Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

nephrology > 4) Acute Glomerulonephritis and rapid progressive glomerulonephritis > Flashcards

4) Acute Glomerulonephritis and rapid progressive glomerulonephritis Flashcards

1
Q

what are the classification for acute glomerulonephritis ?

A

acute post infection glomerulonephritis :

  • post strep GN +
  • GN of infective endocarditis
  • shunt nephritis

acute glomerulonephritis with systemic disorders :

> SLE (lupus nephritis in another topic)

> henoch scholein purpura (systemic form of IgA nephropathy) +

> microscopic polyangitis and other vasculitis disorders +

> wegner granulomatosis +

> Good pasture’s syndrome + ( anti glomerular basement membrane antibody mediated glomerulonephritis AntiGBM)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

what is the classification rapidly progressive glomerulonephritis ?

A

type 1 : anti glomerular basement membrane antibody - good pasture syndrome

type 2 - immune complex mediates glomerulonephritis :

membranoprolifertive neuropathy
henoch scholen purpura
lupus nephritis
post strep GN

Type III: glomerulonephritis associated with vasculitis (pauci GN)

ANCA associated crescentic glomerulonephritis = wegner

= microscopic polyangitis

= eosinophilic granulomatosis / shrug strauss syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

what are the proliferative glomerulonephritis in acute and rapidly progressive glomerulonephritis ? ££££££££££££££££££££££££££££££££££££££££££££££££££££££££££££££££££££££££££££££££££££££££££££££££££££££££££££

A

rapidly progressive glomerulonephritis

membranoproliferative glomerulonephritis

post infectious glomerulonephritis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

post strep GN most commonly seen in

A

children of 3–12 years and patients > 60 years of age

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

post strep GN are high risk in ?

A

patients with diabetes, malignancies, and alcohol dependency.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

what is the etiology post strep GN ?

A

Occurs approximately 10–30 days following an acute infection:

most common cause :group A beta-hemolytic streptococci
inflaming the the mouth and tonsillitis, pharyngitis

Soft tissue infections : erysipelas : group A Streptococcus

non bullous impetigo : S. aureus or Group A streptococci

bullous : S. aureus

Osteomyelitis : Staphylococcus aureus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

immune complex mediated acute GN occurs in ?

A

infective endocarditis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

what is the pathophysiology of post strep GN ?

A

immune complexes containing the streptococcal antigen deposit within the subepithleila part of glomerular basement membrane

nephrotogenic aswell - molecular mimicry also likely

complement activation → destruction of the glomeruli → immune complex-mediated glomerulonephritis and nephritic syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

what are the clinical features of post strep GN ?

A

50% asymptomatic

Nephritic syndrome :

Hematuria

Hypertension: can lead to headaches

generalised Edema;
may be associated with dyspnea
neurologic symptoms (e.g., seizures)

Oliguria

azotemia related symptoms - fatigue , axterixis (flapping tremor) , decreased alertness and confusion

===================

Influenza-like symptoms - fever

Flank pain

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

how can we diagnose post strep GN?

A

Laboratory tests:

Normocytic, normochromic anemia

Possibly elevated BUN and creatinine (often transient)

↑ Antistreptolysin-O titer (ASO) (particularly following streptococcal infection of the pharynx)

↑ Anti-DNase B antibody (ADB) titer (particularly following streptococcal infection of the soft tissue)

↓ C3 complement

Urinalysis: nephritic sediment (e.g., hematuria and RBC casts, mild proteinuria)

===========
Ultrasound: enlarged kidneys

============
Renal biopsy (not performed in most cases)
Indication: suspected rapidly progressive glomerulonephritis

Light microscopy:
Glomeruli are globally and diffusely enlarge
diffuse hypercellularity due to mesangial and endothelial cell increase
infiltration of monocytes and polymorphonuclear cells.
all of this block the capillary

Immunofluorescent for every post infection : granular
garland pattern, the starry sky pattern, and the mesangial pattern

The starry sky pattern is an irregular, finely granular pattern with small C3 deposits on glomerular basement membrane This pattern is often seen in the early phase of the

The starry sky pattern may turn into the mesangial pattern, which is characterized by granular deposition of C3 with or without immunoglobulin G. - closely related to resolving

sometimes deposits are large and densely packed and aggregate into a garlandlike pattern. These correspond to the humps on the subepithelial side of the glomerular capillary wall seen with electron microscopy
These types of deposits may persist for months and may be associated with the persistence of proteinuria and the development of glomerulosclerosis

Electron microscopy:
“humps” = subepithelial immune complexes (between epithelial cells and the glomerular basement membrane)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

what is the treatment of post strep GN ?

