case_images: PMC11763301_01
case_images: (a) Two completely sclerosed glomeruli with mild interstitial mononuclear inflammatory cell infiltrate (light microscopy, H and E stain, 10x). (b) Intense IgA deposition in the mesangium and in the capillary walls (immunofluorescence, IgA antiserum, 40x). (c) IgG deposition in the mesangium and in the capillary walls (immunofluorescence, IgG antiserum, 40x). (d) Immunohistochemistry shows bright staining for DNAJB9 in the mesangium and the capillary walls (immunohistochemistry, 20x), DNAJB9 = DnaJ heat shock protein family (Hsp40) member B9, H and E = Hematoxylin and eosin.
case_images: IJN-35-1-98-g1.jpg
case_images: PMC11763301_01_IJN-35-1-98-g1.jpg
case_images: F1
case_images: Light microscopic examination of kidney biopsy showed that all nine glomeruli were completely sclerosed with few foci of tubular atrophy and mild interstitial mononuclear inflammatory cell infiltrate [Figure 1a]. On immunofluorescence microscopy, all glomeruli showed an intense deposition of IgA (3+) with IgG, kappa, and lambda localized in the mesangium as well as in the capillary [Figure 1b and c], whereas IgM, C3, C1q, and fibrinogen were negative. These deposits showed fibrillary substructure on high power with cross diameter ranging from 17 to 22 mm, which was DNAJB9 positive on Immunohistochemistry (IHC) [Figures 1d and 2a, b].
case_images: (a) Electron-dense deposits located in the basement membrane and mesangium (**). (electron microscopy, 2600x). (b) Mesangial deposits composed primarily of short and rigid fibrils without a hollow core, with a diameter ranging between 10 and 23 nm (**), contained in dense fine granular material (electron microscopy, 8500x).
case_images: IJN-35-1-98-g2.jpg
case_images: PMC11763301_01_IJN-35-1-98-g2.jpg
case_images: F2
case_images: These deposits showed fibrillary substructure on high power with cross diameter ranging from 17 to 22 mm, which was DNAJB9 positive on Immunohistochemistry (IHC) [Figures 1d and 2a, b].