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Glomerulous
Glomerulus consists of a combination of
Cellular elements:
Endothelial cells
Mesangial cells
Visceral and parietal epithelial cells
Extracellular matrix:
Glomerular basement membrane (GBM)
Mesangial matrix
Glomerular filtration
Extraordinarily high
permeability to water
and small solutes
Impermeability to
proteins, such as
molecules of the size of
albumin (3.6-nm
radius; 70 kDa) or larger.
Discrimination depends
on
size (the larger, the less
permeable)
charge (the more
cationic, the more
permeable).
Glomerulonephritis
Glomeulonephritis is an immune-mediated renal
Antibody-mediated
Antibody react with fixed glomeular antigen (i.e.,
IgA)
Circulating antigen-antibody complex that trapped
by glomerulous (i.e., acute (diffuse) proliferative GN).
Cell-mediated: caused by sensitized T-cells
Activation of alternative complement pathway
Terminology
All glomeruli are involved: Diffuse (generalized)
< 50% glomeruli are involved: Focal
In one glomerulus:
if the whole glomerulus is involved : Global
portion of the tuft is involved: Segmental
Other terminologies in common use:
Proliferative: increase in cell number cells
Sclerosing: Hardining of the tissue
Histologic Lesions
Hypercellularity.
Cellular proliferation of mesangial or endothelial cells.
Leukocytic infiltration
Formation of crescents. These are accumulations of
Epimembranous deposits
Subepithelial humps
Mesangial deposits
Subendothelial
deposits
Histologic Lesions
Hyalinosis denotes the accumulation of homogeneous and
Glomerular diseases
PRIMARY GLOMERULOPATHIES
Diffuse proliferative
glomerulonephritis
Post-infectious
Other
Rapidly progressive
(crescentic)
glomerulonephritis
Membranous glomerulopathy
Minimal-change disease
Focal segmental
glomerulosclerosis
Membranoproliferative
glomerulonephritis
IgA nephropathy
Chronic glomerulonephritis
GLOMERULAR
INVOLVEMENT
Systemic lupus
erythematosus
Diabetes mellitus
Amyloidosis
Goodpasture syndrome
Microscopic
polyarteritis/polyangiitis
Wegener granulomatosis
Henoch-Schnlein purpura
Bacterial endocarditis
HEREDITARY DISORDERS
Alport syndrome
Thin basement membrane
disease
Fabry disease
Glomerulonephritis
Primary glomeulonephritis (GN):
Acute (diffuse) proliferative GN
Rapidly progressive (crescentic) GN
IgA nephropathy
Nephritic syndrome
Anti-GBM Disease
Membranoproliferative GN
Minimal change disease
Nephrotic syndrome
Focal segmental glomeulosclerosis
Membraneous GN
Primary glomerulonephritis
(GN)
Nephritic syndrome:
Nephrotic syndrome:
Minimal change disease (lipoid nephrosis or nil)
Focal segmental glomeulosclerosis
Membranous GN
Membranoproliferative GN
Poststreptococcal GN
Classic presentation - acute nephritic picture with hematuria, pyuria,
red blood cell casts, edema, hypertension, and oliguric renal failure,
which may be severe enough to appear as RPGN.
nephrotic range.
a renal biopsy.
Poststreptococcal GN
Poststreptococcal GN
Treatment is supportive, with control of hypertension,
Cresentric GN
Classification and
Pathogenesis.
Type 1; Anti-GBM disease:
Renal limited
Goodpasture syndrome
agents.
IgA nephropathy
The most common type of primary GN worldwide (15-40%).
It is classically characterized by episodic hematuria associated with
Male preponderance
Peak incidence in the second and third decades of life with worse
clinical picture.
between Henoch-Schnlein purpura and IgA nephropathy. HenochSchnlein purpura is distinguished clinically from IgA nephropathy
by prominent systemic symptoms, a younger age (<20 years old),
preceding infection, and abdominal complaints. .
IgA nephropathy
Immune complex-mediated glomerulonephritis defined by the
with IgA.
IgA1 subclass.
presents as RPGN.
IgA nephropathy
IgA nephropathy is a benign disease for the majority of
Anti-GBM Disease
Goodpasture's syndrome = GN + Pulm. Haemorrhage
Target Epitope 3 NC1 domain of collagen IV
Bimodal Presentation
young men in their late 20s
men and women in their 6070s.
Anti-GBM Disease
Renal biopsy
H&E
Focal or segmental
necrosis
Crescent formation
Interstitial nephritis
Fibrosis and tubular
atrophy.
IF - linear staining for
Treatment:
Plasmapheresis
Steroids
Cyclophosphamide
Membranoproliferative
Glomerulonephritis
Also Known as Mesangiocapillary
glomerulonephritis or lobar
glomerulonephritis.
It is an immune-mediated GN characterized by
or young adulthood.
MPGN is subdivided pathologically into Type I,
Membranoproliferative
Glomerulonephritis
Type I MPGN - most proliferative of the three types.
Mesangial proliferation with lobular segmentation on renal biopsy.
Tram-tracking- mesangial interposition between the capillary basement
Membranoproliferative
Glomerulonephritis
Type I Disease (Most Common)
Idiopathic
Subacute bacterial endocarditis
Systemic lupus erythematosus
Hepatitis C cryoglobulinemia
Mixed cryoglobulinemia
Hepatitis B
Cancer: Lung, breast, and ovary (germinal)
Type II Disease (Dense Deposit Disease)
Idiopathic
Partial lipodystrophy
Type III Disease
Idiopathic
Membranoproliferative
Glomerulonephritis
Patients present with Proteinuria, hematuria, and pyuria
(30%)
Treatment
allografts
MCD -Treatment
Steroids- Prednisone is first-line therapy
other immunosuppressive drugs, such as cyclophosphamide,
tapered.
taper.
Focal segmental
glomeulosclerosis
Focal Segmental
Glomerulosclerosis
Focal segmental
glomeulosclerosis
Focal segmental
glomeulosclerosis
corticomedullary junction
Focal and segmental scarring
Other variants
Endocapillary hypercellularity and heavy
proteinuria.
Collapsing glomerulopathy with
segmental/global glomerular collapse & rapid
decline in renal function.
Glomerular tip lesion, which seems to have a
better prognosis.
Membranous
glomerulonephritis
nonselective proteinuria.
Microscopic hematuria is seen in up to 50% of
patients.
MGN has the highest reported incidences of renal
Membranous
glomerulonephritis
Treatment
Treatment of edema, dyslipidemia, and hypertension,