Table 1.

Classification of MGRS entities based on presence and site of deposition of monoclonal proteins in renal structures

MGRS with monoclonal protein deposition in renal structures
Glomerular depositionTubulointerstitial depositionIntrarenal vascular deposition
Amyloidosis (AL/AH/AHL) LCPT with and without crystals Amyloidosis 
Monoclonal fibrillary glomerulopathy CSH Crystalglobulinemia 
Immunotactoid glomerulopathy Amyloidosis MIDD 
Cryoglobulinemic GN (types I and II) MIDD Cryoglobulinemic GN (types I and II) 
MIDD (LCDD/HCDD/HLDD)   
PGNMID   
MPGN associated with monoclonal immunoglobulin   
MN secondary to monoclonal immunoglobulin   
MGRS with monoclonal protein deposition in renal structures
Glomerular depositionTubulointerstitial depositionIntrarenal vascular deposition
Amyloidosis (AL/AH/AHL) LCPT with and without crystals Amyloidosis 
Monoclonal fibrillary glomerulopathy CSH Crystalglobulinemia 
Immunotactoid glomerulopathy Amyloidosis MIDD 
Cryoglobulinemic GN (types I and II) MIDD Cryoglobulinemic GN (types I and II) 
MIDD (LCDD/HCDD/HLDD)   
PGNMID   
MPGN associated with monoclonal immunoglobulin   
MN secondary to monoclonal immunoglobulin   
MGRS without monoclonal protein deposition in renal structures
Glomerular involvementTubulointerstitial involvementIntrarenal vascular involvement
C3GN  TMA 
TMA  POEMS syndrome 
POEMS syndrome   
MGRS without monoclonal protein deposition in renal structures
Glomerular involvementTubulointerstitial involvementIntrarenal vascular involvement
C3GN  TMA 
TMA  POEMS syndrome 
POEMS syndrome   

AH, heavy chain amyloidosis; AHL, heavy and light chain amyloidosis; AL, light chain amyloidosis; C3GN, C3 glomerulonephritis; CSH, crystal-storing histiocytosis; HCDD, heavy chain deposition disease; HLDD, heavy and light chain deposition disease; LCDD, light chain deposition disease; LCPT, light chain proximal tubulopathy; MN, membranous nephropathy; POEMS, polyneuropathy organomegaly endocrinopathy monoclonal gammopathy, and skin changes.

Adapted from Ciocchini et al with permission.

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