Masri Its an Inflammatory process that involves glomeruli damage involving the basement membrane capillary endothelium and mesangium while in nephr O tic syndrome the main structure to be damaged is ID: 934440
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Slide1
Nephritic Syndrome
Done by: Ahmad Al-
Masri
Slide2It’s an
Inflammatory process
that involves
glomeruli
(
damage involving
the
basement
membrane
,
capillary endothelium
, and
mesangium
)
(while in
nephr
O
tic
syndrome the main structure to be damaged is
Podocyte
)
Due to
the glomeruli
damage:
filtration barrier is weakened
and
enables
passage
of
proteins
,
erythrocytes
↓GFR
Oliguria
&
↑
BUN/Cr ratio
↑Hydrostatic pressure
Hypertension
&
Edema
Slide3Slide4Classic
presentation:
Dark
urine (RBCs)
Proteinuria (<3.5g/day)
Swelling/edema
Fatigue
(uremia)
Hypertension
Slide5Causes
Could be classified
Primary
( idiopathic)
v.s
.
Secondary
Or classified as
Immune mediated
v.s
.
Pauci
-immune
Slide6Rapid progressive glomerulonephritis
(
RPGN
)
is an acute
GN resulting
from the secondary
sequelae
of inflammatory (
crescenteric) GN, of which there are many causes.
Slide7Crescenteric
apperance
Negative IF
Granular
IF
Linear IF
Slide8Post-infectious GN
GN secondary
to
bacterial infection
most commonly the
nephritogenic
strains
of streptococcus
(
group A beta hemolytic streptococci).Immune complexes deposit in
sub
endo
thelial
space.Common in children (can also occur in adults
)Classic case:Child presented 2-3 weeks following strep throat infection or 6 weeks post impetigo with Nephritic syndrome findings
Slide9Post-infectious
GN
If we take a Bx. we will see
Under
light
microscope: Enlarged,
hypercellular
glomeruli
Under
immunofluorescence: Granular
apperance
(
IgG, IgM, C3)Under Electron microscopy:
Subepithelial “humps’’
Slide10Post-infectious GN
Good prognosis
in
children
(95
% recover
completely)
Adults
have worse prognosis (About
60%
recover)
No specific therapy
(supportive with management of high blood pressure and fluid overload , spontaneous resolution is expected
)
Slide11IgA Nephropathy
Berger’s
Disease
Most
common
form glomerulonephritis
in adults
worldwide.
Most common cause of
repeated
episodes of hematuria
(nephritic
).
Typically cause hematuria 2 days post URTI
or Diarrheal illness.Over time leads to ESRD and HD (50% patients)Affect mainly Males ,especially Asian or white population.
Slide12IgA Nephropathy
Berger’s Disease
IgA
Immune
complexes
deposit
in
mesangium
.
Bx. under
immunofluorescence:
Granular apperance (IgA)
Classic case:Recurrent episodes hematuria since childhood, follow URI or diarrheal illness, Slowly worsening renal function (BUN/Cr) over time, Possible progression to ESRD and HD (20yrs+)Don’t
confuse with other glomerular disordersPost-strep GN: weeks after infectionIgA GN: days after infection
Minimal change: nephrotic syndrome after URI
Slide13IgA NephropathyBerger’s Disease
Patients who have IgA nephropathy with a low risk of progressive disease should be treated with
ACE-I
to
maintain a goal
24 hour
urine protein excretion
of
less than 1 g /24 hours
, anda
blood
pressure
of
less than 130 / 80 .Immunosuppressive therapy must be considered for:1- patients with
persistent proteinuria ( more than 1g/24 h) , 2-progressive kidney dysfunction, 3-or histological findings indicative of RPGN ( like crescents )This includes pulse corticosteroids and cyclophosphamide followed by azathioprine with ACE-I .
Few studies shows that Fish Oil have role in delay some of the late complications of IgA nephropathy
Slide14Henoch-Schonlein Purpura
IgA
nephropathy
+
extra-renal
involvement
Skin
: palpable
purpura
on buttocks/legs GI: abdominal pain, melena
Joint
pains
Most
common childhood systemic vasculitis Diffuse IgA deposition
Tissue biopsy: demonstrates IgA
Slide15Lupus Nephritis
They are subdivided into 6 types, here in
nephrtic
syndrome we are concern with
type
lll
(
Focal
Proliferative lupus nephritis)
&
type IV
(
Diffuse Proliferative lupus nephritis).Focal: less than 50% glomeruli affected
Diffuse: More than 50% glomeruli affectedAnti-dsDNA Subendothelial deposits
Slide16Lupus
Nephritis
If we take a Bx. we will see
Under
light microscope
:
capillary loops
thickened
“
Wire looping”
Under immunofluorescence
: Granular
apperance
“Full house”
immunofluorescence (IgG, IgA, IgM, C3, C1q)
Slide17Lupus Nephritis
Usually we take Bx. To know the severity to decide the prepare treatment.
Steroids alone or with Cyclophosphamide?
Slide18Membranoproliferative
glomerulonephritis
Also called
mesangiocapillary
GN
.
Immune complex
formation secondary to chronic antigen stimulation.
Common causes are :
Chronic indolent infections
including hepatitis C, syphilis ,
mastoiditis
…etc. .
Autoimmune such as Sjogren , lupus …etc.
Essential cryoglobulinemia ( types I and II ) Malignancies such as carcinomas , sarcomas , lymphomas and leukemias .
Slide19immune-complex and/or complement protein deposition in the
mesangium
and sub endothelium of the capillaries
this results in proliferation of the
mesangial
and remodeling of the capillary wall
Slide20We usually treat them with:
Corticosteroids
Immunosuppressive agents
Antiplatelets
More than 50 % progress to advanced CKD .
Slide21Anti-GBM antibodies disease
Goodpasture’s
Syndrome
IgG
antibodies develop
against the GBM
(
alpha-3
chain to
type IV collagen
) , deposited in a
linear
patter
.Classic case:Young adult, Male present with Hemoptysis
& HematuriaTreatment usually is Plasmapheresis using albumin replacement for 1-2 weeks ,followed by corticosteroids and cyclophosphamide for 3-6 months , Maintenance with azathioprine for 1-2 years
Slide22Vasculities
syndrome
Most
patients
ANCA
positive
(
c-ANCA or p-ANCA
)
All
vasculities
are associated with Fever + weight loss.Churg-Strauss syndrome (p-ANCA): they
present with Eosinophilia, history of resistant asthma.Wegener's Granulomatosis (c-ANCA): they present with upper respiratory findings (sinusitis/otitis)All can lead to pauci-immune nephritis
Slide23Alport
Syndrome
Hereditary Nephritis
Genetic Mutations in
alpha-3, alpha-4, or alpha-5
chains of
type
IV
collagen.
Chains found in basement membranes
kidney
,
eye,
earInherited: X-linked Classic triad:
Hematuria, Hearing loss, eye abnormalitiesLook for child with triad and family history
Slide24Thank You