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Renal / Urology > Glomerulonephritis + PCKD > Flashcards

Glomerulonephritis + PCKD Flashcards

1
Q

What is glomerulonephritis

A

Disease of the glomerulus that presents as a spectrum from nephrosis to nephritis

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2
Q

What is nephritic syndrome

What is seen on microscopy

If proteinuria…?

A

Haematuria
Hypertension + fluid retention causing intravascular overload
Oliguria / AKI / renal failure

On microscopy
Dysmorphic RBC
RBC cast = pathopneumonic

Proteinuria can occur if GN causes scarring
If >3g in 24 hours tends to be more nephrotic

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3
Q

What conditions cause nephritic syndrome

A 
Post strep 
IgA = most common worldwide 
Focal necrotizing
Membraneous 
Anti-GBM 
Autoimmune - SLE / vasculitis A
Mesnagiocapillary 
- Hep C
- Cyroglobulinaemia 
Alport
HSP 
HUS
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4
Q

What is nephrotic syndrome

When do you beware

A

Proteinuria >3g
Hypoalbuminaemia <30g/l
Oedema due to change in Starling forces causing intravascular depletion - no protein to keep water
- Unusualy for HF to cause facial and peri-oorbtial oedema

Can have hypertension wth the conditions that cause so beware if resistant + low albumin

Will have low Na as intravascular dry but increased TBW due to oedema

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5
Q

What are complications of nephrotic

A 
Hyperlipidaemia - lose lipoprotein 
VTE as lose anti-thrombin / protein C+S
- Pain / haematuria if renal thrombosis 
Hypertension 
Immune deficiency - lose Ig
Hypocalcaemia - lose vit D 
Acute renal failure
Increased CVS risk / anaemia
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6
Q

What is nephrotic often mis Dx as / how do you investigate it

A

HF or liver failure
Always do dipstick if suspect this

MSSU 
U+E
Albumin
Urine PCR
Clotting
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7
Q

What conditions cause nephrotic

What are congenital causes and how are they inherited

A 
Minimal change
Membraneous 
FSGS - if unresponsive to minimal change Rx 
Diabetic nephropathy
Amyloid

Congenital

  • Alport - XL
  • Thin BM disease - AD
  • Podocin / nephron damage - AR
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8
Q

What can VTE cause

A

Renal vein thrombosis

  • Pain
  • Haematuria
  • Decreased renal function
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9
Q

What is needed in nephrotic

A

Statin
Anti-coagulation
Ax prophylaxis / Ig if exposed
Accurate fluid balance

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10
Q

How do you investigate GN

A 
Bloods - FBC, U+E, LFT, CRP, Ig, complement, clotting, albumin 
Autoimmune - auto Ab, ANA, ANCA, anti-dsDNA, anti-GBM
Blood culture
ASO titre
Urine dip + MC+S
ACR
Renal USS
CXR
Renal biopsy =. only definite
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11
Q

When do you think of GN in respect to AKI

A

Atypical AKI
Not responding to Rx
Active urinary sediment with no evidence of infection

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12
Q

What is general management of GN

A

Immunosuppression and BP control via ACEI = main stay of Rx
Control BP
ACEI / ARB to minimise proteinuria and slow progression
Salt and fluid restriction for oedema
Diuretics if oedema - slowly to decrease risk of AKI
Manage CKD complications

Prophylaxis if nephrotic
Statin
Anti-coagulation if severe
Infection prophylaxis

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13
Q

ACEI

A

Renoprotective as dilate efferent arterioles

Also nephrotoxic

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14
Q

What causes post strep nephritis

A

10-21 days after group A strep infection
Deposts in glomerulus
Immune complex formation and inflammation

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15
Q

What are the symptoms of post strep

A 
Nephritic syndrome 
Haematuria
High BP
High creatinine / oliguria
Systemically unwell  
Proteinuria and oedema
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16
Q

How do you Dx post strep

Differentiate from IgA

A

Clinical
Strep infection - ASO titre raised
Complement decreased (differs from IgA)
Urine culture

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17
Q

How do you treat post strep

What do you suspect if complement doesn’t settle

A

Self-limiting
Loop diuretic for Sx
Control BP - ACEI
Ax questionable

If complement doesn’t settle suspect membranous GN

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18
Q

What do you do if on diuretic

A

Daily weight

Monitor U+E

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19
Q

What are complication of post strep

A

Left ventricular dysfunction due to hypertension

ESRD but rare

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20
Q

What is the commonest cause of GN

A

IgA nephropathy

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21
Q

What causes IgA nephropathy

A

Excess IgA in mesangium of capillaries

Activates C3

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22
Q

How does IgA present

A 
Variable
Presents 1-2 days after trigger 
Nephritic
Haematuria - NV or episodic visible linked to URTI 
Hypertension
High creatinine
Sometimes nephrotic or RPGN
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23
Q

Who is more at risk of IgA

A 
2nd / 3rd decade
Male
Alcohol 
Coeliac
HSP
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24
Q

