Nephritic Glomerulonephritis

Question 1

What is the most common primary glomerulonephritis?

Answer 1

IgA nephropathy

Question 2

How does IgA nephropathy present?

Answer 2

Young patient (male)

• Asymptomatic non-visible haematuria
• OR Episodic visible haematuria which may be synpharyngitic aka within 12-72h of infection

Question 3

What conditions is IgA nephropathy associated with?

Answer 3

• Alcoholic cirrhosis
• Coeliac disease/dermatitis herpetiformis
• Henoch-Schonlein purpura

Question 4

What are symptoms of IgA nephropathy?

Answer 4

Asymptomatic or increased BP ± increased creatinine, proteinuria (usually < 1g)

Question 5

What % of patients progress to renal failure?

Answer 5

20-50% (minority)

Question 6

How do you diagnose IgA nephropathy?

Answer 6

Renal biopsy

Question 7

What would you see on renal biopsy in IgA nephropathy?

Answer 7

• IgA deposition in mesangium
• Mesangial hypercellularity
• Positive immunofluorescence for IgA & C3

Question 8

How do you treat IgA nephropathy and Henoch-Schonlein purpura?

Answer 8

• ACE-i/ARB (reduce proteinuria and protect renal function)
• Corticosteroids (good for gut involvement in HSP) + fish oil if proteinuria > 1g despite 3-6 months of ACE-i/ARB and GFR > 50

Question 9

What happens in Henoch-Schonlein purpura?

Answer 9

• Small vessel vasculitis (systemic variant of IgA nephropathy)
• IgA deposition in kidney, skin, joints, gut

Question 10

How do you diagnose Henoch-Schonlein purpura?

Answer 10

• Usually clinical diagnosis
• Confirm with positive immunofluorescence for IgA & C3 in skin
• Renal biopsy identical to IgA nephropathy

Question 11

What is symptoms of Henoch-Schonlein purpura?

Answer 11

• Purpuric rash on extensor surface, typically legs
• Flitting polyarthritis
• Abdominal pain (GI bleeding)
• Nephritis

Question 12

What is nephritis?

Answer 12

Haematuria due to inflammatory damage

Question 13

What is nephrosis?

Answer 13

Proteinuria due to podocyte pathology

Question 14

What investigations do you want to do in all glomerulonephritis?

Answer 14

• Bloods
• Immunoglobulins
• Electrophoresis
• C3 & C4
• ANA, ANCA, ds-DNA, anti-GBM (autoantibodies)

Question 15

What imaging/further investigations do you want to do in all glomerulonephritis?

Answer 15

• CXR - pulmonary haemorrhage

- Renal US + biopsy

Question 16

What happens in post-streptococcal glomerulonephritis?

Answer 16

Following throat (2 weeks) or skin infection (3-6 weeks), strep antigen deposits in glomerulus causing immune complex formation and inflammation

Question 17

How does post-streptococcal glomerulonephritis present?

Answer 17

• Varies - haematuria or acute nephritis
• Oedema
• Raised BP
• Oliguria

Question 18

How do you diagnose post-streptococcal glomerulonephritis?

Answer 18

• Evidence of streptococcal infection
• Increased ASOT (antistreptolysin O titre) - blood test that looks for antibodies to the streptococcus A bacteria
• Anti-DNAase B - antibody produced by the immune system in response to a strep infection
• Reduced serum levels of C3

Question 19

How do you treat post-streptococcal glomerulonephritis?

Answer 19

• Supportive

- Antibiotics - to clear the nephritogenic bacteria

Question 20

What is goodpasture’s syndrome now called?

Answer 20

Anti-GBM disease

Question 21

What happens in anti-GBM disease?

Answer 21

Autoantibodies to type IV collagen present in glomerular and alveolar basement membranes

Question 22

How does anti-GBM disease present?

Answer 22

• Renal disease (oligouria/anuria, haematuria, AKI, renal failure)
• Lung disease (pulmonary haemorrhage - SOB, haemoptysis)

Question 23

How do you diagnose anti-GBM disease?

Answer 23

Anti-GBM in circulation/kidney (immunofluorescence for IgG)

Question 24

How do you treat anti-GBM disease?

Answer 24

• Plasma exchange
• Corticosteroids
• Cyclophosphamide

Question 25

What is rapidly progressive glomerulonephritis (crescentic GN)?

Answer 25

Any aggressive glomerulonephritis, rapidly progressing to renal failure in days or weeks

Question 26

What are causes of rapidly progressive glomerulonephritis?

Answer 26

- Small vessel/ANCA vasculitis - Lupus nephritis - Anti-GBM disease - Other GNs may transform to become rapid progressive incl. IgA, membranous

Question 27

How do you diagnose rapidly progressive glomerulonephritis?

Answer 27

Crescents seen on renal biopsy (due to breaks in the GBM allowing an influx of inflammatory cells)

Question 28

How do you treat rapidly progressive glomerulonephritis?

Answer 28

- Corticosteroids - Cyclophosphamide - Plasma exchange for anti-GBM/ANCA vasculitis - ?Mabs in lupus nephritis

Question 29

What indicates worse prognosis in anti-GBM disease?

Answer 29

- Dialysis dependence at presentation | - Increased crescents seen on biopsy