2: Nephritic Syndrome

Question 1

What is glomerulonephritis

Answer 1

Immume-mediated damage to the glomerulus causing inflammation and damage that enables protein and blood to pass into the urine

Question 2

What are the two types of glomerulonephritis

Answer 2

Nephritic syndrome

Nephrotic syndrome

Question 3

How does nephritic syndrome present

Answer 3

• Haematuria
• Oedema
• RBC casts in urine
• Oliguria
• HTN
• Proteinuria <3.5g/day

Question 4

What is a mnemonic to remember causes of nephritic syndrome

Answer 4

PIGARMS

Question 5

What are the causes of nephritic syndrome

Answer 5

Post-streptococcal glomerulonephritis

IgA neprhopathy

Good-pastures, Granulomatous with polyangitis

Alport’s syndrome

Rapidly progressive glomerolonephritis

Membroproliferative glomerulonephritis

SLE

Question 6

What is post-streptococcal glomerulonephritis

Answer 6

Occurs following infection with Group A streptococcus

Question 7

What age does post-streptococcal glomerulonephritis affect

Answer 7

Children: 3-12 years-old

Question 8

What time frame after infection does post-streptococcal glomerulonephritis occur

Answer 8

1-2 Weeks

Question 9

Name two streptococcal infections that can lead to post-streptococcal glomerulonephritis

Answer 9

• Pharyngitis

- Impetigo

Question 10

What are the symptoms of post-streptococcal glomerulonephritis

Answer 10

• Coca-cola urine (haematuria)
• Proteinuria (>3.5g/day)
• Oliguria

Question 11

What investigations can be ordered in post-streptococcal glomerulonephritis

Answer 11

Antistreptolysin O titre (ASOT)

Question 12

Explain prognosis of post-streptococcal glomerulonephritis

Answer 12

Self-resolving in one month

Question 13

What is IgA neprhopathy also known as

Answer 13

Berger disease

Question 14

What is the most common cause of nephropathy worldwide

Answer 14

IgA nephropathy

Question 15

What is the stereotype for Bergers disease

Answer 15

Young male, presents with episodes of recurrent haematuria following recurrent URTIs

Question 16

How does Bergers disease present

Answer 16

1-2 days after URTI with macroscopic haematuria

NO proteinuria

Question 17

What is a way to remember time frame that IgA neprhopathy is 1-2 days and post-streptococcal is 1-2W

Answer 17

A comes before S

Question 18

What blood-test can be ordered in IgA nephropathy to confirm diagnosis

Answer 18

Raised total serum IgA

Question 19

What is a good prognostic factor in IgA nephropathy

Answer 19

Frank Haematuria

Question 20

What are poor prognostic factors in IgA nephropathy

Answer 20

Male
Smoker
HTN
Hyperlipidaemia 
ACEi

Question 21

What is anti-GBM disease also known as

Answer 21

Goodpasture’s syndrome

Question 22

When should good pasture’s be suspected

Answer 22

Haemoptysis and haematuria

Question 23

What age does goodpasture’s tend to occur

Answer 23

20-30 or 60-70 years-old

Question 24

What gene is goodpasture’s associated with

Answer 24

HLADR2

Question 25

What environmental factors increase risk of good pastures

Answer 25

Smoking Infection Oxidative-stress Hydocarbon-based solvent

Question 26

How does goodpasture's present clinically

Answer 26

Haemoptysis and haematuria

Question 27

Do lung or kidney symptoms start first in good pastures

Answer 27

Lung symptoms occur before kidney symptoms

Question 28

What type of collagen are autoantibodies raised to in good pasture's syndrome

Answer 28

Collagen type IV

Question 29

What happens when autoantibodies bind to collagen type IV

Answer 29

Activate the complement system

Question 30

How is good pastures syndrome managed

Answer 30

- Plasmapheresis - Glucocorticoids - Cyclophosphamide

Question 31

What can good pastures syndrome lead to

Answer 31

- Rapidly progressive glomerulonephritis

Question 32

What is alports syndrome

Answer 32

Genetic condition

Question 33

What is the inheritance pattern of Alport syndrome

Answer 33

X Linked Dominance

Question 34

Given Alport syndrome is an x-linked dominant condition, which gender are affected more

Answer 34

Males

Question 35

What age does Alport Syndrome tend to occur

Answer 35

Haematuria may be present during infancy, however severe symptoms manifest during adolescence

Question 36

Outline the clinical presentation of Alport Syndrome

Answer 36

- Intermittent gross haemaruia - ESRD: as glomerular damage progresses causes decrease in GFR - SNHL - Eye involvement

Question 37

What collagen is defective in Alport syndrome

Answer 37

IV Collagen

Question 38

What is the confirmatory test for Alport syndrome

Answer 38

Skin Biopsy - absence of type IV collagen a5 chain fibres

Question 39

What will be seen on skin biopsy in Alport Syndrome

Answer 39

Absence of type IV collagen a5 chain fibres

Question 40

What ocular deficit may be seen in Alport Syndrome

Answer 40

Anterior lenticonus | Perimacular flecks

Question 41

Explain management of Alport Syndrome

Answer 41

- ACEi - Hearing aids - Surgery for anterior lenticonus RRT = only definitive management

Question 42

What is rapidly progressive glomerulonephritis also known as

Answer 42

Cresenteric glomerulonephritis

Question 43

What is rapidly progressive glomerulonephritis

Answer 43

Inflammatory disease causing rapid destruction of glomeruli leading to ESRD

Question 44

What is type I RPGN

Answer 44

anti-GBM mediated

Question 45

What is type II RPGN

Answer 45

Due to immune complex deposition

Question 46

Give examples of immune complex mediated type II RGPN

Answer 46

- SLE - Post-streptococcal glomerulonephritis - IgA neprhopathy

Question 47

What is type 3 RPGN

Answer 47

Pauci-immune

Question 48

What is pauci immune

Answer 48

No immune complex deposition

Question 49

What are two types of type 3 RPGN

Answer 49

- Granulomatosis with polyangitis | - Eosinophillis granulomatosis with polyangitis

Question 50

What antibody is present in granulomatosis with polyangitiis

Answer 50

C-ANCA

Question 51

What antibody is present in eosinophillic granulomatosis with polyangitis

Answer 51

P-ANCA

Question 52

How does rapidly progressive glomerulonephritis present

Answer 52

Nephritis syndrome: haematuria, oliguria, oedema

Question 53

What will be seen on light-microscopy in rapidly progressive glomerulonephritis

Answer 53

Cresenteric cells

Question 54

How is cresenteric glomerulonephritis treated

Answer 54

- Anticoagulants - Glucocorticoids - Cyclophosphamide - RRT