Glomerulonephritis

Question 1

Glomerulonephritis cover many conditions which may present with symptoms of either _____ or ______ syndrome

Answer 1

nephritic or nephrotic

Question 2

Types of glomerulonephritis all cause inflammation of what?

Answer 2

Glomeruli in the kidneys

Question 3

What is the most common cause of primary glomerulonephritis?

Answer 3

IgA nephropathy (Berger’s disease)

Question 4

What happens in IgA nephropathy (Berger’s disease)?

Answer 4

Exact cause is unclear.
Typically patient in 20s with haematuria. Histology shows IgA deposits and mesangial proliferation.

Abnormal IgA clump together and build up causing inflammation and damage to glomeruli.

Question 5

Is IgA nephropathy primarily a nephritic or nephrotic syndrome?

Answer 5

Nephritic - characterised by haematuria and hypertension

Question 6

Membranous nephropathy involves deposits of i______ c_______ in the g_____ b_____ m_____

Answer 6

immune complexes in the glomerular basement membrane

Question 7

What happens in membranous nephropathy?

Answer 7

Autoimmune attack on glomeruli. Antibodies form deposits triggering inflammation and causes membrane thickening. Protein leaks through into urine.

Question 8

What does histology show in membranous nephropathy?

Answer 8

IgG and complement deposits on the basement membrane.

Question 9

Why does membranous nephropathy occur?

Answer 9

Often idiopathic.
Can be secondary to malignancy, SLE or drugs like NSAIDs.

Question 10

Is membranous nephropathy primarily a nephritic or nephrotic syndrome?

Answer 10

Nephrotic - characterised by proteinuria, oedema and hypoalbuminaemia.

Most common cause of nephrotic syndrome in adults.

Question 11

What age group does membranoproliferative glomerulonephritis typically affect?

Answer 11

Under 30s

Question 12

What is membranoproliferative glomerulonephritis?

Answer 12

Similarly to membranous glomerulonephritis, immune complexes in glomeruli lead to inflammation and thickening. Also causes proliferation so more mesangial cells present.

Question 13

What disease is membranoproliferative glomerulonephritis associated with?

Answer 13

Hepatitis C
SLE

Question 14

What kind of glomerulonephritis can occur 1-3 weeks after an infection?

Answer 14

Post-streptococcal glomerulonephritis (eg tonsillitis, impetigo)

Question 15

Does post-streptococcal glomerulonephritis present with more nephritic or nephrotic symptoms?

Answer 15

Nephritic (haematuria, oliguria and oedema)

Question 16

What can be seen on a biopsy of rapidly progressive glomerulonephritis?

Answer 16

Microscopic glomerular crescent formations

Question 17

Does rapidly progressive glomerulonephritis present with more nephritic or nephrotic symptoms?

Answer 17

Nephritic - haematuria

Question 18

What are defining features of rapidly progressive glomerulonephritis?

Answer 18

Rapid loss of kidney function
Weakness, fatigue, fever, N+V, abdo pain
haematuria
Progresses to renal failure within weeks if untreated.

Question 19

What are causes of rapidly progressive glomerulonephritis?

Answer 19

Anti-glomerular basement membrane antibody disease
Granulomatosis with polyangiitis
Microscopic polyangiitis

Question 20

What is anti-glomerular basement membrane disease (anti-GBM) also called?

Answer 20

Goodpasture syndrome

Question 21

In Goodpasture syndrome, the Anti-GBM antibodies attack what?

Answer 21

The glomerulus and the pulmonary basement membrane

Question 22

In Goodpasture syndrome, when the anti-GBM attacks the glomerulus and pulmonary basement membranes, it causes g_____ and p____ h_______

Answer 22

glomerulonephritis
pulmonary haemorrhage

Question 23

How do patients with Goodpasture syndrome present?

Answer 23

In 20s or 60s
Have AKI
and haemoptysis

Question 24

Which antibody would indicate microscopic polyangiitis in a patient with AKI and haemoptysis?

Answer 24

p-ANCA

Question 25

Which antibody would indicate granulomatosis with polyangiitis in a patient with AKI and haemoptysis?

Answer 25

c-ANCA

Question 26

What antibodies would you expect to find in a patient with Goodpasture syndrome?

Answer 26

Anti-GBM antibodies

Question 27

How is Goodpasture syndrome managed?

Answer 27

High dose immunosuppression eg IV prednisolone

Question 28

What sized vessels does granulomatosis with polyangiitis affect?

Answer 28

Small and medium-sized vessels in many organs

Question 29

What antibodies cause granulomatosis with polyangiitis (vasculitis causing rapidly progressive glomerulonephritis)?

Answer 29

c-ANCA (anti-neutrophil cytoplasmic antibodies)

Question 30

What happens in granulomatosis with polyangiitis?

Answer 30

Autoantibodies (c-ANCA) activate neutrophils, causing them to damage vessel walls leading to inflammation, necrosis and granuloma formation. There is little/no immune complex deposition.

Question 31

What would a biopsy of the kidney show in granulomatosis with polyangiitis?

Answer 31

Necrotising granulomas (cell death surrounding by giant cells and histiocytes) Vasculitis Glomerulonephritis with crescents V few immune deposits.

Question 32

What sized vessels does microscopic polyangiitis affect?

Answer 32

Small vessel vasculitis

Question 33

Is there any evidence of necrotising granulomatous inflammation in microscopic polyangiitis?

Answer 33

No (there is in granulomatosis with polyangiitis)

Question 34

What drives the vessel wall inflammation in microscopic polyantiitis?

Answer 34

p-ANCA

Question 35

Why is plasmapheresis useful in managing acute microscopic polyangiitis?

Answer 35

Removes p-ANCA antibodies

Question 36

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

Answer 36

Minimal change glomerulonephritis

Question 37

What is minimal change glomerulonephritis also called?

Answer 37

Minimal change disease

Question 38

What happens in minimal change disease?

Answer 38

Not well understood. T-cells release a factor that injures podocytes and they become leaky to albumin. Very minimal inflammation and no immune complex deposition. Under light microscopy, looks normal (hence name). Is mostly idiopathic, may be due to drugs like NSAIDs or Hodgkin lymphoma or allergic reactions.

Question 39

What are clinical features of minimal change disease?

Answer 39

Mass proteinuria Hypoalbuminaemia Oedema Hyperlipidaemia

Question 40

How is minimal change disease managed?

Answer 40

High dose corticosteroids (prednisolone) are very effective. Salt and fluid restriction.

Question 41

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

Answer 41

Focal segmental glomerulosclerosis (FSGS)

Question 42

What occurs in focal segmental glomerulosclerosis?

Answer 42

There is podocyte damage (eg from virus, mechanical stress, genetics...) Podocytes detach and bare areas of glomerular basement membrane appear. Hyaline is deposited in these areas causing sclerosis. Proteins pass through barrier. Remaining nephrons hyperfilter to compensate causing more podocyte injury.

Question 43

Does focal segmental glomerulosclerosis respond well to steroids?

Answer 43

Often not well, unlike minimal change disease (in children more commonly, responds very well to steroids).

Question 44

What does focal segmental glomerulosclerosis often progress to?

Answer 44

CKD