Case 23 - Glomerulonephritis and PKD Flashcards

1
Q

What are glomerular diseases?

A

Can be due to a variety of reasons

Diseases in which the function of the glomerulus has been reduced

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2
Q

As what syndromes can glomerular disease present?

A
Isolated haematuria/proteinuria/both
RPGN
Acute nephritic syndrome
Nephrotic syndrome
CKD - and eventually ESRD
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3
Q

Investigations for glomerular diseases?

A

Urine dip - for protein and blood (dysmorphic on film)
Quantigfy protein with ACR

FBC
U and E
Bone profile
Clotting
HbA1c
CRP
Immunology
Lipid profile
LFTs

USS kidney
Kidney biopsy

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4
Q

What is nephritic syndrome?

A

Combination of:

  • Haematuria
  • HTN
  • Proteinuria
  • Oligouria

Can be caused by a variety of diseases

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5
Q

What diseases can cause nephritic syndrome?

A

IgA nephropathy
PSGN
RPGN

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6
Q

What is IgA nephropathy?

A

Classically seen 1-3 days after a throat infection (synpharyngitic GN)
The antibodies made to combat the throat infection start to attack the kidney
IgA deposited in the kidney
In biopsy will see clumps of IgA in the mesangium
May present with Henoch Schonlein Purpura which is systemic vasculitis

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7
Q

What is PSGN?

A

Post-streptococcal GN
Presents 1-2 weeks after a throat infection
Either the antibodies get lodged in GBM, or the antigens do
Inflammatory cells cause damage to the glomerulus

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8
Q

What is RPGN?

A

Rapidly progressive GN
Can present for a number of reasons
Sudden deterioration in kidney function due to GN
Crescentic GN

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9
Q

What are the types of vasculitis?

A

Categorised based on vessel size - large, medium and small

Smalls vessels :
ANCA positive
-Wegeners granulomatosis
-Microscopic polyangitis
-Churg Strauss syndrome
-Pauci-immune GN

Induce remission with cyclophosphamide and steroids and maintain with azothioprine and steroids

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10
Q

How can vasculitis present?

A
Nasal crusting
RPGN
Epistaxis
Rashes e.g. Henoch schonlein purpura
Joint pain
Myalgia
Haemoptysis
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11
Q

What is nephrotic syndrome?

A

Combination of:

  • Proteinuria >3.5g/day
  • Oedema
  • Hypercholesterolaemia
  • Hypoalbuminaemia
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12
Q

What are the types of nephrotic syndrome on biopsy?

A

Minimal change
Focal segmental glomerular sclerosis
Membranous GN
Membranoproliferative GN

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13
Q

Causes of nephrotic syndrome

A

Primary is idiopathic

Secondary - can be a variety of conditions that can cause nephrotic syndrome:
Infections
Drugs
Tumours
Autoimmune
Heavy metals
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14
Q

Treatment of nephrotic syndrome

A

Salt restriction
Fluid restriction
Diuretics for the oedema
ACE-i/ARB
Biopsy to check for histology which may help direct serology for the cause
Treat the cause
Prophylaxis of renal vein thrombosis due to urinary loss of AT-III

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15
Q

Causes of RPGN?

A

Type 1 -anti-GBM mediated
Ab to GBM are formed and attack the GBM
Can present as part of Good-Pastures syndrome - anti-GBM + Haemoptysis + haematuria

Type 2 - immune complex mediated
Antibodies and antigens clog up the glomerulus and cause damage
e.g. IgA nephropathy, PSGN

Pauci immune - when there is no immune complex deposition
This is triggered by ANCA instead
Could be Wegeners or microscopic polyangitis etc.

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16
Q

What are some genetic cystic kidney diseases?

A

Autosomal domininat:
ADPKD
Von Hippel Lindau syndrome

Autosomal recessive:
ARPKD

X-linked:
Chromosomal disorders

17
Q

What are some non-genetic cystic kidney diseases?

A

Developmental:
Medullary sponge kidney
Multicystic dysplastic kidney

Acquired:
Acquired cystic kidney disease - dialysis
Simple cysts
Hypokalaemia-related cysts

18
Q

What is von Hippel Lindau disease?

A

Autosomal dominant cystic kidney disease
Mutated gene has a role as a TSG
Increased risk of renal Ca
Can treat with surgery so should monitor with USS and CT

19
Q

What is PKD?

A

Makes up 10% of dialysis population
Most common cystic kidney disease
Adult PKD has triad of liver cysts, renal cysts and berry aneurysms

20
Q

What is ADPKD?

A

Less severe form
More common
Mutations in PKD1 and PKD2 genes

21
Q

What is ARPKD?

A

More severe form
Diagnosed in childhood
Liver fibrosis essential to diagnose

22
Q

Imaging for PKD?

A

USS - to assess cyst size and number on each kidney
CT - may be able to see liver cysts too
MSU - proteinuria, the higher the risk this is, the higher the risk of progressing to CKD

23
Q

Management for PKD

A
Fluids and monitor CKD
Control HTN from childhood
Family screening
CV risk control
Treat CKD when it comes
Tolvaptan can slow the progression - but need to drink high levels of fluids
Nephrectomy for severe pain
24
Q

Treatment for RPGN

A

Anti-coagulants to reduce fibrin deposition
Plasmaphoresis and immunosuppressants in combination
RRT