Nephrotic Syndrome incl. Nephrotic Glomerulonephritis Flashcards

1
Q

What are the 3 key features of nephrotic syndrome?

A

1) Proteinuria
2) Hypoalbuminaemia
3) Oedema

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2
Q

What level of hypoalbuminaemia suggests nephrotic syndrome?

A

< 30

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3
Q

What level of proteinuria suggests nephrotic syndrome?

A

> 3g/24h

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4
Q

What happens in nephrotic syndrome?

A
  • Renal disease - primary or secondary to systemic disorder
  • Proteinuria due to podocyte pathology
  • Generalised pitting oedema (rapid, severe) incl. periorbitally
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5
Q

What are the complications of nephrotic syndrome?

A
  • Thromboembolism
  • Infection
  • Hyperlipidaemia
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6
Q

What is nephrosis?

A

Proteinuria due to podocyte pathology

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7
Q

What is the most common cause of nephrotic syndrome in children?

A

Minimal change disease

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8
Q

What are causes of minimal change disease?

A
  • Idiopathic (most common)
  • Drugs e.g. lithium, NSAIDs
  • Paraneoplastic esp. Hodgkin’s lymphoma
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9
Q

How do you diagnose minimal change disease?

A
  • Electron microscopy - shows effacement of podocyte foot processes
  • Light microscopy is normal
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10
Q

How do you treat minimal change disease?

A

Prednisolone (1mg/kg 4-16 weeks) - give steroids w/o biopsy in children (biopsy only if no response or signs suggestive of other cause)
- If frequent relapses - cyclophosphamide or calcineurin inhibitors (longer term immunosuppression)

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11
Q

What is the most common primary cause of nephrotic syndrome in adults?

A

FSGS

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12
Q

What are the causes of FSGS?

A
  • Idiopathic (primary)
  • HIV
  • Heroin
  • Lithium
  • Lymphoma
  • Any cause of decreased kidney mass/nephrons
  • Kidney scarring due to other glomerulonephritis
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13
Q

What are the risks of FSGS?

A

Progressive CKD and kidney failure (more proteinuria worsens prognosis) - disease will recur in 30-50% of kidney transplants

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14
Q

How do you diagnose FSGS?

A

Renal biopsy - glomeruli scarring of certain segments (focal sclerosis)

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15
Q

How do you treat FSGS?

A
  • ACE-i/ARB and BP control

- Corticosteroids in idiopathic disease - calcineurin inhibitors second line

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16
Q

How do you treat recurrent FSGS in transplants?

A
  • Plasma exchange

- Rituximab

17
Q

What is the most common glomerulonephritis seen on renal biopsy?

A

FSGS

18
Q

What is important to remember about minimal change disease?

A

It does not cause renal failure - if progressive CKD consider missed FSGS

19
Q

What are causes of membranous nephropathy?

A
  • Idiopathic (primary)
  • Malignancy
  • Infection
  • Autoimmune disease
  • Drugs
20
Q

How do you diagnose membranous nephropathy?

A
  • Anti phospholipase A2 receptor antibody in 70-80% of idiopathic disease
  • Diffusely thickened GBM due to sub epithelial deposits (mainly IgG4 in idiopathic)
  • ‘Spikes’ on silver stain
21
Q

How do you treat membranous nephropathy?

A
  • ACE-i, ARB and BP control

- Corticosteroids + cyclophosphamide if risk of progression

22
Q

What are the two types of membranoproliferative glomerulonephritis?

A

1) Immune-complex associated - Increased/abnormal immune complex deposition in kidney activates complement
2) C3 glomerulopathy - genetic or acquired defect in alternative complement pathway

23
Q

What is membranoproliferative glomerulonephritis?

A

C3 glomerulopathy - genetic or acquired defect in alternative complement pathway

24
Q

How do you diagnose membranoproliferative glomerulonephritis?

A
  • Proliferative glomerulonephritis with electron dense deposits
  • Ig deposition distinguishes immune complex associated disease from C3 glomerulopathy
25
Q

How do you treat membranoproliferative glomerulonephritis?

A
  • ACE-i/ARB and BP control

- Immunosuppression

26
Q

What are underlying cause of immune-complex associated membranoproliferative glomerulonephritis in adults?

A
  • Infection
  • Cryoglobulinaemia
  • Monoclonal gammopathy
  • Autoimmunity
27
Q

What are secondary causes of nephrotic syndrome?

A
  • Diabetes
  • Lupus nephritis
  • Myeloma
  • Amyloid
  • Pre-eclampsia
28
Q

What is first line investigation for nephrotic syndrome?

A

Urine dipstick (if oedema)

29
Q

How do you manage nephrotic syndrome?

A
  • Reduce oedema - fluid and salt restriction, loop diuretics e.g. furosemide (thiazide if resistant to high dose loop)
  • Treat underlying cause - adults need renal biopsy, look for underlying condition
  • Reduce proteinuria - ACE-i/ARB