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Abstract: TH-PO629

Clinical Outcome of NELL1-Associated Membranous Nephropathy

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Ramachandran, Raja, Post Graduate Institute of Medical Education and Research, Chandigarh, Chandigarh, India
  • Trivedi, Mayuri, Lokmanya Tilak Municipal General Hospital and Lokmanya Tilak Municipal Medical College, Mumbai, Maharashtra, India
  • Duseja, Ritambhra Nada, Post Graduate Institute of Medical Education and Research, Chandigarh, Chandigarh, India
  • Kumar, Vinod, Post Graduate Institute of Medical Education and Research, Chandigarh, Chandigarh, India
  • Kohli, Harbir Singh, Post Graduate Institute of Medical Education and Research, Chandigarh, Chandigarh, India
Background

Neural epidermal growth factor-like 1 -associated membranous nephropathy (NELL1 MN) is related to multiple secondary causes, including malignancy and intake of complementary and alternative medicines. The association of CAM intake to NELL1 MN is well-reported in India. However, the outcome of the NELL1 MN remains to be determined.

Methods

We analysed retrospectively clinicopathologic features of NELL1 MN and compared them to non-M-type Phospholipase A2 receptor (PLA2R)/NELL1 MN from our PGIMER-primary MN registry and LTMC, Mumbai. We express the data as numbers, percentages, mean ± standard deviation (range) for normally distributed variables and median and interquartile range, otherwise.

Results

A total of 21 patients had NELL1 MN; the median age, proteinuria, creatinine, and albumin were 37 (26.5,55.5) years, 6.7(3.9,10.5) g/day, 1 (0.7,1.4) mg/dl and 2.4 (1.9,2.7) g/dl, respectively. 14(67%) patients received a modified Ponticelli regimen. For comparison, we included 21 consecutive patients of non-PLA2R/NELL1 MN. The median age, proteinuria, creatinine and albumin were 41 (30,54) years, 5.3 (2.7,8.7) g/day, 1 (0.7,1.3) mg/dl and 2.4 (1.9,3.0) g/dl, respectively. 14 (67%) had a history of CAM intake in the NELL1 MN group, which was higher than the non-PLA2R/NELL1 MN group (p<0.001); the malignancy work-up was negative in all the cases. The requirement for immunosuppressive therapy was higher in the NELL1 MN group compared to the non-PLA2R/NELL1 MN group (p=0.04). In the NELL1 MN group, we lost two patients to follow-up after a median follow-up of 18 (10,28) months; 15 (79%) patients achieved remission. Two patients died due to infectious complications. 78% and 65% of the patients in the NELL1 and non-PLA2R/NELL1 MN achieved remission (p=0.48).

Conclusion

The present report highlights the association of NELL1 MN with CAM intake compared to non-PLA2R/NELL1 MN. Three-fourths of the NELL1 MN responded to in-sighting drug withdrawal/ immunosuppressive therapy.

Funding

  • Government Support – Non-U.S.