A

In most cases the disease is self-limiting

treatmnet for nephritic syndrome :
low sodium diet
water restriction

if porteinirea or hypertension : acei or arb

if edema and pulmonary edema - loop diuretics

persisting streptococcal infection :penicillin G benzathine

If severe going into rapidly progressive : glucocorticoids,cyclophosphahemodialysis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

what are the complications of post strep GN ?

A

Acute renal failure

Rapidly progressive glomerulonephritis

Nephrotic syndrome later in the course of the disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

what does microscopic polyangitis affect ?

A

small vessels

such as arterioles , venules and capillaries

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

what are the clinical features of microscopic polyangitis ?

A

NEPHRITIC SYNDROME symptoms
but hypertension

palpable purpura
raynaud phenomena

neuropathy - numbness and tingling

myalgia an arthralgia

epistaxis
erosin and perforation of nasal septum - saddle nose deformity

scleritis , uveitis

recurrent otitis media

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

what is microscopic polyangitis glomerulonephritis called ?

A

pauci immune glomerulonephritis = not associated with immune complex , antibodies or complements

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

what is the diagnosis of microscopic polyangitis ?

A

x ray
alveolar haemorrhages = come as opacities

========
nephritic sediment
mild porteinurea

========
biopsy of the organ it affects
(kidneys and organs)

= NO GRANULOMAS different to wegners granulomatosis

= but there is FIBRINOID NECROSIS of neutrophils

immunoflurecsnce
NO IMMUNOGLOBULIN OR COMPLEMENT DEPOSITION

=========
nromocytic normochromic anemia

azotemia

MPO / pANCA positive
= different to polyarteritis nodosa (large vessel affecting)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

what is the treatment of microscopic polyangitis ?

A

immunosuppression with corticosteroids - prednisone with cyclophosphamide / azathioprim
or rituximab
Plasmapheresis may also be indicated in the acute setting to remove ANCA antibodies.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

what type of vessels do henoch scholen affect ?

A

arterioles , venules and capillaries

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

what causes henoch purpura ?

A

preceding viral infection most commonly and upper respiratory tract one - group A strep

Gi infections also possible

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

what is the pathophysiology of henoch purpura ?

A

exposure to antigen
STIMULATION TO iGa

depostion of these iga in vascular walls and mesangium - activation of components - inflammation and damage

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

what are the clinical manifestation of hooch purpura ?

A

skin
symmetric distribution erythramatous macule colaescing into palpable purpura - esp in lower extremities and buttocks

jonts - arthritis and arthralgia

GI - colicky abdominal pain
bloody stools

kidneys - signs and symptoms of nephritic syndrome
there is nephrtic syndrome 
edema 
hypertension possibly and its symptoms 
there is heamturea
22
Q

what is the diagnosis for henoch purpura ?

A 
cbc 
increas platelt count 
increase wbc 
normocytic normochornic
bun andcreatinin transient elevation

serum antibodies and complement
IgA increase
IgA complex
decrease in complement

increase in creatinine or BUN

urinalysis - of nephritic syndrome

======
biopsy
for severe renal involvement or persistent nephritic syndrome

skin - leukocytoclastic vasculitis with iga and C3 immune complex depostion is the hallmark

kidney
LM - diffuse mesangial proliferation because iga are dumped here
endocapillary proliferations

IF
mesangial IgA deposition
C3 complement and firbin

23
Q

what is the treatmnet for henoch scholen purpura ?

A

milld
no treatment
nsaids and pain management and adequate hydration

severe
Iv methylprednisolone pulse therapy with cyclophosphamide and azathioprim
plasmapheresis of refractive

renal transplant if ESRD

dialysis for AKI

antihypertensive drugs
reduce salt intake and water intake

24
Q

what is a serious complication of henoch scholen?

A

turns into rapid progressive glomerulonephritis with crescent formation

GI - intussusception or infraction

25
Q

granulomatosisi with polyangitis or others known as wegners etiology ?

A

idiopathic = occurs more often after a upper respiratory tract infection

26
Q

what are the clinical features for wegners granulomatosis ?