What has better prognosis

A

Frank haematuria

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25
Q

How do you Dx IGA

A

Urine culture
Bloods
USS
Renal biopsy shows IgA deposition

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26
Q

How do you differentiate from post strep

A

Proteinuria in strep
Low complement in strep
Longer interval between infection with strep

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27
Q

How do you treat IgA

A

ACEI for BP and reduce protein = mainstay
BP meds aim 125/75
Kidney transplant for ESRD
Immunosuppression if rapidly progressive

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28
Q

What is the prognosis and what does it depend on

A 
1/3 ESRD over 20 yearrs
BP
Proteinuria
Hypertenion
SMoking
Male 
Kidney function
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29
Q

What is linked to IgA

A

HSP in children

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30
Q

What causes rapidly progressive GN

A 
IgA 
Post infection 
Macro granulomatosis
Microscopic polyarteritis
Anti-GBM
HSP 
SLE
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31
Q

What are features of rapidly progressive GN

A 
Nephritic 
Often present in AKI 
Haematuria
High BP
High creatinnien
Proteinuria

Features of cause

  • Fatgiue
  • Weight loss
  • Rash
  • Fever
  • Lung or nerve involvement
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32
Q

How do you Dx rapidly progressive GN

A

MSSU
Bloods
USS
Biopsy shows crescentic GN

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33
Q

What is serum test for macroscopic

A

C-ANCA

Proteinase 3 - PRE

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34
Q

What is serum test for microscopic polyarteritis

A

P-ANCA

Myeloperioxidase - MPO

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35
Q

How d you treat rapidly progressive

A

IV steroid
Immunosuppression - cyclosporine / azathioprine / cyclophosphamide
Plasma exchange if Anti-GBM / ANCA
Monoclonal Ab

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36
Q

What is prognosis

A

Good if Rx early

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37
Q

What are risks of cyclophosphamide

A

Bladder TCC

Cystitis - haemorrhage

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38
Q

Complication of steroid

A 
Growth
Infection
Obesity
Papilloedema / benign intracranial HTN 
Adrenal crisis
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39
Q

What is Anti-GBM disease

A

Ab against type IV collagen in glomerulus and basement membrane
Type 2 hypersensitivity

40
Q

What are the symptoms of Anti-GBM

A 
Hx chest sx
Oliguria
Haematuria
Rapid AKI 
Pulmonary / kidney haemorrhage = SOB and haemoptysis
41
Q

What puts you at risk of pulmonary haemorrhage

A

Male
Pulmonary oedema
LRTI
Smoking

42
Q

How do you Dx

A

CXR

Renal biopsy

43
Q

How do you treat

A

Plasma exchange to get rid of Ab
Corticosteroid
Immunosuppression - cyclophosphamide
May need transplant or dialysis if can’t get control

44
Q

What is HSP

A

Autoimmune inflammation of small blood vessels
IgA vasculitis
Common in children following infection
IgA deposition in skin, joints, gut and kidney

45
Q

What are the symptoms HSP

A

Purpuric PALPABLE rash on leg / buttock
- Menigococcal = non-palpable
Joint pain
Arthralgia
Swelling
Abdo pain / colic
- Can lead to GI haemorrhage,
- Red current jelly stool / Intussception
- Infarction
Haematuria / renal failure due to IgA nephritis
- RBC cast = pathopneumonic of nephritic
- Proteinuria
- Increased creatinine is not common but could occur

46
Q

How do you Dx and what must you excluded

A 
Clinical 
\+ve immunofluresence for IgA in skin
Biopsy = identical to IgA
Do U+E, albumin for nephrotic, ACR, BP 
Must exclude meningococcal septicaemia, leukaemia , ITP, HUS 
- FBC + film (normal platelet)
- Normal coagulation 
- Blood cultures + CRP
47
Q

How do you treat HSP and what do you do after 1 year

A 
Supportive 
NSAID - joints
Steroids if severe
Air or contrast enema for intussception + surgery 
Manage as IgA if kidney involvement 
BP lowering drugs/. ACEI

Monitor with urine dip and BP to screen or HTN and proteinuria at 1 year

48
Q

What causes minimal change disease

A 
NO INFLAMMATION 
Children / young adults usually idiopathic 
Idiopathic
Drugs - NSAID / lithium
Hodgkin
EBV
49
Q

What is most common cause of nephrotic

A

Minimal change

50
Q

What are the symptoms

A

Sudden oedema
Proteinuria
Frothy urine
Normal BP and renal function

51
Q

How do you Dx nephrotic

A

Urinanalysis
Bloods - increased creatinine and low albumin
Clotting

52
Q

When is biopsy indicated

A

No response
Other cause suspected e.g. autoimmune
Abnormal renal function

53
Q

How do you treat

A

Salt and fluid
Diuretic
Penicillin prophylaxis
Check VZV status + pneumococcus

Rx
Steroid (prednisone for 8 weeks) - taper over 3-4 months
Immunosuppression if frequent relapse, resistant to steroid to toxic
- cyclophosphamide / ccycloporin
Tell may relapse

