Urinary 10 - Glomerular disease

Question 1

What is the main problem in the kidney causing nephrotic syndrome?

Answer 1

Damaged glomerulus = increased permeability = LEAKS PROTEIN

Question 2

Give 2 major symptoms in nephrotic syndrome:

Answer 2

1) Proteinuria > 3.5g/24hrs (+ oedema)

2) Foamy/frothy urine

Question 3

Where is the most likely site of injury in the glomerulus causing glomerular disease?

Answer 3

Subepithelial layer (podocytes)

Question 4

Name 3 common primary causes of Nephrotic syndromw:

Answer 4

1) Minimal change glomerulonephritis
2) Focal segmental glomerulosclerosis
3) Membranous glomerulonephritis

Question 5

What is glomerulonephritis?

Answer 5

Any condition associated with inflammation of the glomerular tuft

Question 6

At what age does Minimal Change Glomerulonephritis typically present?

Answer 6

Childhood/Adolescence

Question 7

What is the main treatment for Minimal Change Gloerulonephritis?

Answer 7

Steroids

Question 8

Does Minimal Change Glomerulonephritis typically progress to renal failure?

Answer 8

No

Question 9

Describe the pathogenesis causing Minimal Change Glomerulonephritis:

Answer 9

Unknown circulating factor damaging podocytes, causing increased permeability of glomerulus

Question 10

What is the name given to any condition associated with inflammation of the glomerular tuft?

Answer 10

Glomerulonephritis

Question 11

What is the meaning of ‘focal’ when describing glomerular pathology?

Answer 11

Involving <50% of glomeruli on light microscopy

Question 12

What is the meaning of ‘segmental’ when describing glomerular pathology?

Answer 12

Involving part of the glomerular tuft

Question 13

What is the meaning of ‘glomerulosclerosis’ when describing glomerular pathology?

Answer 13

Segmental/global capillary collapse, presumed little/no filtration in hardened/sclerotic areas

Question 14

At what age does Focal Segmental Glomerulosclerosis typically present?

Answer 14

~ 40-60 yrs

Question 15

Does Focal Segmental Glomerulosclerosis typically progress to renal failure?

Answer 15

Yes

Question 16

Describe the pathogenesis causing Focal Segmental Glomerulosclerosis:

Answer 16

Unknown circulating factor damaging podocytes

Question 17

Name the 2 glomerular pathologies caused by unknown circulating factors damaging podocytes:

Answer 17

1) Minimal Change Glomerulonephritis

2) Focal Segmental Glomerulosclerosis

Question 18

What is the meaning of ‘membranous’ when describing glomerular pathology?

Answer 18

Thickening of the glomerular capillary wall

Question 19

Name the commonest cause of Nephrotic syndrome in adults:

Answer 19

Membranous glomerulonephritis

Question 20

What age does membranous glomerulonephritis typically present?

Answer 20

Adulthood

Question 21

Describe the outlook of a patient with membranous glomerulonephritis:

Answer 21

Rule of thirds:
1/3 = will get better
1/3 = will stay the same
1/3 = will progress to renal failure

Question 22

Describe the pathogenesis of membranous glomerulonephritis:

Answer 22

Deposition of immune complexes in the basement membrane under the podocyte layer
= Complement cascade activated
= Cellular injury to podocytes
(Often secondary to malignancies like Lymphoma)

Question 23

Name the antigen and antibody which forms the immune complexes deposited in membranous glomerulonephritis:

Answer 23

Antigen = Phospholipase A2 receptor on podocyte
Antibody = IgG

Question 24

How does proteinuria in nephrotic syndrome lead to oedema?

Answer 24

Increased protein in urine
= decreased protein in blood
= water moves from blood into interstitial space
= oedema

Question 25

What is the most likely cause of persistently foamy urine?

