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Podocytic infolding glomerulopathy: A case report
Kye Won Kwon1, Hyeon Joo Jeong2, Jang Han Lee3
1a Department of Pathology , Bundang Jesaeng General Hospital , Seongnam , South Korea.
Abstract:
Podocytic infolding glomerulopathy (PIG) is a rare glomerular abnormality involving glomerular basement membrane (GBM) bubbling viewable by light microscopy, extensive invagination of the podocytic cytoplasm, and the presence of microstructures viewable by electron microscopy. PIG was proposed as a new disease entity in 2008. However, cases have been reported exclusively in Japan and no case reports outside Japan have been published. Here, we report a case of PIG in a 44-year-old Korean female. The patient showed mild proteinuria without renal functional impairment or other systemic diseases. Glomeruli were normocellular, but GBMs were diffusely and mildly thickened and showed a bubbly appearance with periodic acid methenamine silver (PAMS) staining. Immunofluorescence microscopy showed minimal mesangial IgM deposition, but staining was negative for IgG, IgA, C3, C4, C1q, and fibrinogen. Electron microscopy showed diffuse distribution of microtubules and microspherules within thickened GBM (620-1180 nm). Additional serologic tests revealed positive antinuclear antibodies, but other autoimmune markers were normal or negative. The patient was treated with steroids for three months, after which proteinuria decreased to the normal range.
Insights
Podocytic infolding glomerulopathy (PIG), a rare kidney disease, was identified in a Korean patient. Steroid treatment effectively reduced proteinuria, suggesting a potential therapeutic approach.
Area of Science:
- Nephrology
- Pathology
Background:
- Podocytic infolding glomerulopathy (PIG) is a rare glomerular disease characterized by specific ultrastructural abnormalities.
- First described in 2008, PIG cases have been exclusively reported in Japan.
Observation:
- This report details the first case of PIG diagnosed in a Korean female patient.
- The patient presented with mild proteinuria, normal renal function, and no systemic disease.
- Light microscopy revealed GBM bubbling, while electron microscopy showed microtubules and microspherules within thickened GBM.
Findings:
- Immunofluorescence microscopy showed minimal mesangial IgM deposition.
- Serologic tests were positive for antinuclear antibodies, with other autoimmune markers negative.
- The characteristic GBM thickening and bubbly appearance were confirmed via PAMS staining.
Implications:
- This case expands the geographic distribution of PIG beyond Japan.
- Steroid therapy led to a significant reduction in proteinuria.
- Further research is needed to understand the pathogenesis and optimal management of PIG.
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