Glomerulonephritis Flashcards

1
Q

What is Glomerulonephritis?

A

Glomerulonephritis includes a range of immune-mediated disorders that cause inflammation within the glomerulus and other compartments of the kidney

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

What is the pathological basis underlying Glomerulonephritis?

A

Immunological attack by antibody/T-cell upon antigens in glomerulus
-antigens can be primary or secondary (acquired/deposited)

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

What are the secondary factors causing deposition of antigens?

A

NSAID HSP

  • neoplasm
  • SLE
  • amyloid
  • infection
  • diabetes
  • Henoch Schonlein Purpura
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What is the pathological response to the primary immunological attack in the glomerular capillaries?

A

Endothelial cell proliferation
Capillary wall necrosis
Glomerulosclerosis

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

What is the pathological response to the primary immunological attack in the basement membrane?

A

Thickened membrane –> structural distortion –> increased permeability

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

What is the pathological response to the primary immunological attack in the tubules?

A

Deposition of cells in Bowman’s space

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

What are the three layers of the glomerulus?

A

Fenestrated capillary epithelium
Basement membrane
Visceral layer (interdigitating podocytes)

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

What words are used to describe glomerular pathology histologically?

A
Global = whole glomerulus diseased
Segmental = small patches of one glomerulus damaged
Diffuse = affects >50% glomeruli
Focal = affects <50% glomeruli
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

How can glomerular damage/glomerulonephritis present?

A
AKI (if initially severe enough)
CKD
Asymptomatic haematuria
Nephrotic syndrome
Nephritic syndrome
Rapidly progressive glomerulonephritis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What are the stages of progression of glomerulonephritis?

A

Asymptomatic dipstick abnormalities
Nephrotic syndrome
Nephritic syndrome
ESRD

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

What investigations are appropriate in suspected glomerulonephritis?

A
Bloods (FBC, U&amp;Es, CRP, culture)
Urine dip (?infec)
MCS (red cells/casts)
Urine protein:creatinine ratio
Nephritic screen
Renal USS + biopsy
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Why is urine protein:creatinine ratio used?

A

More convenient than 24hr urinary protein & equally accurate

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

What random protein:creatinine ratio would be suggestive of disease?

A
50-100mg/mmol = significant proteinuria
>300mg/mmol = nephrotic range
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What are the main pathological processes affecting the glomerulus?

A
IgA nephropathy (Beurger's disease)
Minimal change nephropathy
Membranous nephropathy
Focal segmental glomerulosclerosis
Membranoproliferative GN
Post-streptococcal GN
HSP
Goodpasture's syndrome
Systemic vasculitis
Rapidly progressive GN
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What is IgA nephropathy (Beurger’s disease)?

A

Lower end of a spectrum ending in HSP

Most common cause of GN in adults

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

How does IgA nephropathy present?

A

Mostly affects young males, often after an URTI
Haematuria (macroscopic)
Nephritic syndrome

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

How should suspected IgA nephropathy be investigated?

A

Renal biopsy (IgA/C3 deposits)

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

How should IgA nephropathy be managed?

A

Supportive
Steroids may slow decline in renal function
-20% progress to ESRD over 20yrs

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

What is minimal change nephropathy?

A

Most common cause of GN in children (75%)

Common cause of nephrotic syndrome in adults (20%)

20
Q

What are the causes of minimal change nephropathy?

A

NSAID
Allergy
Hodgkin’s lymphoma

21
Q

How should suspected minimal change nephropathy be investigated?

A
Light microscopy (normal)
Immunofluorescence (negative)
Electro-microscopy (fusion of podocyte foot processes)
22
Q

How should minimal change nephropathy be managed?

A

Oral steroids
-99% of cases resolve in 4-6wks
-1% of cases progress to ESRD
Cyclosporin

23
Q

What is membranous nephropathy?

A

Rare cause of nephrotic syndrome

-80-90% idiopathic

24
Q

How should membranous nephropathy be investigated?

A

Renal biopsy (global diffuse GN w/ IgG & C3 deposits)

25
How should membraneous nephropathy be managed?
Alternating steroids & cyclophosphamide | -25% progress to ESRD in 10yrs
26
What is focal segemental glomerulosclerosis?
Idiopathic areas of segmental sclerosis
27
How should focal segmental glomerulosclerosis be investigated?
Renal biopsy (IgM & C3 deposits)
28
How should focal segmental glomerulosclerosis be managed?
Poor response to treatment
29
What is membranoproliferative glomerulonephritis?
Cause of nephrotic OR mixed nephrotic/nephritic syndrome
30
How should membranoproliferative glomerulonephritis be investigated?
Renal biopsy (large glomeruli w/ double BM)
31
How should membranoproliferative glomerulonephritis be managed?
No proven treatment 50% develop ESRD in 10yrs High recurrence rate in transplants
32
How does post-streptococcal glomerulonephritis present?
Nephritic syndrome 1-2wks post sore throat/skin infection
33
How should suspected post-streptococcal glomerulonephritis be investigated?
``` Renal biopsy (diffuse proliferative GN w/ IgG & C3 deposits) -no need to biopsy in typical cases Bloods (raised ASOT/anti-DNAse B, reduce complement) ```
34
What is HSP?
Small vessel vasculitis & nephritic syndrome post URTI -typically affects children 3-15yrs Severe end of a spectrum starting w/ IgA nephropathy
35
How does HSP present?
``` Purpuric rash on extensor surfarces Polyarthritis Abdo pain (GI bleeding) Scrotal/scalp swelling GN ```
36
How should suspected HSP be investigated?
Clinical diagnosis | Confirm w/ +ve immunofluorescence in skin/renal biopsy
37
How should HSP be managed?
Attacks typically self limiting | If relapses/evidence of progression give corticosteroids
38
What is Goodpasture's Syndrome?
Type II hypersensitivity reaction to type IV collagen | -present in glomerular/alveolar BM & ear/eye
39
How does Goodpasture's Syndrome present?
Haematuria Rapidly progressive GN Pulmonary haemorrhage (SOB, haemopytsis)
40
How should suspected Goodpasture's Syndrome be investigated?
``` CXR (pulmonary shadowing) Renal biopsy (IgG deposition across glomerular BM) ```
41
How should Goodpasture's Syndrome be managed?
Plasma exchange Corticosteroids +/- cytotoxics -variable prognosis
42
Describe the effect of systemic vasculitis on the kidney
Leads to focal segmental GN | ANCA +ve
43
What is rapidly progressive glomerulonephritis?
Glomerulonephritis leadinging to ESRD over a few days | Presents w/ signs of renal failure & systemic disease
44
What are the causes of rapidly progressive glomerulonephritis?
Immune complex disease (45%, SLE, IgA nephropathy) Vasculitis (50%, HSP, Wegeners, Churg-Strauss) Goodpasture's (5%)
45
How should rapidly progressive glomerulonephritis be managed?
Aggressive immunosuppression -high dose steroids -cyclophosphamide Variable prognosis