Case report: a peculiar glomerulopathy in a patient suffering from nephrotic syndrome

Abstract

Background: Podocyte infolding glomerulopathy (PIG) is a rare histopathologic finding with global infolding of the podocytes into the glomerular basement membrane (GBM), accompanied by microstructures underneath. Described in 2002 for the first time, PIG was proposed as a new pathological entity in 2008 based on the largest case series so far. Yet all of the described cases derive from Asian countries. We report a case from Germany fulfilling the diagnostic criteria of PIG. Considering the scarcity of data on this entity especially in Western countries, collecting cases like ours and multicentric meta-analyses will be crucial to obtain a better understanding of PIG, its causes, clinical course and potential treatment options.

Case presentation: A 56-year-old Caucasian woman with a history of rheumatoid arthritis (RA), no other comorbidities and no known renal disease was admitted to the hospital with acute kidney injury (AKI) and nephrotic syndrome. Physical examination was unremarkable except for anasarca. Renal ultrasound revealed no abnormalities. Laboratory and urine analyses were consistent with the nephrotic syndrome and renal failure. Serological studies regarding ANA, ANCA, anti-PLA2R autoantibodies, complement, virus infections, immunofixation and quantitative light chain analysis were unremarkable. A renal biopsy was performed. Light microscopic examination showed flattened tubular epithelium consistent with acute tubular damage, no infiltrates and unremarkable glomeruli except diffuse and global holes in the GBM (Fig. 1a) and negative staining for immunoglobulin heavy-chains, light-chains and complement split products. Electron microscopy revealed a rare correlate for these holes: global peculiar infolding of podocyte cytoplasm into the GBM. Most of these infoldings were accompanied by condensation of the GBM underneath. No such condensation or electron dense deposits were found without these infoldings or outside the GBM.

Conclusion: Here we report the first case of PIG outside of Asia. Since there are only few reports about this specific finding, we feel there is a need to share information in an attempt to accumulate knowledge about this possible new entity and potential treatment options.

Keywords: Membranous Glomerulopathy; Microspheres; Nephrotic syndrome; Podocyte infolding; Renal biopsy.

Fig. 1

a Jones silver staining shows holes in the glomerular basement membrane (circle). Original magnification × 630, scale bar represents 50 μm. b Electron microscopy shows the ultrastructural correlate to these holes: infolding of the podocyte cytoplasm into the glomerular basement membrane, most of them with underlying densities (red arrows). This finding is characteristic for the podocyte infolding glomerulopathy (PIG). Transmission electron microscopy, original magnification × 10000, scale bar represents 1000 nm)

Fig. 2

Graph showing the course of serum creatinine, albuminuria and serum albumin after onset of the disease and after initiation of the very effective treatment with prednisolone and rituximab. Note the logarithmic scale of the y-axis