Glomerulopathies

Mohammed Barghash

 3 RD COMMNEST CAUSE OF ESRD

AFTER DM & HTN.
1RY OR 2RY TO OTHER DISEASE e.g
LN.
GENETIC SUSPTABILTIY +
EVIROMENTAL Fx.
AUTOANTIBIODIES OR IUMMNE
COMPLEX AND COMPLEMENTS

TERMONOLOGY
FOCAL: some but not all.
SEGMENTAL: part of G affected.
DIFFUSE:> 75% of G are affected.
CRESCENT: epithelial hyperplasia +
monocyte infalx.

HOW TO APPROACH
1- Nephrotic
proteinuria >3.5 g/day, hypoalbuminemia,
edema.
2- Acute Nephritic
Hematuria(RBC CAST) NON NEPHROTIC
proteinuria, HTN, edema, transient impairment
3- RPGN
Acute nephritis + rapid progressive renal
failure.
Asymptomatic: hemat/proteinuria

Investigate?
U microscopy: dysmorphic/cast RBCs
U protein/cr ratio 24h-protien
u/e ANCA C3C4 CRYO ANTI-GBM ASO
ANA THROATE SWAP
X-RAY
KIDNEY US
BIOPSY

ACUTE NEPHRITIC

PSGS:
CHILD HAD URTI or CELLULITIS 1-3 W
GROUP A b-HYMPLYTIC STREPTOCCOCI
INFLAMMATORY CELL+SUBEPITHELIAL
(HUMPS).IgG & COMPLEMENT.
TTT:
Supportive+eradiction of infection
Good prognosis

OTHERS
GN with IE
NON PSGS
Staph,pnuococcus,syphlis,hepC,B
TOXO.MALARIA
SLE
HENOCH-SCHONLEN
CRAYO
ABSCESS

ASYMPTOMATIC

IgA nephropathy= Bergers disease.

The commonest cause of GN world wide new.
IgG,M,A C3 all seen.
Results from exagrated bone marrow or tonsllar IgA
immune response to viral or Ag.
Presents in young M or children after URTI or
gastroenteritis.
Microscopic hemato/proteinuria.
Macro----good prognosis.
5% Nephrotic.
25% ESRD.
50% high serum IgA.

TTT
Mild changes+normal cr+ 1-3g
proteinuria: steroid.
Progressive disease:
Fish oil or steroid + cyclophosph for
3M.
Maintenance : prednisolone +AZA.

 Alports syndrome:
Rare hereditary nephritis, hemato/proteinuria 1-2g/d
,renal impairment and deafness.
Histology :
Split basement membrane.
TTT: ACEI.

OTHERS:
THIN GLOMURLAR BASE MEBRANE DISDASE.
C3 GLOMERULONPHRITIS
COMPLEMENT FACTOR H-5 RELATED PROTEIN
NEPHROPATHY.

RPGN
Syndrome of glomerular hematuria RBCs cast rapid
renal impairment over weeks + focal necrosis, w or w/o
crescents.
Can be :
Immune anti BGM
Non- or pauci-immune ANCA +
2ry to other glomerular
CLASS BY IMMUNE STAIN:
LINEAR:
GRANULAR:
NEGATIVE:

LINEAR:
Anti-GBM:
M=F OVER 50yr
Called Goodpastures syndrome if
lung hemgx.
Alfa 3-4-5 type IV collagen Ab.
IgG & C3.
Ab bind to BM activate protease &
complements damage, crescents
and proteinuria.

ttt
Plasma exchange : to remove Ab.
Steroids: to suppress inflammation.
Cyclophosphamide:
To reduce further Ab production.
{no relapsing/remitting}

GRNULAR
Idiopathic RPGN.
ASOCIATED W 1RY GN
-MCGN membernoprolifrative II>I
-membranous GN
- IgA nephropathy
ASSOCIATED W 2RY GN
- post-infx
- SLE
- HENOCH-SCHONLEN
-cryoglobulinemia

NEGATIVE IMMUNE
WAGNAR G
MICROSCOPIC POLYANGIITIS
CHURG-STRASS G
are small vessels vasculitis associated
with +ANCA.

Lung hemrgx
Crescent GN
HTN
Vasculitic rash/ulcers.
cANCA = PR3-ANCA

WAGNAR G

pANCA = MPO-ANCA

MICROSCOPIC POLYANGIITIS
CHURG-STRASS

TTT
Steroid pulsed + cyclo/reitox
Fulminant disease plasma
exchange 7x(3-4L)
Maintenance: with STEROID + AZA

Other:
ATG / CAMPATH-IH antiCD52

LN
The International Society of
Nephrology classification system
divides glomerular disorders
associated with SLE into

six different patterns (or classes)

based upon kidney biopsy
histopathology .

Minimal mesangial lupus nephritis

(class I)
patients typically have a normal
urinalysis, no or minimal proteinuria,
and a normal serum creatinine.
As a result, a biopsy is not usually
performed.
Usually Asymptomatic.
have only mesangial immune deposits,
identified by immunofluorescence alone,
TTT: no

Mesangial proliferative LN
(class II)
Patients with class
II disease typically
present with
microscopic
hematuria and/or
subnephrotic
proteinuria;
Benign only
steroid

hypertension is
uncommon, and
the nephrotic
syndrome and
renal insufficiency
are virtually never
seen unless the
patient progresses
to more advanced
disease.

Mesangial proliferative LN
(class II)

Focal proliferative LN (class

III)
Hematuria and proteinuria are seen in
almost all patients with class III disease,
some of whom also have the nephrotic
syndrome, hypertension, and an
elevated serum creatinine .
fewer than 50 percent of glomeruli
affected.
mesangial proliferative changes may
also be present

Diffuse proliferative LN
(class
IV)
more than 50 percent of have active or inactive,
segmental or global endocapillary or extracapillary
glomerulonephritis

Membranous LN (class V)
diffuse thickening of the glomerular
capillary
subepithelial immune deposits
Mesangial
HyperCellulrity in
LN V

TTT of III,IV & V

INDUCTION:
Steroid +
CYC WHITE or MMF BLACK

REMISSION MENTENANCE:
MMF> AZA or CIC
Good prognosis:
I,II & V

Advanced sclerosing LN
(class VI)
more than 90 percent of glomeruli
are globally sclerosed as well as the
advanced stage of chronic class III,
IV, or V LN.
TTT: nothelpful

Referance
UpTo Date
Pocket of medicine
Kumar & Clarkss 2012

THANK YOU ..