A

ENT
chronic rhinitis- saddle nose
chronic otitis / mastoiditis

lower respiratory tract - potentially life threatening :
cough , dyspnea , hemoptysis

renal - potentially life threatening
pauci immune glomerulonephritis which goes into rapidly progressive crescentic glomerulonephritis with possible pulmonary renal syndrome

with nephritic syndrome

skin
papules , vessies , ulcers
purpura of lower extremities

ocular
conjunctivitis , episcleritis , retinal vasculitis
corneal ulcers

cardiac - potentially life threatening
pericarditis , myocarditis

27
Q

what are the diagnostics for granulomatosis with polyangitis

A

blood
creatinn and BUN high

PR3 - ANCA / C-anca

normocytic normochromic anemia

chest x ray - multiple bilateral cavitating nodular lesions

urinalyisis
nephritic sdiment
and typical nephritic syndrome

biopsy = NECESSARY TO CONFIM BIOPSY
necrotic granulomatous vasculitis of small and medium sized

28
Q

WHAT IS THE TREATMNET FOR WEGNER disease ?

A

if mild
glucocrticoids plus methotrexate / cyclophosphamide / rituximab

moderate to severe
glucocorticoids with cyclophosphomide . rituximab

glucocrticoids tapered as soon as they respond

remission maintenace - azathioprine , rituximab and methotrexate = should be considered for a year due to the high relapse

29
Q

what is the cause for good pasture syndrome ?

A

exposure to organic solvents - chloroform

  • tobacco
  • influenza a
30
Q

what is the pathophysiology of good pasture syndrome ?

A

abnormal plasma cell production of anti - GBM - against collagen type 4

anti -GBM attack the alveoli and glomeruli basement membrane

attach to the epitope and activate the complement cascade

31
Q

what are the clinical manifestations of good pasture syndrome

A

lungs antedate the kidney symptoms
hemoptysis
cough
dyspnea

nephritic syndrome symptoms

32
Q

what is the diagnosis of good pasture’s disease ?

A

renal biopsy immunofluorescence
prescence of anti GBM antibodies specifically showing a strong linear ribbon-like appearance

also can have c-ANCA = antedates

x ray - alveolar haemorrhages

33
Q

what is the treatmnet for goodpasturedisease ?

A

plasmapheresis

immunosuppressants = cyclophosphamide , prednisone , rituximab - prevent new anti-GBM antibodies

azathioprine for maintenance

34
Q

what is the pathophysioogy of membranoproliferative disorders of type 1

A

type 1 - most common
circulating immune complexes - caused by hep b or hep c
over time they build in the glomerulus and activate CLASSICAL complement pathway

or

inapporpriate activation of complement pathway by the alternate complement pathway - C3 is converted to c3a and c3b by enzyme c3 converts

this can be due to =
> mutation

> autoanitbodies against protein that regulate the process

> c3 convertase only exist for a short time = inapropriate activation of IGg that bind to C3 converatse - making c3 converatse more stable
that special type of IgG is called nephritic factor

HERE NO IMMUNE COMPLEXES

the immune complexes or the complement end up in the sub endothelium of the basement membrane - recruiting inflammatory cells - damaging the capillary wall

and thickneing of the basement membrane

triggers mesangial cells - to proliferate and reach through the thick basment membrane = mesangial interposition causing the basemnet membrane to split around the basement membrane = DUPILICATION OF BASEMENT MEMBRANE OR TRAM TRACK

35
Q

what is the pathophysioogy of membranoproliferative disorders type 2

A

in type 2 membranoproliferative GN there is no IMMUNE COMPLEXES - but complement deposits

dense deposits DISEASE or C3GN

activation of the alternate complement pathway

IgG auto - nephritic factor involved

c3GN - not intramembranous

DDD is more aggressive than C3GN

c3 levels are usually lo

36
Q

what is the pathophysiology of membranoproliferative GN of type 3

A

VERY RARE

like type 1 involves immune complexes and and complements
deposited in the subendothelial space ANDsubepithelial sapce too

37
Q

why should membranoproliferative GN not be confused with membranous gn ?

A

both have basement membrane thickening however mebranoproliferative Gn there is also mesangium thickening

38
Q

what are the clinical features specific for membranoproliferative GN type 2

A

develop drusen - deposts within the bruch’s membrane and beneath the retinal epithelium = lipodystrophy =over time vision deteriorates , macular detachment

39
Q

what is membranoproliferative GN associated with ?

A

type 1 immune complex mediated :

hep C / B associated nephropathy
hcvasociated cyroglobulinemia

monoclonal gammopathies

chronic bacterial - endocarditis

autoimmune
SLE

40
Q

what is the diagnosis for membranoproliferative disorders

A

CAN HAVE BOTH NEPHROTIc AND NEPHRITIC SYNDROME - most common

renal biopsy

LM -
MPGN is derived from two histological changes
1_ glomerular basmenet membane thicening
2_increase mesangial and endocapillary proliferation giving lobular appearance f the glomerular tuft

type 1 -
silver staining we can see tram tracking

immunoflorescence findings
type 1 = igG and C3
type 2 = C3 alone NO immunoglobulin
type 3 = IgG and C3

EM =
type 1
subendothelila and mesangial immune deposits and tram track appearance

type 2 -
DDD dense
ribbon like appearance of the subendothelilal space of the basement membrane and mesangium
INTRAMEMBRANOUS depositions

c3GN = not dense deposition and not intamembranous
light depositions in mesangium , sub endothelium

type 3 = sub endothelia and mesangial and subepithelial

41
Q

how can we know if MPGN is induced by secondary causes?