Answer 25

Proteinuria

Question 26

Name 2 common secondary causes of nephrotic syndrome:

Answer 26

1) Diabetes mellitus | 2) Amyloidosis

Question 27

Describe the pathogenesis of diabetes mellitus causing nephrotic syndrome:

Answer 27

Persistent high blood glucose injures all parts of the glomerulus: - Basement membrane thickens and becomes abnormal - Sclerotic nodules form in mesangium

Question 28

What is the main problem in the kidney causing nephritic syndrome?

Answer 28

Blocked glomerulus

Question 29

List the main features of nephritic syndrome:

Answer 29

- Renal failure = oedema, oliguria, SOB - Haematuria (microscopic/macroscopic) - Decreased eGFR - Red cell casts in urine

Question 30

Where is the most likely site of injury of nephritic syndrome?

Answer 30

Endothelium

Question 31

Which glomerular syndrome describes the blocking of the glomerulus?

Answer 31

Nephritic syndrome

Question 32

Which glomerular syndrome describes protein leaking through the glomerulus?

Answer 32

Nephrotic syndrome

Question 33

Give 5 diseases associated with Nephritic syndrome:

Answer 33

1) IgA nephropathy 2) Thin GBM nephropathy 3) Alport syndrome 4) Goodpasture syndrome 5) Vasculitis

Question 34

Which glomerular syndrome is IgA nephropathy associated with?

Answer 34

Nephritic syndrome

Question 35

Which glomerular syndrome is Thin GBM nephropathy associated with?

Answer 35

Nephritic syndrome

Question 36

Which glomerular syndrome is Alport syndrome associated with?

Answer 36

Nephritic syndrome

Question 37

Which glomerular syndrome is Goodpasture syndrome associated with?

Answer 37

Nephritic syndrome

Question 38

Which glomerular syndrome is Vasculitis associated with?

Answer 38

Nephritic syndrome

Question 39

Which disease is the most common primary cause of glomerulonephritis?

Answer 39

IgA nephropathy

Question 40

Describe the presentation of IgA nephropathy:

Answer 40

Any age Haematuria Proteinuria (foamy urine) Activated by URTI

Question 41

Describe the pathogenesis of IgA nephropathy:

Answer 41

IgA deposited anywhere in glomerulus | = Blocked filter (irreversible)

Question 42

Which nephropathy is typically activated by a URTI?

Answer 42

IgA nephropathy = Nephritic syndrome

Question 43

What is the treatment of IgA nephropathy?

Answer 43

No cure - IgA deposition = irreversible - Treat hypertension, dialysis, transplant

Question 44

What is the inheritance pattern of Thin GBM nephropathy?

Answer 44

Autosomal dominant

Question 45

What is the typical inheritance pattern of Alport syndrome?

Answer 45

X-linked recessive

Question 46

Why is Goodpasture's syndrome called a 'cresentic' glomerulonephritis?

Answer 46

Causes accumulation of cells in Bowman's capsule, which compresses the glomerulus forming a crescent shape

Question 47

Describe the pathogenesis of Goodpasture's syndrome:

Answer 47

Autoimmune: = IgG (anti-GBM autoantibody) attacks collagen IV in basement membrane = Glomerulus becomes ball of inflammatory cells = Heals by scarring therefore reduces filtration

Question 48

Why does Goodpasture's syndrome sometimes present with haemoptysis?

Answer 48

Autoantibodies attack collagen IV, which is also present in alveoli membranes. = More common when lungs are already damaged, ie in smokers

Question 49

What is the treatment of Goodpasture's syndrome?

Answer 49

No cure, can prevent worsening via immunosuppresants and plasmaphoresis

Question 50

Name 2 crescentic glomerulonephritis', and their associated glomerular syndromes:

Answer 50

1) Goodpasture's syndrome 2) Vasculitis (ANCA) Both cause nephritic syndrome

Question 51

Describe the pathogenesis of Vasculitis (nephropathy):

Answer 51

ANCA (Anti-Neutrophil Cytoplasmic Antibody) causes inflammation of blood vessels