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Glomerular disease Flashcards

1
Q

What is glomerulonephritis

A

Inflammation of or around the glomerulus and nephron

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2
Q

What can all forms of glomerulonephritis lead to

A

Nephritic / nephrotic syndrome
Different forms have tendency to cause one or the other or mixed

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3
Q

What are the features of nephritic syndrome

A

Haematuria
Red cell casts in urine (microscopic bleeding)
Hypertension
Mild to moderate proteinuria
Oliguria

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4
Q

What are the features of nephrotic syndrome

A

Massive proteinuria >3.5g/day
Hypoalbuminaemia
Oedema
Hypercoagulability
Hyperlipidaemia
Low total thyroxine levels

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5
Q

How does nephrotic syndrome predispose patients to thrombosis

A

Due to loss of antithrombin-III proteins and rise in fibrinogen levels

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6
Q

Why may low total thyroxine levels be seen in nephrotic syndrome

A

Due to loss of thyroxine binding globulin proteins

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7
Q

What are the symptoms of nephritic syndrome

A

Haematuria
Oedema (to a lesser extent compared to nephrotic syndrome)
Oliguria
Uraemic symptoms - fatigue, reduced appetite, pruritus

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8
Q

What are the symptoms of nephrotic syndrome

A

Facial oedema
Peripheral oedema
Frothy urine (due to heavy proteinuria)
Fatigue
Recurrent infections
Hypercoagulability -> MI / DVT / renal vein thrombosis

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9
Q

Why are patients with nephrotic syndrome predisposed to recurrent infections

A

Due to loss of immunoglobulins

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10
Q

Why is oedema more severe in nephrotic syndrome

A

Due to heavy loss of protein in urine causing hypoalbuminaemia.
Hypoalbuminaemia causes oedema because of reduced oncotic pressure so water goes out of blood vessels and into tissues

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11
Q

Clinical signs of nephrotic syndrome

A

Periorbital oedema / lower limbs oedema / Ascites
SOB due to pleural effusion
Xanthelasma due to hyperlipidaemia

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12
Q

Differences between nephritic and nephrotic syndrome

A

Nephritic syndrome causes haematuria, oliguria, hypertension whereas nephrotic syndrome does not

Nephrotic syndrome causes HEAVY proteinuria > 3.5 whereas proteinuria in nephritic syndrome is mild to moderate

Nephrotic syndrome causes more severe oedema

Nephrotic syndrome causes hyperlipidaemia and hyper coagulability whereas nephritic syndrome does not

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13
Q

What are the different types of glomerulonephritis

A

Minimal change disease
Focal segmental glomerulosclerosis
Membranous GN
IgA nephropathy
Post-infectious GN
Membranoproliferative
Rapidly progressive GN
Goodpasture’s syndrome
Alport’s syndrome

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14
Q

Which form of GN is the most common

A

IgA nephropathy

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15
Q

What are the types of GN regarding to its pathophysiology

A

Proliferative
Non-proliferative

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16
Q

What is proliferative GN

A

Presence of proliferation of endothelial and mesangial cells

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17
Q

Proliferative GN typically presents with

A

Nephritic syndrome

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18
Q

What is non-proliferative GN

A

Non-proliferative process.
Due to damage to podocytes

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19
Q

Non-proliferative GN typically presents with

A

Nephrotic syndrome

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20
Q

Which types of GN are non-proliferative

A

Minimal change
Focal segmental
Membranous GN

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21
Q

Which types of GN are proliferative

A

IgA nephropathy
Rapidly progressive GN
Anti-glomerular basement membrane antibody disease
Vasculitic disorders - GPA, MPA

