Fig 1: Immunohistochemistry (IHC) of DNAJB9 exclusively highlights fibrillary glomerulonephritis (FGN) glomeruli. (a,b) Immunohistochemistry shows strong glomerular staining for DNAJB9 in 2 different cases of FGN. (c) Normal and (d) ? light-chain amyloidosis do not show glomerular staining for DNAJB9. (a, Original magnification ×20; b-d, original magnification ×200.)
Fig 2: DNJAB9 immunohistocheimstry is negative in non-fibrillary glomerulonephritis renal biopsy samples. Staining for DNAJB9 in bacterial infection-associated glomerulonephritis (GN) (a), diabetic glomerulosclerosis (b), membranous nephropathy (c), immunotactoid GN (d), lupus nephritis (e), and ? heavy-chain deposition disease (f). (Original magnification ×100.)
Fig 3: Extraglomerular deposits of DNAJB9 in fibrillary glomerulonephritis (FGN). (a) Focal smudgy staining of tubular basement membranes (arrows) similar to the glomerular staining. (b) Smudgy staining of an arteriole (arrow). (c) Smudgy staining of splenic arterioles. (d) Linear to smudgy staining of tubular basement membranes for IgG. (e) Smudgy staining on an arteriole (arrow) for IgG. (f) Splenic arterioles from the same specimen as in (c) show smudgy staining for IgG by pronase immunofluorescence (arrows). (a-e, Original magnification ×400; f, original magnification ×200.)
Fig 4: Ultrastructural immunohistochemical localization of DNAJB9 on fibrils of fibrillary glomerulonephritis (FGN). An immunoelectron microscopy micrograph from a patient with FGN (FGN 1 in Table 4) showing many gold particles labeling anti-DNAJB9 bound to FGN fibrils in the mesangium. (Original magnification ×60,000.)
Fig 5: Histomorphological features of monoclonal fibrillary glomerulonephritis. a PAS positive-mesangial deposits (stain PAS, ×40). b Capillary loop thickening with variable argyrophilia (stain Jones' silver methenamine, ×40). c Non-Congophilic nature of the mesangial deposits (stain, Congo red, ×40). d Wooly capillary wall deposition of IgG and strong positivity for lambda light chain (inset). e DNAJB9 positivity in the deposits. f Randomly arranged fibrils identified in the mesangium and along glomerular basement membrane measuring 10–30 nm in diameter (200 kv, ×7,000).
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