A

hep C
granular depositionof IgM and C3 and BOTH kappa and lambda light chains
IgG may or may not be present and C1q is typically negative

similar pattern for viruses

monoclonal gammapathies - monotype kappa or lambda light chains

autoimmune - full house pattern

EM - cyropercipitates in mesangium shows fingerprint pattern

42
Q

how do we know if MPGN is triggered by autoimmune disease ?

A

full house patten of Ig deposotion - IgG and Igm , iGa , c1q , c3 , kappa andlambda light chains

43
Q

how do we know if MPGN is triggered by monoclonogammpoathy

A

one monoclonal antibodies sound with C1q , C3 and light chain restricts

44
Q

what is the mangement of MPGN?

A

immune complex mediated MPGN - should be evaluated for following disorders
such as hep b or c serology
chronic bacterial infections in blood cultures
test for fungi
test for parasitic infections

autoimmune disease screening

monoclonal gammopathy : serum protein electrophoresis , multiple myeloma , low grade b cell lymphoma

=====
compliment mediated MPGN

measure c3 , c4
genetic analysisi of mutation
normal C3 levels do not rule out COMPLIMENT MEDIATED MPGN

=========

give treatment to the underlying disease then treatment for only 3 conditions remain

idiopathic immune complex mediated MPGN
C3GN
DDD

=======
IICM MPGN

normal GFR and non nephrotic range proteinurea = ACEI, regular follow up

GFR LOW /NEphrotic range proteinurea / severe histological changes - CRESCENTS /
progressive even with ACEI

prednisolone and ACEI

add cyclophosphamide if no response

no response to cyclo = stop it and go rituximab

==========
c3GN / DDD idiopathic

autoantibodies to complement protein C3nef positive= glucocorticoids and rituximab

combination therapy with corticosteroids and cyclophosphamide.
 Methylprednisolone 3 day pusle treatment 10-15 mg/kg, after which the patient received a conventional dose of prednisone

plasma exchange with albumin

genetic mutation in the complement pathway - benefit from treatment stopping mac from forming = eculizumab

factor h deficiency
= plasma exchange WITH albumin

if ESRD = renal transplant

========
if nephrotic syndrome
Anti-aggregant therapy includes Dypiridamol along with Aspirin.
 At severe nephrotic syndrome it is appropriate to administer Heparin

45
Q

what is RPGN ?

A

characterized by rapid destruction of the renal glomeruli that often leads to end-stage renal disease

46
Q

what is Eosinophilic granulomatosis with polyangiitis

A

characterized by necrotizing granulomatous vasculitis with eosinophilia, commonly involves the lungs and the skin

but can also affect the renal, cardiovascular, gastrointestinal, and peripheral nervous systems

47
Q

what is the Etiology of eosinophilic granulomatosis with polyangiitis

A

unknownmaybe montelucast triggere

48
Q

what are the clinical features ofeosinophilic granulomatosis with polyangiitis

A

Severe allergic asthma attacks (chief complaint)

Allergic rhinitis/sinusitis

Skin nodules, palpable purpura

nephritic syndrome

CNS: impaired mental status, mononeuritis multiplex (loss of motor and sensory function, with wrist or foot drop)

Pericarditis symptoms

Gastrointestinal involvement: bleeding , perforation

49
Q

diagnosis of eosinophilic granulomatosis with polyangiitis

A

nephritic syndrome

Peripheral blood :
eosinophilia

↑ IgE level

Circulating MPO /pANCA

Biopsy (confirmatory test)
Tissue eosinophilia
Necrotizing vasculitis, and necrotizing granuloma

50
Q

treatment for Churg-Strauss syndrome ?

A

immunosuppression with glucocorticoids; possible in combination with cyclophosphamide

51
Q

diagnosis of RPGN ?

A

nephritic syndrome

biopsy : crescent moon - epithelial cell proliferation that displaces the glomerulus

52
Q

always suspect RPGN and initiate testing immediatelywhen ?

A

serum creatinine rises rapidly due to renal damage

nephrology (16 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions