Protocadherin 7 Positive Membranous Nephropathy in China

Yongzhong Zhong1,2, Yunyun Liu3, Dan Zhou1

  • 1National Clinical Research Center for Kidney Diseases, Jinling Hospital, Affiliated Hospital of Medical School, Nanjing University, Nanjing, China.

Abstract

Insights

Protocadherin-7 (PCDH7) is a rare autoantigen in membranous nephropathy (MN). This study found PCDH7-positive MN cases exhibit diverse clinical features and outcomes, with 84% achieving remission.

Area of Science:

  • Nephrology
  • Immunology
  • Pathology

Background:

  • Protocadherin-7 (PCDH7) is a newly identified autoantigen in membranous nephropathy (MN).
  • Limited data exists on the characteristics and outcomes of PCDH7-positive MN.
  • This study aims to elucidate the clinicopathological profile of PCDH7-positive MN.

Purpose of the Study:

  • To investigate the clinicopathological characteristics of patients with biopsy-confirmed PCDH7-positive membranous nephropathy.
  • To analyze the outcomes and identify potential secondary factors in PCDH7-positive MN.
  • To compare isolated PCDH7-positive MN with PLA2R/PCDH7 dual-positive MN.

Main Methods:

  • Retrospective analysis of biopsy-confirmed PCDH7-positive MN cases from January 2023 to October 2024.
  • Collection of demographic, clinical, and pathological data.
  • Subgroup analysis of isolated vs. dual-positive MN and measurement of serum anti-PCDH7 antibody levels.

Main Results:

  • 19 patients (2.75%) were PCDH7-positive: 7 isolated, 12 dual-positive for PLA2R/PCDH7.
  • The cohort presented with variable renal function and proteinuria; 4 patients had nephrotic syndrome.
  • 84% of patients achieved partial or complete remission within a median 13-month follow-up.

Conclusions:

  • PCDH7-positive MN is clinically heterogeneous and can occur in both PLA2R-positive and PLA2R-negative patients.
  • No consistent clinical presentation was observed; secondary conditions were noted in some patients.
  • Preliminary findings suggest anti-PCDH7 antibody levels may not correlate with clinical manifestations or outcomes.

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