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

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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)

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3
Q

What are the secondary factors causing deposition of antigens?

A

NSAID HSP

  • neoplasm
  • SLE
  • amyloid
  • infection
  • diabetes
  • Henoch Schonlein Purpura
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4
Q

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

A

Endothelial cell proliferation
Capillary wall necrosis
Glomerulosclerosis

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5
Q

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

A

Thickened membrane –> structural distortion –> increased permeability

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6
Q

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

A

Deposition of cells in Bowman’s space

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7
Q

What are the three layers of the glomerulus?

A

Fenestrated capillary epithelium
Basement membrane
Visceral layer (interdigitating podocytes)

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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
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9
Q

How can glomerular damage/glomerulonephritis present?

A
AKI (if initially severe enough)
CKD
Asymptomatic haematuria
Nephrotic syndrome
Nephritic syndrome
Rapidly progressive glomerulonephritis
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10
Q

What are the stages of progression of glomerulonephritis?

A

Asymptomatic dipstick abnormalities
Nephrotic syndrome
Nephritic syndrome
ESRD

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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
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12
Q

Why is urine protein:creatinine ratio used?

A

More convenient than 24hr urinary protein & equally accurate

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13
Q

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

A
50-100mg/mmol = significant proteinuria
>300mg/mmol = nephrotic range
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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
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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

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16
Q

How does IgA nephropathy present?

A

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

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17
Q

How should suspected IgA nephropathy be investigated?

A

Renal biopsy (IgA/C3 deposits)

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18
Q

How should IgA nephropathy be managed?

A

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

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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
Q

How should membraneous nephropathy be managed?

A

Alternating steroids & cyclophosphamide

-25% progress to ESRD in 10yrs

26
Q

What is focal segemental glomerulosclerosis?

A

Idiopathic areas of segmental sclerosis

27
Q

How should focal segmental glomerulosclerosis be investigated?

A

Renal biopsy (IgM & C3 deposits)

28
Q

How should focal segmental glomerulosclerosis be managed?

A

Poor response to treatment

29
Q

What is membranoproliferative glomerulonephritis?

A

Cause of nephrotic OR mixed nephrotic/nephritic syndrome

30
Q

How should membranoproliferative glomerulonephritis?

A

Renal biopsy (large glomeruli w/ double BM)

31
Q

How should membranoproliferative glomerulonephritis be managed?

A

No proven treatment
50% develop ESRD in 10yrs
High recurrence rate in transplants

32
Q

How does post-streptococcal glomerulonephritis present?

A

Nephritic syndrome 1-2wks post sore throat/skin infection

33
Q

How should suspected post-streptococcal glomerulonephritis be investigated?

A
Renal biopsy (diffuse proliferative GN w/ IgG &amp; C3 deposits)
   -no need to biopsy in typical cases
Bloods (raised ASOT/anti-DNAse B, reduce complement)
34
Q

What is HSP?

A

Small vessel vasculitis & nephritic syndrome post URTI
-typically affects children 3-15yrs
Severe end of a spectrum starting w/ IgA nephropathy

35
Q

How does HSP present?

A
Purpuric rash on extensor surfarces
Polyarthritis
Abdo pain (GI bleeding)
Scrotal/scalp swelling
GN
36
Q

How should suspected HSP be investigated?

A

Clinical diagnosis

Confirm w/ +ve immunofluorescence in skin/renal biopsy

37
Q

How should HSP be managed?

A

Attacks typically self limiting

If relapses/evidence of progression give corticosteroids

38
Q

What is Goodpasture’s Syndrome?

A

Type II hypersensitivity reaction to type IV collagen

-present in glomerular/alveolar BM & ear/eye

39
Q

How does Goodpasture’s Syndrome present?

A

Haematuria
Rapidly progressive GN
Pulmonary haemorrhage (SOB, haemopytsis)

40
Q

How should suspected Goodpasture’s Syndrome be investigated?

A
CXR (pulmonary shadowing)
Renal biopsy (IgG deposition across glomerular BM)
41
Q

How should Goodpasture’s Syndrome be investigated?

A

Plasma exchange
Corticosteroids +/- cytotoxics
-variable prognosis

42
Q

Describe systemic vasculitis

A

Leads to focal segmental GN

ANCA +ve

43
Q

What is rapidly progressive glomerulonephritis?

A

Glomerulonephritis leadinging to ESRD over a few days

Presents w/ signs of renal failure & systemic disease

44
Q

What are the causes of rapidly progressive glomerulonephritis?

A

Immune complex disease (45%, SLE, IgA nephropathy)
Vasculitis (50%, HSP, Wegeners, Churg-Strauss)
Goodpasture’s (5%)

45
Q

How should rapidly progressive glomerulonephritis be managed?

A

Aggressive immunosuppression
-high dose steroids
-cyclophosphamide
Variable prognosis