1
Q
what is virtually pathognomic of GN in the urine
A
red cell cast
2
Q
nephritic syndrome
A
- Syndrome comprising signs of nephritis, presents with haematuria, hypertension and oedema (not as severe as that in nephrotic syndrome)
- indicative of a proliferative process affecting the endothelial cells
3
Q
nephrotic syndrome
A
- Defined as proteinuria >3g/d, hypoalbuminuria (<25g/L) and oedema with severe hyperlipidaemia (and cholesterol)
- This is indicative of a non-proliferative process affecting podocytes.
4
Q
how does proteinuria affect BP target
A
aim for 120/75 rather than 130/80
5
Q
what is the most common GN in the developed world
A
IgA nephropathy
6
Q
IgA nephropathy
A
- there is raised abnormal IgA, possibly due to infection, which is targeted by the immune system and forms immune complexes (with IgG) which are deposited in mesangial cells
- They accumulate and cause local immune activation (pro-inflammatory cytokines are released and macrophages arrive) and injury. RBC pass through into urine
7
Q
which systemic condition does IgA Nephropathy overlap with
A
HSP
8
Q
typical patient with IgA Nephropathy
A
- young man with episodic macroscopic haematuria almost immediately after resp/gi infection
- recovery is rapid between attacks
9
Q
how soon after infection does IgA Nephropathy tend to occur
A
1-2 days
10
Q
presentation of IgA Nephropathy
A
- asymptomatic/intermittent macroscopic/microscopic haematuria ± nephritic syndrome
11
Q
what is seen on investigation of IgA nephropathy
A
- LM: diffuse mesangial IgA deposition. Mesangial cell proliferation and expansion
- IF: immune deposits (IgA and C3) in mesangium
- The finding of complexes that contain IgA can differentiate it from other GN
- EM: electron dense deposits in the mesangium
- normal complement levels
12
Q
prognosis of IgA nephropathy
A
25% progress to ESRF n ten years
13
Q
management of IgA nephropathy
A
main focus is preventing further damage and avoiding end stage kidney disease
- BP control: ACEi and ARBs/fish oil
- Prevent immune complex formation: corticosteroids
- With nephritic presentation, immunosuppression may slow down decline of renal function
14
Q
how does post-streptococcal GN present
A
nephritic syndrome
15
Q
how common is post-streptococcal
A
very rare IRL
16
Q
how long after infection does post-streptococcal present
A
few weeks
17
Q
notifiable biochemical markers in post-streptococcal
A
low complement
18
Q
what is found in the urine in post-streptococcal
A
proteinuria (even tho it is associated with nephritic syndrome - remember!)
19
Q
RPGN
A
- purely a marker of severeity
- acute deterioration of kidney function, associated with crescent formation
20
Q
how severe is RPGN
A
can cause ESRF over a few days
21
Q
causes of RPGN
A
Vasculitis e.g. ANCA+:
- Pauci-immune (no anti-GBM, no immune complexes)
- cANCA (GPA) and pANCA (microscopic polyangiitis and eGPA)
Immunological e.g. ANCA-:
- Anti-GBM: Good pastures disease
- Immune complex disease: HSP/IgA, SLE, post-streptoccocal glomerulonephritis
22
Q
how do crescents form
A
- seen in RPGN and Goodpasture’s
- Glomerular disease leads to gaps or holes in the GBM resulting in epithelial cell proliferation with mononuclear infiltration in Bowman’s space. Ultimately, crescent is replaced by scar tissue
- Lose ability to filter, can quickly lead to AKI.
