Malignancy-associated membranous nephropathy with PLA2R double-positive for glomeruli and carcinoma

Itaru Yasuda, Hirobumi Tokuyama, Akinori Hashiguchi, Kazuhiro Hasegawa, Kiyotaka Uchiyama, Masaki Ryuzaki, Marie Yasuda, Ryuichi Mizuno, Shigeto Ishidoya, Shu Wakino, Hiroshi Itoh

Research output: Contribution to journalArticlepeer-review

Abstract

Phospholipase A2 receptor (PLA2R) is the most common primary target antigen of idiopathic membranous nephropathy (MN) although PLA2R antibodies are also reported to be present in malignancy-associated MN. However, a case of PLA2R-positive MN secondary to PLA2R-positive carcinoma has not been reported. A 26-year-old Japanese woman presented with general fatigue, fever, and nonproductive cough. Computed tomography demonstrated a left kidney mass with pathologic diagnosis of Xp11.2 translocation renal cell carcinoma (RCC). After the second time of administration with Sunitinib, the patients exhibited significant proteinuria and hypoalbuminemia. Renal biopsy revealed a diagnosis of diffuse MN secondary to RCC. Immunofluorescence staining showed granular patterns positive for immunoglobulin (Ig) G, IgA, and C3c. PLA2R and IgG1-3 were positive, while IgG4 was negative. For the treatment of severe nephrotic syndrome, we attempted steroid therapy without any clinical improvement. Open nephrectomy was performed and surprisingly, RCC was stained for PLA2R with polarity for the basal side. At outpatient follow-up, 4 months after the operation, urinary protein had still persisted, although serum albumin was slightly increased. We report a case of PLA2R-positive MN secondary to PLA2R-positive RCC.

Original languageEnglish
Pages (from-to)281-286
Number of pages6
JournalCEN case reports
Volume10
Issue number2
DOIs
Publication statusPublished - 2021 May 1

Keywords

  • Immunoglobulin G subclass
  • Membranous nephropathy
  • Phospholipase A2 receptor
  • Xp 11.2 translocation renal carcinoma

ASJC Scopus subject areas

  • Medicine(all)

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