Flashcards in 5/6 Chronic Glomerulonephritis Deck (28)
what are the classification of chornic glomerulonephrtis ?
minimal change disease = mostly in children
measngioproliefartive glomerulonephritis IgM
focal and segmnetal sclerosis and hyalinosis
idiopathic IgA berger = NEPHRITIC
what is the diagnosis of minimal change disease ?
nephrotic syndrome =SELECTIVE ALBUMINUREA WITHOUT evidence of hypertension and hematuria - the only one with selective
renal function is preserved
no changes when viewing kidney biopsy specimens under light microscopy,
Under immunofluorescence, there are no immunoglobulins or complement deposits
= hence minimal change disease
electron microscopy =
hallmarks of the disease were discovered.
diffuse loss of visceral epithelial cells foot processes podocyte effacement,
vacuolation, and growth of microvilli on the visceral epithelial cells, allowing for excess protein loss in the urine
etiology of minimal change disease ?
unknown - idiopathic
clinical presentation of minimal change disease ?
swellings that often occur suddenly, most prominent in the morning, initially covering the eyelids and the face, and later on spreading across the limbs, in the sacral region and can generalize to anasarca with leak in the body cavities.
abdominal discomfort due to ascitis , diarrhea
edema of scrotum
increased tendency for infection and thrombotic events
what is the treatment for minimal change disease
everything for nephrotic syndrome
high dose methyl prednisolone replaced by by oral prednisolone
= good reposes then we taper it
if not effective add
cyclophosphamide/ cyclosporin - monitoring of bone marrow and gonadal toxicity
high dose of immunovenin if still not responsive
ACE inhibitors to reduce protein
direct and indirect anticoagulants
iv heparin , the end of the month switched to indirect anticoagulant (dabigartan)
mesangioproliferative glomerulonephritis is classified as a ?
type 2 lupus nephritis
what is the pathophysiology of mesangioproliferative GN?
Mesangial use endocytosis degrade circulating immunoglobulin.
This normal process stimulates mesangial cell proliferation and matrix deposition.
Therefore, during times of elevated circulating immunoglobulin (i.e. lupus / IgA nephropathy) increased number of mesangial cells and matrix
we can also find but not necessary to find IgM and C3 deposits in the mesangium which is why the disease is also called igm nephorpathy
what is the diagnosis of mesangioproliferatve glomerulonephritis ?
immunoflorecence - igm and c3 deposits in mesangium
LM - varied mesangial proliferation affecting ALL glomeruli to the same extent
what is the clinical presentation of mesangioporliferative glomerulonephritis
hematuria is established for majority of patients
igm positive have more nephrotic syndrome
HOWEVER igM negative have macroscopic hematourea
what is the treatmnet of MSGN ?
pulse treatmnet with methylprednisolone
add cyclosphosphamide monthly pulses
if iGm positive - cytosporin a
immunovenin if still not responsive
then oral prednisone
anticoagulants such as IV heparin
focal segmental glomeruloscelrosis is the most common what ?
most common cause of nephrotic syndrome in adults
esp african american and hispanic
what is the characteristics of FSGS
it has subacute lesions , segmental sclerotic changes , poor prognosis and resistance to corticosteroid therapy
what is the classification of FSGS?
primary = when no underlying cause is found =
role of circulating permeability factor
because FSCG rapidly occurs in graft of renal transplant , however if plasmaphoresis is done before transplant the disease is limited
secondary = underlying cause is identified
presents with kidney failure
toxins = such as heroin , pamidronate
familial = mutatation in nephron and podocin
hypertension , DM and obesity causing glomerular hyper filtration = mechanical stress on podocytes
what are the pathological variants of FSGS?
collapsing variant = higher risk for ESRD = heavy proteinurea
one glomerulus segmnetal or global obliteration of the glomerular capillary by wrinkling and collapse of the glomerular basement membrane because of podocyte hypertrophy and hyperplasia
glomerular tip lesions variant = low risk
at least 1 segmental lesion involving the tip domain (ie, the outer 25% of the tuft next to the origin of the proximal tubule
cellular variant =
1 glomerulus with endocapillary hypercellularity involving at least 25% of the tuft and causing occlusion of the capillary lumen/lumina
perihilar variant :
of at least one glomerulus with perihilar hyalinosis with or without sclerotic lesions
not otherwise specified variant
how do we diagnose focal segmental glomerulosclerosis ?
massive non selective porteinurea
microscopic hematurea in pronounced mesangial proliferation
progressive decrease in GFR
increase in serum urea and creatinine
LM : segmental sclerosis and hyalinosis
IM : most commonly negative
maybe I'm , C1 and C3 inside the sclerotic regions
EM : effacement of podocyte foot process
what is the clinical presentation of FSGS?
hypertension and deterioration of renal function - oligourea - usual find
management of FSGS?
extremely important to differentiate the primary from the secondary because the secondary leads to more cases in ESRD , and to treate the underlying disease
ACE AND arb
in primary forms
corticosteroids - prendisole
renal transplant has not been success due to frequent reoccurrence
what is the pathopsyiology of membranous glomerulonephritis ?
and clinical presentation?
immune complex in subeipthelium! - between podicytes and gbm
the antigens may be part of the basement membrane - which are M type phospholipase A2 receptor
or neutral endopeptidase -expressed in the podocyte surface lining the basement membrane
or deposited there from systemic circulation
immune complex forms the MAC
inflammation of the basement membrane , PODOCYTES
initially, proteinuria may be mild, and nephrotic syndrome may develop at a later stage.
