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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
How do you Dx IGA
Urine culture Bloods USS Renal biopsy shows IgA deposition
26
How do you differentiate from post strep
Proteinuria in strep Low complement in strep Longer interval between infection with strep
27
How do you treat IgA
ACEI for BP and reduce protein = mainstay BP meds aim 125/75 Kidney transplant for ESRD Immunosuppression if rapidly progressive
28
What is the prognosis and what does it depend on
``` 1/3 ESRD over 20 yearrs BP Proteinuria Hypertenion SMoking Male Kidney function ```
29
What is linked to IgA
HSP in children
30
What causes rapidly progressive GN
``` IgA Post infection Macro granulomatosis Microscopic polyarteritis Anti-GBM HSP SLE ```
31
What are features of rapidly progressive GN
``` Nephritic Often present in AKI Haematuria High BP High creatinnien Proteinuria ``` Features of cause - Fatgiue - Weight loss - Rash - Fever - Lung or nerve involvement
32
How do you Dx rapidly progressive GN
MSSU Bloods USS Biopsy shows crescentic GN
33
What is serum test for macroscopic
C-ANCA | Proteinase 3 - PRE
34
What is serum test for microscopic polyarteritis
P-ANCA | Myeloperioxidase - MPO
35
How d you treat rapidly progressive
IV steroid Immunosuppression - cyclosporine / azathioprine / cyclophosphamide Plasma exchange if Anti-GBM / ANCA Monoclonal Ab
36
What is prognosis
Good if Rx early
37
What are risks of cyclophosphamide
Bladder TCC | Cystitis - haemorrhage
38
Complication of steroid
``` Growth Infection Obesity Papilloedema / benign intracranial HTN Adrenal crisis ```
39
What is Anti-GBM disease
Ab against type IV collagen in glomerulus and basement membrane Type 2 hypersensitivity
40
What are the symptoms of Anti-GBM
``` Hx chest sx Oliguria Haematuria Rapid AKI Pulmonary / kidney haemorrhage = SOB and haemoptysis ```
41
What puts you at risk of pulmonary haemorrhage
Male Pulmonary oedema LRTI Smoking
42
How do you Dx
CXR | Renal biopsy
43
How do you treat
Plasma exchange to get rid of Ab Corticosteroid Immunosuppression - cyclophosphamide May need transplant or dialysis if can't get control
44
What is HSP
Autoimmune inflammation of small blood vessels IgA vasculitis Common in children following infection IgA deposition in skin, joints, gut and kidney
45
What are the symptoms HSP
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
How do you Dx and what must you excluded
``` 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
How do you treat HSP and what do you do after 1 year
``` 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
What causes minimal change disease
``` NO INFLAMMATION Children / young adults usually idiopathic Idiopathic Drugs - NSAID / lithium Hodgkin EBV ```
49
What is most common cause of nephrotic
Minimal change
50
What are the symptoms
Sudden oedema Proteinuria Frothy urine Normal BP and renal function
51
How do you Dx nephrotic
Urinanalysis Bloods - increased creatinine and low albumin Clotting
52
When is biopsy indicated
No response Other cause suspected e.g. autoimmune Abnormal renal function
53
How do you treat
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
What do you consider if minimal change progresses to CKD or non-responsive
FSGS - most common cause of nephrotic in adults | Congenital nephron / podocyte deficiency if child
55
What is pathology of membranous GN (most common GN overall)
No inflammation IgG between lamina and podocyte in GBM Activates complement - Ddx of post-strep not responding Punches hole
56
What causes membranous
``` Idiopathic Malignancy - lymphoma or solid organ RA Infection - hep B/C/strep / malaria/schistosomiasis / HIV SLE Amyloid Drugs e.g. NSAID ```
57
How does membranous present
Nephrotic
58
How do you Dx
``` Urine MSSU Bloods USS Biopsy Focussed history / CXR / exam to look for 2 cause ```
59
What Ab +ve in membranous
Anti-phospholipase A2 in primary
60
How do you treat
``` General nephrotic ACEI Diuretic Immunosuppression if idiopathic Steroid Rituximab if severe ```
61
When would you not give immunosuppression
If due to malignancy
62
What are complications of membranous
Chronic renal failure | Transplant / dialysis
63
What causes FSGN
``` Idiopathic Malignancy - lymphoma Infection - HIV Drug - heroin / lithium Scarring IgA Alport Sickle cell ```
64
How does FSGN present
Nephrotic High BP Haematuria
65
How do you Dx
Bloods | Biopsy shows scarring of certain segments
66
How do you Rx
``` H20 and salt restriction ACEI + BP control Diuretic Steroids - don't usually work Immunosuppression 2nd line Transplant Nephrotic Rx ```
67
What is prognosis
Common ESRD | Proteinuria worsens prognosis
68
What is commonest cause of end stage renal failure
Diabetic nephropathy
69
How does DM lead to nephropathy
``` Hyperglycaemia = activates RAAS Thickened capillary Reduced flow Increased glomerular pressure Podocyte damage Endothelial dysfunction Adhesions to Bowman Fibrosis in late ```
70
What is 1st sign of diabetic nephropathy
Slow albuminuria
71
How do you screen
ACR - early morning urine annually
72
What accelerates disease
Co-existing high BP
73
What does biopsy show and uSS
Excess masangial matrix due to glucose compressing capillaries - KImmelsteil Wilson Scarring Fibrosis USS = bilaterally enlarged kidney
74
How do you treat
``` DM control HbA1c <53 reduces microvascular + progression BP control ACEI Na and protein restriction Statin for CVS risk ```
75
Who has very high CVD risk
DM on dialysis
76
What is aport
X-linked XR | Disorder of glomerulus basement membrane
77
How does Alport present
``` Haematuira Nephrotic Gradual renal failure Bilateral sensorineural deaf Eye signs - retinitis pigments / lentus ```
78
How do you Dx
Renal biopsy
79
What are complications
CKD
80
When do you think Alport
Failing renal transplant
81
What causes retroperitoneal fibrosis if kidney
Autoimmune IgG 4 Can affect kidney / pancreas ENT
82
How does it present
Renal failure Increased CRP Low complmnt
83
How do you treat
Steroids | Stent - can cause bladder irritation
84
What causes PCKD
AD | PKD1 = most common
85
What are the symptoms of PCKD
``` 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
What are the complications
``` SAH Cysts in other organ - hepatomegaly most common Diverticulosis MVP / MVR Aortic dissection ```
87
How do you Dx and screen
USS + genetic test | USS to screen family
88
When do you screen for aneurysm
If FH
89
What do you do for renal colic
CT as cyst will obscure view on USS
90
How do you treat
``` Mainly supportive High water intake ACEI for bP Thiazide / BB 2nd line Tolvaptan Diaylsis Kidney transplant ```
91
What does tolvapatan do
Vasopressor 2 antagonist Blocks ADH Makes you pee lots and thirsty Slows progression
92
What must you monitor
LFT strictly
93
When do you use tolvaptan
Rapidly progressing | CKD 2/3 at time of RX
94
What other monitoring in PCKD
``` 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
SE of steroid
Beware
96
What is vHL
AD RCC Phaeochromcytoam Haemangioblastoma

Renal / Urology (16 decks)

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