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Glomerular Disease - Clinical Flashcards

1
Q

What is glomerulonephritis?

A

Inflammatory disorder of the kidney

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2
Q

What percentage of end-stage kidney disease is glomerulonephritis responsible for?

A

Up to 30%

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3
Q

What is the presentation of glomerulonephritis?

A 
Can present in many ways 
Mostly vague symptoms/signs 
- proteinuria
- renal failure
- hypertension
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4
Q

What is the function of mesangial cells?

A

Provide extracellular support for the capillaries

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5
Q

What is the permeability of the glomerular basement membrane?

A

Selectively permeable - in normal, healthy people, protein should not be present in the urine

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6
Q

What is present in the nuclei/cells in proliferative glomerulonephritis?

A

Overlap and congestion of the nuclei/cells

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7
Q

What are the classes of glomerulonephritis?

A

Proliferative

Non-proliferative

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8
Q

What are the features of non-proliferative glomerulonephritis?

A

Glomeruli look normal or have areas of scarring
Normal numbers of cells
Tubules and interstitium may be damaged

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9
Q

What are the features of proliferative glomerulonephritis?

A

Characterised by excessive numbers of cells in the glomeruli, including infiltrating leukocytes

Presents with nephrotic syndrome
Blood on dipstick, no or minimal proteinuria

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10
Q

What are the types of proliferative glomerular disease?

A

Diffuse proliferative
Focal proliferative
Focal necrotising (crescentic) nephritis
Membrano-proliferative nephritis

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11
Q

What are the features of post-infective glomerulonephritis?

A

Diffuse, congested nuclei
Immune deposits due to activation of the complement system
Follows 10-21 days after infection, typically of throat or skin

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12
Q

What type of bacteria is post-infective glomerulonephritis most commonly associated with?

A

Lancefield group A streptococci

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13
Q

Acute nephritis is typical of what?

A

Post-infective glomerulonephritis

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14
Q

What are the features of acute nephritis?

A

Fluid retention with oedema - periorbital oedema common
Normal serum albumin
Little proteinuria
Renal impairment

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15
Q

What is the treatment of post-infective glomerulonephritis?

A

Antibiotics for any active infection
Loop diuretics e.g. furosemide for oedema
Vasodilator drugs e.g. amlodipine for hypertension
Consider immunosuppression in severe/unresolving disease

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16
Q

What is the commonest cause of glomerulonephritis?

A

IgA nephropathy

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17
Q

What are the features of IgA nephropathy?

A

Congestion
Mesangial and endocapillary inflammation
Most inflammatory deposits are in the mesangium
No characteristic serology
Diagnosed by renal biopsy

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18
Q

In what patients does IgA nephropathy typically occur in?

A

Young - presents in late 30s-40s

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19
Q

What indicates that a patient with IgA nephropathy is likely to deteriorate?

A

Heavy proteinuria
High creatinine
Hypotension

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20
Q

What is the presenting feature of IgA nephropathy?

A

Macroscopic haematuria

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21
Q

What is IgA nephropathy provoked by?

A

Intercurrent infection

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22
Q

What is the typical onset of IgA nephropathy in adults?

A

Insidious onset

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23
Q

What is the prognosis of IgA nephropathy in adults?

A

Worse in adults - 25% develop renal failure

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24
Q

What is the treatment of IgA nephropathy?