The swelling could appear without any other symptoms and later disappear spontaneously
Arterial hypertension and worsing of the renal function
what re the causes for membranous glomerulonephritis ?
idiopathic - antigen targets
immune complex deposition by cationic bovine serum albumin seen in cows milk and beef protein
autoimmun - sle
infection such as - hep c , hep b , HIV , syphilus
drugs - captopril and nsaids
lung carcinoma, colon carcinoma, lymphoma, leukemia
diagnosis of membranous glomerulonephritis ?
High-grade non-selective proteinuria,
in a small percentage of patients there is a microscopic haematuria
Hypoproteinaemia with hypoalbuminemia and hyperlipidemia is observed.
Coagulation status shows a tendency to hypercoagulability
serum c3 are usually low esp in case of viral hep c
1) diffuse thickening of basement membrane
GBM appears to be spiked and holey appearance appearance - Swiss cheese).
em : spike and dome pattern of basement membrane
subepitheila deposits - as it progresses these deposits cleared and leave cavities - can then become sclerosed and hylainsed
IMF= granular deposition of immunoglobulin and complement along basement membrane
what is the treatment of membranous glomerulonephritis ?
ACE inhibitors or ARB’s
ponticelli regiment :
Starts with a 3-day pulse of methylprednisolone cyclophsopamide alternating with corticosteroid (mixed results) such as prednisolone
On the second month, the corticosteroid treatment stops and the oral intake of cyclophosphamide or chlorambucil iniciated for one month.
Corticosteroids and cytostatics alternate for a total of 6 months
corticosteroid must always be combined with one of the mentioned above or these :
Besides the Pontichelli regiment there is the pulse treatment with corticosteroids and cyclophosphamide that we treate of idiopathic nephrotic syndrome.
A very good effect in patients is the tacrolimus
spontaneous remission common and higher remission rates with anti proteinuria therapy
progression - dialysis
complication of membranous glomerulonephritis
more prone to thromboembolism than other nephrotic syndrome diseases
what is the etiology berger disease ?
most likely upper respiratory tract infection or go infection triggered - isa antibodies form immune complex that are deposited in kidney (TYPE 3 HYPERSENTITIVTY)
systemic disease - henoch
neoplasma 0 giga monoclonal gammopathy , lung carcinoma
what is the clinical features of berger disease ?
asymptomatic - microscopic hematurea
recurring epodes of gross hematurea may have flank pain
hypertension is initially absent but may develop
it may progress into rpgn or nephrotic syndrome
what is the diagnosis of iga nephropathy ?
- perisitant microhematurea
Dysmorphic erythrocytes with erythrocyte cylinders are found in the sediment
in flare ups or intercurrent infections - gross hematurea
low to moderate proteinurea mostly less than 2g/24
or nephrotic syndrome findings
GFR usually remains unchanged
serum iga elevated
C3 levels are generally normal so is the rest of the complement panel
renal biopsy - usually indicated only when severe progression f disease such as hypertension increases , creating levels increase
lm - mesangial proliferation
IMF : mesangial iga deposits
maybe also igG and ism
EM - mesangial immune complex deposits
what is the treatmnet for berger disease ?
patients with just isolated hematurea
- monitor kidney function and start treatmnet if progresses
symptoms usually resolve spontaneously for these patients
persistence of proteinurea above 1g/24hr and hypertension -
acei and arb
polyunsaturated fatty acids
corticosteroids at a conventional dose for two years
pronounced nephrotic syndrome in histological variation, appropriate to administer corticosteroid pulse then conventional corticosteroid
for proteinuria below 1 g/24h, the arterial pressure should be maintained below 130/80 mmHg
With proteinuria above 1 g/24h it should not exceed 125/75 mmHg.
patients with recurrent macroscopic haematuria, recommendations include aggressive hydration.
severe or rapidly progressive = glucocorticoids plus ,cyclophosphamide / azathioprine
selective plasma plasmapheresis synchronized with cytostatic therapy.
high chance of reoccurrence with renal transplant
what is the pathopshyiology of isa nephropathy ?
iga depostion in mesangium
thought to produce mac
b lymphocytes also produce glycan specific antibody of the egg class directed at the abdonamlly gluycsilated igA1