54
Q

What do you consider if minimal change progresses to CKD or non-responsive

A

FSGS - most common cause of nephrotic in adults

Congenital nephron / podocyte deficiency if child

55
Q

What is pathology of membranous GN (most common GN overall)

A

No inflammation
IgG between lamina and podocyte in GBM
Activates complement - Ddx of post-strep not responding
Punches hole

56
Q

What causes membranous

A 
Idiopathic
Malignancy - lymphoma or solid organ
RA
Infection - hep B/C/strep / malaria/schistosomiasis / HIV 
SLE 
Amyloid
Drugs e.g. NSAID
57
Q

How does membranous present

58
Q

How do you Dx

A 
Urine MSSU
Bloods
USS
Biopsy
Focussed history / CXR / exam to look for 2 cause
59
Q

What Ab +ve in membranous

A

Anti-phospholipase A2 in primary

60
Q

How do you treat

A 
General nephrotic
ACEI
Diuretic
Immunosuppression if idiopathic
Steroid 
Rituximab if severe
61
Q

When would you not give immunosuppression

A

If due to malignancy

62
Q

What are complications of membranous

A

Chronic renal failure

Transplant / dialysis

63
Q

What causes FSGN

A 
Idiopathic
Malignancy - lymphoma 
Infection - HIV
Drug - heroin / lithium 
Scarring
IgA
Alport
Sickle cell
64
Q

How does FSGN present

A

Nephrotic
High BP
Haematuria

65
Q

How do you Dx

A

Bloods

Biopsy shows scarring of certain segments

66
Q

How do you Rx

A 
H20 and salt restriction
ACEI + BP control
Diuretic 
Steroids - don't usually work
Immunosuppression 2nd line
Transplant 
Nephrotic Rx
67
Q

What is prognosis

A

Common ESRD

Proteinuria worsens prognosis

68
Q

What is commonest cause of end stage renal failure

A

Diabetic nephropathy

69
Q

How does DM lead to nephropathy

A 
Hyperglycaemia = activates RAAS
Thickened capillary 
Reduced flow
Increased glomerular pressure
Podocyte damage
Endothelial dysfunction
Adhesions to Bowman
Fibrosis in late
70
Q

What is 1st sign of diabetic nephropathy

A

Slow albuminuria

71
Q

How do you screen

A

ACR - early morning urine annually

72
Q

What accelerates disease

A

Co-existing high BP

73
Q

What does biopsy show and uSS

A

Excess masangial matrix due to glucose compressing capillaries - KImmelsteil Wilson
Scarring
Fibrosis

USS = bilaterally enlarged kidney

74
Q

How do you treat

A 
DM control
HbA1c <53 reduces microvascular + progression
BP control
ACEI 
Na and protein restriction
Statin for CVS risk
75
Q

Who has very high CVD risk

A

DM on dialysis

76
Q

What is aport

A

X-linked XR

Disorder of glomerulus basement membrane

77
Q

How does Alport present

A 
Haematuira
Nephrotic 
Gradual renal failure
Bilateral sensorineural deaf
Eye signs - retinitis pigments / lentus
78
Q

How do you Dx

A

Renal biopsy

79
Q

What are complications

80
Q

When do you think Alport

A

Failing renal transplant

81
Q

What causes retroperitoneal fibrosis if kidney

A

Autoimmune
IgG 4
Can affect kidney / pancreas
ENT

82
Q

How does it present

A

Renal failure
Increased CRP
Low complmnt

83
Q

How do you treat

A

Steroids

Stent - can cause bladder irritation

84
Q

What causes PCKD

A

AD

PKD1 = most common

85
Q

What are the symptoms of PCKD

A 
Asymptomatic
Chronic loin pain as capsule stretched
Haematuria if haemarrhage / cyst rupture 
HTN / CVS disease
Fatigue 
Recurrent UTI
Renal stones

Progressive renal failure / ESRF

86
Q

What are the complications

A 
SAH 
Cysts in other organ - hepatomegaly most common
Diverticulosis
MVP / MVR
Aortic dissection
87
Q

How do you Dx and screen

A

USS + genetic test

USS to screen family

88
Q

When do you screen for aneurysm

89
Q

What do you do for renal colic

A

CT as cyst will obscure view on USS

90
Q

How do you treat

A 
Mainly supportive 
High water intake
ACEI for bP 
Thiazide / BB 2nd line
Tolvaptan 
Diaylsis 
Kidney transplant
91
Q

What does tolvapatan do

A

Vasopressor 2 antagonist
Blocks ADH
Makes you pee lots and thirsty
Slows progression

92
Q

What must you monitor

A

LFT strictly

93
Q

When do you use tolvaptan

A

Rapidly progressing

CKD 2/3 at time of RX

94
Q

What other monitoring in PCKD

A 
Genetic counselling
Avoid contact sport due to risk of rupture
Avoid NSAID and anti-coagulat
Regular USS
Regular bloods for kidney function
Regular BP
95
Q

SE of steroid

96
Q

What is vHL

A

AD
RCC
Phaeochromcytoam
Haemangioblastoma

Renal / Urology (16 decks)

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