A

Non-specific

ACEIs and other hypotensives

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25
What are the features of focal necrotising glomerulonephritis?
Segmental necrosis | Ruptured capillary
26
In what 3 main settings does focal necrotising glomerulonephritis occur?
In presence of anti-glomerular basement membrane antibodies (Goodpasture's syndrome) Associated with systemic vasculitis e.g. granulomatosis with polyangiitis, microscopic polyangiitis As a complication of other types of glomerulonephritis, including post-streptococcal glomerulonephritis and mesangial IgA disease
27
What is Goodpasture's disease?
Rare disease caused by autoimmunity to the glomerular basement membrane
28
How does Goodpasture's disease typically present?
As nephritis, with or without lung haemorrhage
29
How is Goodpasture's disease diagnosed?
By demonstrating anti-GBM antibodies in serum and kidney
30
How is Goodpasture's disease treated?
Plasma exchange and immunosuppression | Kidney can only be saved if detected and treated early
31
What is the typical presentation of crescentic glomerulonephritis?
Rapidly progressive glomerulonephritis May occur in isolation, or as a complication of other disease e.g. chronic nephritis Medical emergency
32
What is the management of crescentic glomerulonephritis?
Immunosuppression - prednisolone, cytotoxic drugs, plasma exchange
33
What is the prognosis of crescentic glomerulonephritis?
Good, if treatment is started early enough
34
CASE 1 ``` 7 year old boy presents to GP Generally unwell, dark urine due to haematuria Puffy face, no rash, no oedema BP 125/80 Sore throat two weeks previously Hb 130 g/l WCC 11.3 x 109 Urea 11.2 mmol/l Creatinine 160umol/l Urine microscopy – RBC +++, casts + Complement – low C3 Spontaneous diuresis after 6 days Serum creatinine returned to normal ``` What is the most likely diagnosis?
Diffuse proliferative glomerulonephritis - post-streptococcal nephritis
35
CASE 2 14 year old boy presents to GP with bright red urine No other urinary symptoms Concurrent upper respiratory tract infection Physical examination normal Urine dipstick shows high blood and trace of protein What investigations would you do?
``` FBC Creatinine, urea and electrolytes Urine microscopy Serology Renal ultrasound Renal biopsy ```
36
CASE 2 14 year old boy presents to GP with bright red urine No other urinary symptoms Concurrent upper respiratory tract infection Physical examination normal Urine dipstick shows high blood and trace of protein Urine microscopy shows dysmorphic erythrocytes and red cell casts What is the most likely diagnosis?
Focal proliferative nephritis caused by mesangial IgA disease
37
CASE 3 57 year old woman presents to GP Three month history of increasing tiredness, anorexia and weight loss Developed a cough with haemoptysis over last two weeks Physical examination reveals rash on both ankles and lower legs and bilateral basal crepitations Urine dipstick shows protein and blood ANF and DNA binding negative ANCA positive What is the most likely diagnosis?
Focal necrotising glomerulonephritis secondary to systemic vasculitis
38
What are the types of non-proliferative glomerulonephritis?
Minimal change disease Focal glomerulonephritis Membranous nephropathy Causes nephrotic syndrome
39
What is the presentation of nephrotic syndrome?
Clinical triad of pitting oedema, proteinuria (> 3.5g) and hypoalbuminaemia (< 30 g/l, normal > 45)
40
What is nephrotic syndrome?
A clinical syndrome in which severe oedema is caused by hypoalbuminaemia due to loss of protein in the urine
41
What are the additional features of nephrotic syndrome?
Hyperlipidaemia Hypercoagulable state - increased DVT and venous thromboembolism risk Increased risk of infection, especially pneumococcus
42
What are the differential diagnoses of nephrotic syndrome?
Congestive heart failure - raised JVP, normal albumin, minimal proteinuria Hepatic disease - abnormal LFTs, no proteinuria Presence of proteinuria in nephrotic syndrome differentiates from these
43
What are the principles of management of nephrotic syndrome?
Specific management Treatment of oedema Prophylaxis against complications - identify those at risk of DVT/VTE and give anticoagulation
44
What is the specific management of nephrotic syndrome?
Make specific diagnosis e.g. by renal biopsy Specific treatment when indicated Consider steroid treatment with prednisolone if severe
45
What is the treatment of oedema in nephrotic syndrome?
Fluid and sodium restriction Normal/high protein diet Loop diuretics e.g. furosemide IV infusion of albumin furosemide for resistant oedema
46
What prophylaxis can be given against potential complications of nephrotic syndrome?
Penicillin V for prophylaxis against infection Heparin Treat hyperlipidaemia in those with persistent nephrotic syndrome
47
What are the features of minimal change nephrotic syndrome?
Most common form in children Sudden onset of oedema Complete loss of proteinuria with steroids 2/3rds of patients relapse despite most going into remission
48
What is the treatment of minimal change nephrotic syndrome?
Prednisolone 60mg/m^2 per day, with rapid tapering of dose after a maximum of 8 weeks Initial relapse treated in the same way Subsequent relapses treated with 8 week course of cyclophosphamide or continuous cyclosporine Treat any infection aggressively with antibiotics
49
What are the complications of minimal change nephrotic syndrome?
Bacterial infection, especially with pneumococcus | Thrombosis
50
What is the prognosis of minimal change nephrotic syndrome?
Resolves eventually, long term prognosis good | Patients do not develop renal failure
51
What are the features of focal glomerulosclerosis (steroid resistant nephrotic syndrome)?
Severe nephrotic syndrome, especially in men in fourth decade Very disabling symptoms Incomplete (at best) response to steroids Progresses to renal failure over 2-3 years Can recur in renal transplants Immunohistology negative Sclerotic lesions
52
Why is recover from focal glomerulosclerosis not possible?
Podocytes are killed by the disease process
53
What is the aim of treatment of focal glomerulosclerosis?
To delay the time until end-stage kidney disease
54
What is the treatment of focal glomerulosclerosis?
Initial trial of steroids - if complete response then prognosis is the same as for minimal change, if no response then poor prognosis Continue steroids if clinically useful response Try cyclophosphamide or cyclosporine if steroids fail Steroid-sparing agents can be used, variable success Non-specific drugs to reduce proteinuria e.g. ACEIs and NSAIDs Non-specific treatment for nephrotic oedema and prophylaxis against nephrotic complications
55
What is the commonest cause of nephrotic syndrome in adults?
Membranous nephropathy
56
What is the cause of membranous nephropathy?
About half of cases are idiopathic The other half are associated with another disease e.g. malignancy, systemic lupus, rheumatoid arthritis Can also be caused by hypersensitivity to drugs
57
Where are deposits located in membranous nephropathy?
Between the spikes
58
What is the management of membranous nephropathy?
Control nephrotic symptoms Immunosuppression for those with deteriorating renal function Prednisolone and chorambucil, alternating monthly for six months Cyclosporine probably ineffective General measures in treatment should be done for 6 months before starting immunosuppression
59
What is the prognosis of membranous nephropathy?
Resolves spontaneously in 25% over 5-10 years Prognosis is good in treated patients whose proteinuria resolves About 25% will be on dialysis at 10 years Can recur in renal transplants
60
CASE 4 ``` 22 year old woman Severe oedema which had developed suddenly over a week Breathless Left sided chest pain Urine contained high level of protein Nephrotic syndrome Creatinine upper level of normal Serum albumin low - 15 g/l ``` What is the likely diagnosis?
Minimal change nephrotic syndrome
61
CASE 5 ``` 66 year old man Steadily increasing oedema Recent change in bowel habit High level of proteinuria Serum creatinine high at 135mmol/l ``` What is the likely diagnosis?
Membranous nephropathy Need to determine if it is associated with malignancy

Urinary System (22 decks)

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