutional series of patients with NELL1 MN with a focus on secondary associations, pathology findings, and their impact on outcome. Methods: We retrospectively reviewed clinicopathologic features of NELL1 MN from 3 institutions and analyzed clinical and histologic associations with outcome. Results: Of 70 patients, 53% were male with a median age of 66 years; median proteinuria was 5.9 g/d. NELL1 MN was associated with lipoic acid (36%), heavy nonsteroidal antiinflammatory drug (NSAID) use (27%), autoimmune disease (23%), malignancy (10% recent, 23% any), mercury exposure (1%), and 11% had no known secondary association. At median follow-up of 11 months, 72% achieved complete or partial remission. Remission rate was 91% in patients with lipoic acid-associated NELL1 MN and ≥6 months of follow-up. On multivariable analyses, patients with primary NELL1 MN (adjusted odds ratio [OR]: 19.7, P = 0.01) and increasing degree of tubular atrophy and interstitial fibrosis (IFTA) (adjusted OR 1.1, P = 0.01) were less likely to achieve any remission, whereas complete remission (CR) was associated with lipoic acid use (adjusted OR: 10.9, P = 0.04, 95% confidence interval [CI]: 1.2–100) and lesser degrees of IFTA (adjusted OR: 0.79, P = 0.16, 95% CI: 0.66–0.96). Conclusion: Our findings strengthen the association between lipoic acid and NELL1 MN. Furthermore, our findings suggest that discontinuation of lipoic acid without immunosuppression should be considered as the first-line treatment.

Original languageEnglish (US)
Pages (from-to)1379-1386
Number of pages8
JournalKidney International Reports
Volume9
Issue number5
DOIs
StatePublished - May 2024

Keywords

  • NELL1
  • NSAID
  • drug-induced kidney disease
  • glomerular disease
  • lipoic acid
  • membranous nephropathy

ASJC Scopus subject areas

  • Nephrology

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