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Abstract: TH-PO637

Clinical Manifestation and Prognosis of Membranous Nephropathy in Patients with Primary Sjögren Syndrome

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Hou, Fangxing, Peking Union Medical College Hospital, Dongcheng-qu, Beijing, China
  • Zou, Linfeng, Peking Union Medical College Hospital, Dongcheng-qu, Beijing, China
  • Shi, Xiaoxiao, Peking Union Medical College Hospital, Dongcheng-qu, Beijing, China
  • Xia, Peng, Peking Union Medical College Hospital, Dongcheng-qu, Beijing, China
  • Qin, Yan, Peking Union Medical College Hospital, Dongcheng-qu, Beijing, China
  • Chen, Limeng, Peking Union Medical College Hospital, Dongcheng-qu, Beijing, China
Background

Glomerulonephropathy (GN) in primary Sjögren's syndrome (pSS) was rare. We aimed to compare the clinical features and prognosis between patients with membranous nephropathy and pSS (pSS-MN) and idiopathic MN (iMN) patients.

Methods

From January 1993 to September 2022, at Peking Union Medical College Hospital, we enrolled 96 pSS-MN patients diagnosed with renal pathology and the revised version of the American-European Consensus Group. The control group was 150 iMN patients diagnosed with renal pathology and positive serum anti-M-type phospholipase A2 receptor (PLA2R) antibody.

Results

Compared to the iMN patients, the pSS-MN patients were older, predominantly females (80.2% vs. 44.7%, P <0.05), with lower 24h urine proteins, higher serum IgG levels, more anemia, and hypocomplementemia. pSS-MN patients had lower ratios of positive serum anti-PLA2R antibody (34.0% vs. 59.1%) and positive tissue PLA2R antigen (65% vs. 87.3%) (both P <0.05). During the median time of 22 months, treated with a combination of glucocorticoids, immunosuppressive agents (cyclophosphamide, calcineurin inhibitors, tripterygium glycosides), or rituximab, the patient's eGFR remained normal. Moreover, the 24h urine proteins decreased from 3.1 g/d to 0.8 g/d.

Conclusion

We reported the largest cohort of pSS-MN patients displayed more pronounced immunological anomalies, with a lower prevalence of positive serum anti-PLA2R antibody and tissue PLA2R antigen than the iMN patients, and responded well to the treatment with stable eGFR.

Funding

  • Government Support – Non-U.S.