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22
Q

Which types of GN can cause mixed- nephritic and nephrotic syndrome

A

Membranoproliferative GN
Post-Strep (infectious) GN

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23
Q

Minimal change disease is the most common

A

Most common cause of nephrotic syndrome in children

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24
Q

Causes of minimal change disease

A

Mostly idiopathic

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25
What are the typical investigation results for minimal change disease
Highly selective proteinuria NORMAL renal biopsy under light microscopy NORMAL BP Electron microscopy shows fusion of foot processes
26
What does highly selective proteinuria mean in minimal change disease
Only intermediate sized proteins such as albumin can leak through
27
What is special about minimal change disease
Normal light microscopy results Abnormality only seen on electron microscopy
28
Management of minimal change disease
Oral corticosteroids (80% are responsive) Cyclophosphamide for steroid-resistant cases Salt restriction and fluid management
29
Describe the prognosis of minimal change disease
Relapse is common 1/3 have only 1 episode 1/3 have infrequent relapses 1/3 have frequent relapses which stop before adulthood
30
Focal segmental glomerulosclerosis is the most common
Cause of nephrotic syndrome in adults - young adults
31
Causes of focal segmental glomerulosclerosis
Idiopathic Secondary causes - HIV - SLE - reflux nephropathy - sickle cell disease - heroin
32
Typical investigation results for focal segmental glomerulosclerosis
On light microscopy - mesangial collapse and sclerosis On electron microscopy - fusion of foot processes
33
Management of focal segmental glomerulosclerosis
Salt restriction and fluid management Prolonged Steroids Cyclophosphamide
34
Describe the prognosis of focal segmental glomerulosclerosis
60% go into remission with prolonged steroids 40% process into end stage renal failure after 10 years
35
Membranous glomerulonephritis mostly causes
nephrotic syndrome in adults (2nd most common cause)
36
Causes of membranous glomerulonephritis
Mostly idiopathic Infections - Hep B, malaria Malignancy - Prostate, lung, lymphoma, leukaemia Drugs SLE Thyroiditis
37
What infections are associated with membranous glomerulonephritis
Hep B Malaria
38
What malignancies are associated with membranous glomerulonephritis
Prostate Lungs Lymphoma Leukaemia
39
What drugs are associated with membranous glomerulonephritis
Gold Penicillamine NSAID
40
Typical investigation results for membranous glomerulonephritis
Light microscopy - thickened glomerular basement membrane Immunofluorescence - diffuse uptake of IgG EM - thickened glomerular basement membrane with sub epithelial deposits of immune complexes
41
What is the classic sign of membranous glomerulonephritis in EM
Spike and dome appearance due to subepithelial deposits
42
Management of membranous glomerulonephritis
all patients should receive ACEi / ARB Steroids + cyclophosphamide
43
Describe the prognosis of membranous glomerulonephritis
Rule of thirds - 1/3 spontaneous remission - 1/3 chronic MGN - 1/3 develop end stage renal failure
44
Membranoproliferative GN causes nephritic / nephrotic/ mixed syndrome
Mixed
45
Causes of membranoproliferative GN
Mostly idiopathic Hep C SLE
46
Typical investigation results of membranoproliferative GN
Thickened basement membrane Thickened mesangium Subendothelial deposition of IgG
47
What is the classic appearance of membranoproliferative GN
Tram tracking appearance due to deposits and thickened mesangium (mesangial cells disperse themselves between the basement membrane and endothelium as well)
48
Management of membranoproliferative GN
Steroids for children Dipyridamole and aspirin for adults Kidney transplantation for ESRF
49
Cause of IgA nephropathy
Due to mesangial deposition of IgA immune complexes
50
IgA nephropathy is associated with
URTI Henoch Schonlein purpura Dermatitis herpetiformis Coeliac Cirrhosis
51
What is the classic presentation of IgA nephropathy
Macroscopic haematuria in young people after a URTI
52
IgA nephropathy causes nephritic / nephrotic / mixed syndrome
Nephritic syndrome
53
Typical investigation results for IgA nephropathy
LM - mesangial cell proliferation Immunofluorescence - IgA deposits in mesangium EM - deposits in mesangium
54
How long does GN occur after URTI in IgA nephropathy
1-2 days
55
Management of IgA nephropathy
If only haematuria and no/mild proteinuria = no treatment, regular checkups If persistent proteinuria = ACEi/ARB If GFR is falling / failure to respond to ACEi = corticosteroids
56
Prognosis of IgA nephropathy
25% develop into ESRF
57
What are the markers of poor prognosis of IgA nephropathy
Male Proteinuria Hypertension Smoking Hyperlipidaemia
58
Which pathogen causes post-streptococcal glomerulonephritis
Streptococcus pyogene (group A beta haemolytic)
59
Post-streptococcal glomerulonephritis most commonly affects which age group
Young children
60
Post-streptococcal GN causes which type of syndrome
Mixed
61
Investigations for post streptococcal GN
Bloods Renal biopsy
62
What do blood tests show in post-streptococcal GN
Raised anti-streptolysin O titre - confirms recent strep infection low C3
63
What are the Renal biopsy results suggesting post-streptococcal GN
LM - Endothelial proliferation EM- Lumpy immune complex deposits Immunofluorescence - granular / starry sky appearance
64
Difference between post-streptococcal GN and IgA nephropathy
Post strep GN occurs 7-14 days after infection whereas IgA nephropathy occurs 1-2 days after infection Post-strep GN causes low complement level Post strep GN main symptom is proteinuria whereas IgA nephropathy is haematuria
65
Causes of rapidly progressive GN
GPA MPA Goodpasture's SLE
66
Rapidly progressive GN causes which type of syndrome
nephritic syndrome
67
Investigation results for rapidly progressive GN
Crescent formation in glomeruli (proliferating epithelial cells)
68
Management of rapidly progressive GN caused by goodpasture's
high dose immunosuppression- IV methylprednisolone / cyclophosphamide Plasmapheresis
69
Management of rapidly progressive GN caused by vasculitis
High dose prednisolone + cyclophosphamide
70
What is the defect in Alport's syndrome
Defect in gene that codes for type 4 collagen causing abnormal glomerular-basement membrane
71
Alport's syndrome is more severe in
Males
72
Mode of inheritance for Alport's syndrome
Mostly X linked dominant
73
Symptoms of Alpert's syndrome
Microscopic haematuria Bilateral sensorineural deafness Lenticonus - protrusion of lens Retinitis pigmentosa
74
What is lenticonus
Protrusion of lens into the anterior chamber
75
What is retinitis pigmentosa
Progressive loss of photoreceptors + retinal deposits
76
Investigations of Alport's syndrome
Genetic testing Renal biopsy
77
What does renal biopsy show in Alport's syndrome
Longitudinal splitting of lamina dense of glomerular base membrane Basket weave appearance

Y2 Renal (12 decks)

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