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Published on: August 23, 2024
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.
Summary
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.
Keywords:
anti-PCDH7 antibodyautoantigenmembranous nephropathyphospholipase A2 receptorprotocadherin 7More Related Videos
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