Toward a working definition of C3 glomerulopathy by immunofluorescence

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Presentation transcript:

Toward a working definition of C3 glomerulopathy by immunofluorescence Jean Hou, Glen S. Markowitz, Andrew S. Bomback, Gerald B. Appel, Leal C. Herlitz, M. Barry Stokes, Vivette D. D'Agati Kidney International Volume 85, Issue 2, Pages 450-456 (January 2014) DOI: 10.1038/ki.2013.340 Copyright © 2014 International Society of Nephrology Terms and Conditions

Figure 1 Light microscopy (LM) and electron microscopy (EM) findings in membranoproliferative glomerulonephritis (MPGN) types 1–3. (a) LM showing MPGN1 with lobular accentuation and membranoproliferative features characterized by glomerular basement membrane (GBM) duplication (arrow) and mesangial cell interposition (arrowhead; Jones methenamine silver, × 400). (b) EM of MPGN1 showing electron-dense subendothelial deposits (asterisk) associated with GBM duplication ( × 6000). (c) LM showing MPGN2/DDD (dense deposit disease) with uniform thickening of the GBM (arrow; periodic acid–Schiff, × 600). (d) EM of MPGN2/DDD showing electron-dense deposits in the lamina densa causing alternating prominent thickening (asterisk) and thinning of the GBM ( × 6000). (e, f) EMs of MPGN3: Burkholder (e) and Strife and Anders (f) subtype ( × 6000). Note the discrete subepithelial and subendothelial deposits in Burkholder variant (e) as compared with the more ill-defined, complex intramembranous deposits in Strife and Anders variant (f). Kidney International 2014 85, 450-456DOI: (10.1038/ki.2013.340) Copyright © 2014 International Society of Nephrology Terms and Conditions

Figure 2 Illustrative examples of immunofluorescence (IF) staining meeting criteria 1–3. IF microscopy findings ( × 400). (a, b) Criterion 1: C3G case with features of membranoproliferative glomerulonephritis (MPGN)1 showing 2–3+ mesangial and capillary wall staining for C3 (a), with negative immunoglobulin (Ig)G (b). (c, d) Criterion 2: dense deposit disease (DDD) case showing 2+ glomerular capillary wall and mesangial staining for C3 with mesangial ring forms (c), with trace (±) IgM (d). (e, f) Criterion 3: DDD case showing 3+ glomerular capillary wall and mesangial staining for C3 (e) with 1+ IgG (f). Bar=100μm. Kidney International 2014 85, 450-456DOI: (10.1038/ki.2013.340) Copyright © 2014 International Society of Nephrology Terms and Conditions