Glomerular Disease (Clinical)

Question 1

What is glomerulonephritis? How is it classified?

Answer 1

• Group of immune mediated disorders that affect the glomeruli
• Classified based on kidney biopsy findings

Question 2

What are some features of glomerulonephritis?

Answer 2

• Haematuria (visible / non-visible)
• Proteinuria (low grade or nephrotic)
• Hypertension
• Renal impairment

Question 3

What are some features of nephritic syndromes?

Answer 3

• Haematuria
• Mild proteinuria
• Hypertension
• Renal impairment (low urine volume)

Question 4

What are some features of nephrotic syndromes?

Answer 4

• Severe proteinuria (>3.5g/day or 350mg/mmol creatinine, urine may look frothy)
• Hypoalbuminaemia
• Oedema
• Hyperlipidaemia

Question 5

Why is there hyperlipidaemia associated with nephrotic syndrome?

Answer 5

liver increases production in response to hypoalbuminaemia, excess production of lipids is a side effect

Question 6

What are the two major types of glomerulonephritis? Difference between the two?

Answer 6

• Proliferative: excessive number of cells in the glomeruli, include invading leukocytes
• Non-proliferative: glomeruli look normal or have areas of scarring, normal cell number

Question 7

List some descriptive terminology used when describing glomerulonephritis

Answer 7

Diffuse: >50% of glomeruli affected

Focal: <50% of glomeruli affected

Global: all the glomerulus affected

Segmental: part of the glomeruli affected

Question 8

What is the most common cause of glomerulonephritis? Mechanism?

Answer 8

IgA Nephropathy

• Characterized by IgA deposition in the mesangium & mesangial proliferation.

Question 9

Who tends to be affected by IgA nephropathy?

Answer 9

Most common in 2nd and 3rd decade of life with males more commonly affected

Question 10

How does IgA nephropathy mediated glomerulonephritis tend to present?

Answer 10

• Microscopic haematuria.
• Microscopic haematuria + proteinuria
• Nephrotic syndrome
• IgA crescentic glomerulonephritis

Question 11

What is post-infectious glomerulonephritis? What type of bacteria tend to cause it?

Answer 11

Glomerulonephritis occurring typically 10-21 days past a throat or skin infection

• Most commonly with Lancefield group A Streptococci.

Question 12

How does post-infectious glomerulonephritis tend to present?

Answer 12

• Nephritic syndrome (high RBC in urine)

- Oedema / hypertension

Question 13

How does crescentic glomerulonephritis tend to present?

Answer 13

++ Nephritic syndrome

Slight nephrotic syndrome

Question 14

What is anti-GBM disease?

Answer 14

Anti-Glomerular Basement Membrane disease is an autoimmune disease where autoantibodies attack the capillaries in the kidneys &/or lungs

Question 15

How does anti-GBM disease present? How is it diagnosed?

Answer 15

Presents as nephritic syndrome (may also have lung haemorrhage - Goodpasture’s syndrome)

• Diagnosed by detected anti-GBM antibodies in the serum & kidney

Question 16

How is anti-GBM disease treated?

Answer 16

• aggressive immunesuppression
• steroid, plasma exchange
• cyclophosphamide (immunosuppressant)

Question 17

What are some examples of non-proliferative glomerulonephritis?

Answer 17

Minimal Change Disease

Focal and segmental glomerulonephritis

Membranous Nephropathy

Question 18

How does non-proliferative glomerulonephritis tend to present as a whole?

Answer 18

• Nephrotic syndromes

Question 19

What are some general measures taken to treat nephrotic syndromes?

Answer 19

• Treat oedema: salt and fluid restriction and loop diuretics.
• Hypertension: use Renin-Angiotensin-Aldosterone-blockade.
• Reduce risk of thrombosis: Heparin or Warfarin.
• Reduce risk of infection e.g. pneumococcal vaccine.
• Treat dyslipidemia e.g. statins

Question 20

What is the most common glomerulonephritis in children?

Answer 20

Minimal Change Disease Non-Proliferative Glomerulonephritis

Question 21

How does Minimal Change Disease glomerulonephritis tend to present?

Answer 21

++ Nephrotic Syndrome

classic triad: proteinuria, hypoalbuminaemia, oedema, +/- hyperlipidaemia

Question 22

How is Minimal Change Disease glomerulonephritis treated?

Answer 22

Steroids (tends to cure proteinuria):

Prednisolone – 1mg/kg for up to 16 weeks.
Once remission achieved , slow taper over 6 months.
Initial relapse treated with further steroid course.

Question 23

What is the prognosis like for minimal change disease glomerulonephritis?

Answer 23

• Tends to be favourable, with steroid treatment the risk of end stage disease is low

Question 24

What are Focal and segmental glomerulonephritis (FSGS)? Prognosis?

Answer 24

• Not a single disease, rather a syndrome with multiple causes.
• Pathology is focal and segmental sclerosis (scarring) in the glomerulus.
• Prognosis is poor as generally steroid resistant with high chance of progression to end stage kidney disease

Question 25

How does Focal and segmental glomerulonephritis (FSGS) tend to present? Treatment options?

Answer 25

Presents with nephrotic syndrome

• Treat with steroids and hope for even partial remission

Question 26

What is the most common cause of nephrotic syndrome in adults?

Answer 26

Membranous Nephropathy glomerulonephritis

• Most cases are idiopathic

Question 27

Describe the pathophysiology of membranous nephropathy glomerulonephritis?

Answer 27

Membranous nephropathy is deposition of immune complexes on the glomerular basement membrane (GBM) with GBM thickening

Question 28

What are some diagnostic markers for membranous nephropathy?

Answer 28

• Anti-phospholipase A2 receptor (PLA2R) antibody positive in 70% of idiopathic cases
• Thrombospondin type 1 domain containing 7A (THSD7A) in ~2%.

Question 29

Treatment for membranous nephropathy glomerulonephritis? Prognosis?

Answer 29

• Immunosuppression and steroids
• A third resolve spontaneously, good prognosis for those whose proteinuria resolves w treatment
• 25% on dialysis at 10 years, can recur in transplants

Question 30

What is the most important investigation when it comes to differentiating different types of glomerulonephritis?

Answer 30

• Renal biopsy

- Urine & blood tests also useful for detecting nephrotic vs. nephritic but histology confirms diagnosis often