The Kidney Flashcards
Primary Glomerulopathies 20.1
Acute proliferative GN, postinfectious, other, rapidly progressive (crescentic) GN, MGN, MCD, FSGS, MPGN, dense deposit disease, IgA nephropathy, Chronic GN
Systemic Diseases with Glomerular Involvement 20.1
SLE, DM, Amyloidosis, goodpasture syndrome, microscopic polyarteritis/polyangitis, wegener granulomatosis, henoch-schonlein purpura, bacterial endocarditis
Hereditary Disorders 20.1
Alport Syndrome, thin basement membrane disease fabry disease
Glomerular syndromes 20.2
Nephrotic Syndrome
Hematuria, azotemia, variable proteinuria, oliguria, edema, HTN
Glomerular Syndromes 20.2
RPGN
Acute nephritis, proteinuria, acute renal failure
Glomerular Syndromes 20.2
Nephrotic Syndromes
> 3.5 g/day proteinuria, hypoalbuminemia, hyperlipidemia, lipiduria
Glomerular Syndromes 20.2
Chronic Renal Failure
Azotemia-> uremia progressing for months to years
Glomerular Syndromes 20.2
Isolated urinary abnormalities
Glomeruluar hematuria, and/or sunephrotic proteinuria
Ab-mediated injury, In Situ immune complex deposition 20.3
Fixed intrinsic tissue antigens
NC1 domain of Type IV collagen antigen (anti-GBM nephritis)
PLA2R antigen (membranous glomerulopathy)
Mesangial antigens
Others
Planted antigens
Exogenous (infectious agents, drugs)
Endoegenous (DNA, nuclear proteins, Igs, immune complexes, IgA)
Circulating Immune Complex Depoosition
Endogenous antigens (DNA, tumor antigens) Exogenous antigens (e.g. infectious products)
Summary of Major Primary Glomerulonephritides 20.4
Postinfectious GN
Pres: Nephritic Syndrome
Patho: Immune complex mediated; circulating or planted antigen
Light Micro: Diffuse endocapillary proliferation; leukocytic infiltration
Fluorescence Microscopy: Granular IgG and C3 in GBM and mesangium; granular IgA in some cases
EM Primary subepithelial humps; subendothelial deposits in early disease stages
Summary of Major Primary Glomerulonephritides 20.4
Goodpasture Syndrome
Pres: RPGN
Path: Anti-GBM COL 4-A3 antigen
Light Micro: Extracapillary proliferation with crescents; necrosis
Flurescence Micro: Linear IgG and C3; fibrin in crescents
EM: No deposits; GBM disruptions; fibrin
Summary of Major Primary Glomerulonephritides 20.4
Chronic GN
Pres: Chronic renal failure
Path: Variable
Light Micro: Hyalinized glomeruli
Fluorescence: Granular or negative
Summary of Major Primary Glomerulonephritides 20.4
MGN
Pres: Nephrotic Syndrome
Path: In situ immune complex formation: PLA2R antigen in most cases of primary disease mostly unknown
Light Micro: Diffuse capillary wall thickening
Fluorescence: Granular IgG and C3; diffuse
EM: Subepithelial deposits
Summary of Major Primary Glomerulonephritides 20.4
MCD
Pres Nephrotic Syndrome Path: Unknown; loss of glomerular polyanion; podocyte injury Light: Normal; lipid in tubules Flurosence: Negative EM: Loss of foot processes; no deposits
Summary of Major Primary Glomerulonephritides 20.4
FSGS
Pres: Nephrotic Syndrome; non-nephrotic proteinuria
Path: Unknown, ablation nephropathy plasma factor (?); podocyte injury
Light Micro: Focal and segmental sclerosis and hyalinosis
Fluorescence: Focal; IgM + C3 in many cases
EM: Loss of foot processes; epithelial denudation
Summary of Major Primary Glomerulonephritides 20.4
MPGN Type 1
Pres Nephrotic Syndrome Path Immune Complex Light Micro: Mesangial Proliferative or membranoproliferative patterns of proliferation; GBM thickening; splitting Fluorescence: IgG + C3; C1q + C4 EM: Subendothelial deposits
Summary of Major Primary Glomerulonephritides 20.4
Dense Deposit disease (MPGN Type II)
Pres Hematuria, Chronic renal failure
Path Autoantibody; alternative complement pathway
Light Micro: Mesangial proliferative or membranoproliferative patterns of proliferation; GBM thickening; splitting
Fluorescence: C3; No C1q or C4
Summary of Major Primary Glomerulonephritides 20.4
IgA
Pres Recurrent hematuria or proteinuria
Path unknown
Light Microscopy: Focal Mesangial proliferative GN; mesangial widening
Fluorescence Micro IgA + IgG, IgM, and C3 in mesangium
EM Mesangial and paramesangial dense desposits
Rapidly Progressive Glomerulonephritis 20.5
Type 1 RPGN (Anti-GBM Ab)
Renal Limited, Goodpasture syndrome
Rapidly Progressive Glomerulonephritis 20.5
Type II RPGN (Anti-GBM Ab)
Idiopathic, postinfectious, SLE, Henoch-Schlonlein purpura (IgA), Others
Rapidly Progressive Glomerulonephritis 20.5
Type III RPGN (Anti-GBM Ab)
ANCA-associated, idiopathic, Wegner Granulomatosis, microscopic polyarteritis nodosa/microscpic polyangitis