glomerulonephritis Flashcards

1
Q

definition of glomerulonephritis

A

immunologically mediated inflammation of renal glomeruli.

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2
Q

what does glomerulonephritis encompass

A

are caused by pathology in the glomerulus

present with proteinuria, haematuria, or both

are diagnosed on a renal biopsy

cause CKD

can progress to kidney failure - except minimal change disease

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3
Q

spectrum of glomerulonephritis

A

glomerulonephritides present on a spectrum from nephrosis (proteinuria due to podocyte pathology) to nephritis (haematuria due to inflamm damage)

if glomerulonephritis causes scarring, proteinuria can happen - therefore proteinuria can complete the longer term clinical picture of any glomerulonephritis - even those that are classically nephritic

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4
Q

aetiology of glomerulonephritis

A

deposition of antigen–antibody immune complexes in the glomeruli that lead to inflammation and activation of complement and coagulation cascades.

immune complexes either form within glomerulus or are deposited in there by circ

ag unknown - occaisionally associated with:

infection

bacterial - Streptococcus viridans, group Ab-haemolytic streptococci, staphylococci, gonococci, Salmonella, syphilis.

viral - Hepatitis B/C, HIV, measles, mumps, EBV, VZV, coxsackie.

protazoal - plasmodium malariae, schistosomiasis, filariasis

inflammatory/systemic disease

  • SLE
  • systemic vasculitis
  • cyroglobinaemia (Hepatitis B/C, HIV, measles, mumps, EBV, VZV, coxsackie.)

drugs - gold, penicillamine

tumours - Classified based on the site of nephron pathology and its distribution.

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5
Q

post-streptococcal glomerulonephritis

A

after a throat (2wk) or skin (3-6wk) infection

Streptococcal antigen deposits in the glomerulus leading to immune complex formation and infl ammation

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6
Q

minimal change glomerulonephritis

A

25% of adult nephrotic syndrome

idiopathic/drugs - NSAIDS, Lithium

paraneoplastic - haematological malignancy - usually hodgkin’s lymphoma

doesnt cause renal failure

normal appearance on light microscopy

electron microscopy = loss of epi foot processes

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7
Q

membranous glomerulonephritis

A

25% of adult nephrotic syndrome

Thickening of GBM from immune complex deposition. Associated with Goodpasture’s syndrome

primary - idiopathic

secondary

  • malignancy - lung, breast, GI, prostate, haematological
  • infection - hep B/C, strep, malaria, schistosomiasis
  • immunological disease - SLE, RA, sarcoidosis, Sjogren’s
  • drugs - gold, penicillamine
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8
Q

membraneoproliferative glomerulonephritis (MPGN)

A

10% of adult nephrotic syndrome (higher in low- and middle-income countries due to infection).

thickening of GBM, mesangial cell proliferation and interposition

Type 1: Subendothelial immune complex deposits and reduplication of GBM.

  • immune complexes deposit in skin and activate complement
  • underlying cause - infection, cryoglobulinaemia, monoclonal gammopathy, autoimmunity

Type 2: Dense intramembranous deposits (stain only for C3).

  • genetic or acquired defect in alternative complement pathway eg C3 nephritic factor
  • progressive kidney dysfunction is uncommon
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9
Q

focal segmental glomerulonephrosis

A

commonest GN seen on biopsy

primary - idiopathic

secondary - HIV, heroin, lithium, lymphoma, any cause of reduced kidney mass/nephrons, kidney scarring

all pts at risk of CKD and kidney failure

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10
Q

focal segmental proliferative glomerulonephritis

A

Mesangial and endothelial cell proliferation.

Focal refers to involvement of some of the glomeruli,

segmental refers to involvement of parts of individual glomeruli.

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11
Q

Diffuse proliferative glomerulonephritis

A

Mesangial and endothelial cell proliferation.

affects all glomeruli.

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12
Q

IgA nephropathy

A

Mesangial cell proliferation and mesangial IgA and C3 deposits.

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13
Q

Henoch–Schönlein purpura (HSP)

A

small vessel vasculitis and systemic variant of IgA nephropathy with IgA deposition in skin/joints/gut in addition to kidney

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14
Q

Crescentric glomerulonephritis:

A

Crescent formation by macrophages and epithelial cells,

which fills up Bowman’s space.

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15
Q

Focal segmental necrotizing glomerulonephritis:

A

Peripheral capillary loop necrosis (e.g. inWegener’s granulomatosis, microscopic polyarteritis and other vasculitides).

