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Abstract: FR-PO815

Clinical and Genetic Feature of Membranous Nephropathy in Patients with Primary Sjögren Syndrome

Session Information

Category: Glomerular Diseases

  • 1202 Glomerular Diseases: Immunology and Inflammation

Authors

  • Zou, Linfeng, Division of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, BEIJING, China
  • Zhou, Mengyu, Division of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, BEIJING, China
  • Shi, Xiaoxiao, Division of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, BEIJING, China
  • Xia, Peng, Division of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, BEIJING, China
  • Wen, Yubing, Division of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, BEIJING, China
  • Li, Chao, Division of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, BEIJING, China
  • Li, Hang, Division of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, BEIJING, China
  • Li, Xuemei, Division of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, BEIJING, China
  • Li, Xuewang, Division of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, BEIJING, China
  • Chen, Limeng, Division of Nephrology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, BEIJING, China
Background

In our previous study, membranous nephropathy (MN) was the most common pathological patterns of glomerular involvement in patients with primary Sjögren's Syndrome. In this pilot study, we try to observe the clinical features and genetic background of MN patients with primary Sjögren’s syndrome (pSS-MN).

Methods

Data from pSS-MN patients (n=73) confirmed by renal pathology were retrospectively collected from Peking Union Medical College Hospital, from Jan 30, 1993, to January 30, 2018. The serum anti-PLA2R antibody and renal tissue PLA2R antigen were tested by ELISA and immunofluorescence respectively. Idiopathic MN (iMN) patients were used as controls (n=150). Eight SNP sites, four high-risk sites for pSS and four high-risk sites for MN, as well as 3 HLA class II sites (HLA-DQA1, HLA-DQB1and HLA-DRB1) were typed by Sanger test in 23 pSS-MN patients. Genetic data of MN and normal people from Peking University First Hospital cohort and of pSS and normal people from Peking Union Medical Hospital cohort was used as control.

Results

pSS-MN patients were significantly older than iMN patients (mean age 53.3±14.1 vs 46.4±14.3, p<0.001) with predominantly female (78.1% vs 44.7%, p<0.001). Laboratory test showed significantly higher ESR (58.4±31.8 vs 35.0±26.5, p<0.001) but lower 24h urine protein (4.13±2.96 vs 6.02±5.71, p<0.001) in pSS-MN patients. There was no significant difference in eGFR and albumin level between the two groups when the biopsy was performed.
pSS-MN and iMN patients had a similar positive rate of anti-PLA2R antibody (61.8% vs 74.0%, p=0.205). In pSS-MN patients, the mutation rates of 3 SNP (linked rs35771982- rs3749117 and rs9271588) were significance higher than normal control (30.1% vs 8.7%, p<0.001; 46.8% vs 21.7%, p=0.001, respectively), without significance when compared with iMN (p=0.30) or pSS patients (p=0.13). When compared with normal control, both HLA-DRB1*1501 (p=2.6×10^-5) and the haplotype (HLA-DRB1*1501-DQA1*0102DQB1*0602, 39.13% vs 9.00%, p=9.6×10^-8) in pSS-MN patients were identified as risk alleles, but the difference was not significant between the pSS-MN and iMN patients (p=0.09).

Conclusion

pSS-MN and iMN patients shared similar clinical and genetic feature, as well as PLA2R positive ratio. HLA-DRB1*1501 was the most significant HLA allele in pSS-MN patients.

Funding

  • Government Support - Non-U.S.