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

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.

Medienart:

E-Artikel

Erscheinungsjahr:

2021

Erschienen:

2021

Enthalten in:

Zur Gesamtaufnahme - volume:10

Enthalten in:

CEN case reports - 10(2021), 2 vom: 04. Mai, Seite 281-286

Sprache:

Englisch

Beteiligte Personen:

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

Links:

Volltext

Themen:

Case Reports
Immunoglobulin G subclass
Membranous nephropathy
Phospholipase A2 receptor
Receptors, Phospholipase A2
Xp 11.2 translocation renal carcinoma

Anmerkungen:

Date Completed 24.11.2021

Date Revised 24.11.2021

published: Print-Electronic

Citation Status MEDLINE

doi:

10.1007/s13730-020-00556-9

funding:

Förderinstitution / Projekttitel:

PPN (Katalog-ID):

NLM319570673