Often evolves into crescentric glomerulonephritis.

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16
Q

anti-glomerular basement membrane disease

A

previously known as goodpasture’s disease

rare

auto-ab to type IV collagen in glomerular and alveolar BM

17
Q

rapidly progressive glomerulonephritis

A

any aggressive GN - rapidly progressing to renal failure over days or weeks

Causes include small vessel/ANCA vasculitis, lupus nephritis, anti-GBM disease. Other GNs may ‘transform’ to become rapidly progressive including IgA, membranous.

18
Q

epidemiology of glomerulonephritis

A

Makes up to 25% of cases of chronic renal failure.

IgA nephropathy - commonest primary GN in high-income countries

19
Q

sx of glomerulonephritis

A

haematuria

subcutaneous oedema

polyuria or oliguria

proteinuria

history of recurrent infection

Symptoms of uraemia or renal failure (acute and chronic).

20
Q

sx of IgA nephropathy

A

asymptomatic

non-visible haematuria

episodic visible haematuria - may be ‘synpharyngitic’: within 12–72h of infection

high BP

proteinuria <1g

slow, indolent disease - 20-50% progress to renal failure over 30yrs

21
Q

sx of HSP

A

purpuric rash on extensor surfaces - typically on legs

flitting polyarthritis

abdo pain - GI bleeding

nephritis

22
Q

sx of post-streptococcal glomerulonephritis

A

varies from haematuria to acute nephritis (haematuria, oedema, HTN, oliguria)

23
Q

sx of anti-glomerular basement membrane disease

A

renal disease - oliguria/anuria, haematuria, AKI, renal failure

lung disease - pul haemorrhage in 50-90% = SOB, haemoptysis

24
Q

sx of glomerulonephritis

A

HTN

proteinuria <3g/24hr

haematuria (microscopic or macroscopic, especially IgA nephropathy);

nephrotic syndrome

nephritic syndrome - haematuria, proteinuria, subcutaneous oedema, oliguria, hypertension, uraemia);

renal failure - acute/chronic

partial lipodystrophy (loss of subcutaneous fat in MPGN type II).

25
Q

Ix for glomerulonephritis

A

blood

urine

imaging - renal tract US and CXR

renal biopsy

hep B, C, HIV serology

26
Q

bloods for glomerulonephritis

A

FBC

UE

CRP

creatinine

LFT - albumin

lipid profile

electrophoresis

complement studies -C3, C4, C3 nephritic factor in MPGN

ANA

anti-double stranded DNA

ANCA

anti-GBM Ab

cyroglobins if appropriate

culture

27
Q

urine for glomerulonephritis

A

microscopy - dysmorphic RBC, red cell casts

24hr collection - creatinine clearance, protein

MC&S,

Bence Jones protein

28
Q

imaging for glomerulonephritis

A

renal tract US - exclude other path. Size and anatomy for biopsy

CXR - pul haemorrhage

29
Q

renal biopsy for glomerulonephritis

A

light microscopy, electron light microscopy, immunofluorescence microscopy

needed for diagnosis

30
Q

Ix for IgA nephropathy

A

biopsy - IgA deposition in mesangium

31
Q

Ix for HSP

A

diagnosis clinical

positive IF for IgA and C3 in skin

renal biospy same as IgA nephropathy

32
Q

Ix for post-strep glomerulonephritis

A

evidence of strep infection

  • high ASOT
  • high anti-DNAse B
  • low C3
33
Q

Ix for anti-GBM glomerulonephritis

A

anti-GBM in circ/kidney

34
Q

Ix for rapidly progressive glomerulonephritis

A

breaks in GBM allow influx of inflamm cells so that cresents are seen on renal biopsy - may be referred to as cresentic GN

35
Q

Ix for minimal change disease

A

light microscopy normal

electron microscopy - effacement of podocyte foot processes

36
Q

Ix for FSGS

A

glomeruli have scarring of certain segments (ie focal sclerosis)

may miss early disease if <10 glomeruli in biopsy sample

37
Q

Ix for membranous nephropathy

A

Anti-phospholipase A2 receptor antibody in 70–80% of idiopathic disease.

Diffusely thickened GBM due to subepithelial deposits (IgG4 dominant in idi-opathic, other IgGs in secondary disease).

‘Spikes’ on silver stain

38
Q

Ix for membranoproliferative glomerulonephritis

A

a proliferative glomerulonephritis with electron dense deposits

immunoglobin deposition distinguishes immune-complex-associated disease from C3 glomerulopathy