- There is active urinary sediment: RBC, RBC and granular casts
23
Q
clinical presentation of RPGN
A
- AKI ± systemic features
- There is often an insidious onset, with a long history of general malaise
- General: malaise, fatigue, fevers
- Skin: cutaneous nodules
- Neurological: mononeuritis multiplex
- MSK: myalgia, arthritis
- Eyes: iritis, uveitis
- Respiratory: haemoptysis, SOB
- Other: oral ulceration
24
Q
what must be done urgently with RPGN
A
renal biopsy
25
anti-GBM IF
linear shape
26
immune complex deposition on IF
granular
27
treatment of RPGN
should be prompt and consist of strong IS with supportive care including dialysis if needed
* Poor prognosis
* Anticoagulants to reduce fibrin build up in crescent
* Aggressive immunosuppression with high-dose IV steroids and cyclophosphamide ± plasma exchange
28
how does Goodpasture's present
* haematuria ± nephritic syndrome
* haemoptysis
29
goodpasture's pathophysiology
* type II hypersensitivity reaction in which anti-GBM antibodies bind to type IV collagen in lungs and kidneys
30
lung involvement in goodpasture's
* more common in smokers, and it is thought that smoking is a trigger for the disease
* haemoptysis
31
Goodpasture's - typical patient age and sex
young male
32
which gene is goodpasture's associated with
HLA-DR2
33
what is seen on LM of goodpasture's
crescent formation
34
what is seenon IF of goodpasture's
linear IgG deposiiton along basement membrane
35
management of goodpasture's
* Plasma exchange to remove the circulating antibody
* Steroids
* Cyclophosphamide
36
define nephrotic syndrome
Defined as **proteinuria \>3.5g/d, hypoalbuminaemia (\<25g/L, usually much lower) and oedema**
## Footnote
**severe hyperlipidemia**
37
why is severe hyperlipidemia present in nephrotic syndrome
by product of the liver tyring to make more albumin
38
what does severe hyperlipidemia caause in nephrotic syndrome
procoagulant state, classically renal vein thrombosis
39
how does renal vein thrombosis present
flank pain and haematuria, can be a sudden onset
40
features of nephrotic syndrome
* Periorbital oedema is present, often in the morning and drains throughout the day
* Classically seen in kids
* followed by full body oedema
* thrombotic complication may be the first sign
41
what is the most common type in children
minimal change
42
incidence of minimal change
two peaks - child hood and later on around 60
43
EM of minimal change
podocyte apoptosis, loss of gaps between podocytes
44
treatment of minimal change
* steroids, 94% get total remission
* prednisolone, start at 40-60 mg
* relapses can occur
45
wha must be given with long term steroid use
PPI and bone protection - bisphosphoante
46
how do adults respond to treatment compared o children in minimal change
may be slower
47
how is frequently relapsing minimal change disease treated
cyclophsophamide or cyclosporin (to reduce steroid dose)
48
prognosisof minimal change
good - 1% to ESRF
49
FSGS severity
severe, around 50% have impaired renal function
50
causes of FSGS
* May be primary (idiopathic) or secondary (HIV, heroin use (IVDU), sickle cell disease, obesity, reflux)
51
HIV FSGS
associated with a collapsing type which has a poorer prognosis
52
treatment of FSGS
* :inconsistent response to steroids (around 30%)
* cyclophosphamide or cyclosporine are considered if steroid resistant
53
compare the EM in primary and secondary FSGS
* Primary FSGS: diffuse podocyte process fusion
* Secondary FSGS: podocyte process fusion limited to sclerotic areas
54
causes of membranous nephropathy
* Most idiopathic, but can often secondary to autoantibody generation in response to e.g.
* malignancy
* hepatitis B
* malaria, drugs (gold, penicillamine, NSAIDs)
* autoimmune diseases (SLE – class V, RA)
55
which type of GN can be caused by malignancy
membranous nephropathy - lung, prostate, breast and colon
56
findings on LM of membranous
* immune complex deposition between podocytes and GBM
* spike and dome pattern
57
which stain is used on LM for membranous
special silver stain
58
treatment of membranous
* Underlying cause in secondary
* In idiopathic treatment involves general measures such as ACEi/ARBs and diuretics
* In severe cases, steroids, alkylating agents (e.g. cyclophosphamide), B cell monoclonal antibody (Rituximab)
59
prognosis of membranous
Rule of thirds
* 1/3: spontaneous remission
* 1/3: remain proteinuric
* 1/3: progress to ESRF
* Good prognostic features include female sex, young age at presentation and asymptomatic proteinuria of a modest degree at the time of presentation
60
which specific Ab have been found to be associated with membranous nephropathy
* Antibodies directed against **phospholipase A2 receptor** are found in up to 80% of patients with idiopathic membranous nephropathy
61
membrano-proliferative
mixed nephrotic/nephritic syndrome - more varied clinical presentation
62
causes of Membrano-Proliferative
* Idiopathic
* Autoimmune e.g. SLE, RA
* Cancer
* Hepatitis C
63
LM of membrano-proliferative
tram track GBM
64
management and prognosis of membrano-prolfierative
poor, steroids may be effective
65
low complement GN
endocarditis or post streptococcal
RB Y2 Renal
flashcards
Decks in class (26)
# Cards
-
Anatomy of Upper Renal Tract44
-
Anatomy of Lower Renal Tract56
-
Histology30
-
Fluid Prescribing36
-
Anatomy 341
-
Physiology111
-
Glomerulonephritis79
-
Inherited Kidney Disease60
-
Dysproteinurias17
-
BPH16
-
Renal Malignancy49
-
Prostate Cancer51
-
Bladder Tumours22
-
Penile and Testicular Carcinoma61
-
Urinary Incontinence34
-
Pharmacology40
-
Urinary Tract Infection99
-
Urological Emergencies67
-
Acute Scrotum68
-
Renal Transplant74
-
Renal Investigations6
-
GN 2.065
-
AKI70
-
CKD48
-
Urinary Tract Obstruction12
-
Dialysis30
