2: Nephritic Syndrome Flashcards
What is glomerulonephritis
Immume-mediated damage to the glomerulus causing inflammation and damage that enables protein and blood to pass into the urine
What are the two types of glomerulonephritis
Nephritic syndrome
Nephrotic syndrome
How does nephritic syndrome present
- Haematuria
- Oedema
- RBC casts in urine
- Oliguria
- HTN
- Proteinuria <3.5g/day
What is a mnemonic to remember causes of nephritic syndrome
PIGARMS
What are the causes of nephritic syndrome
Post-streptococcal glomerulonephritis
IgA neprhopathy
Good-pastures, Granulomatous with polyangitis
Alport’s syndrome
Rapidly progressive glomerolonephritis
Membroproliferative glomerulonephritis
SLE
What is post-streptococcal glomerulonephritis
Occurs following infection with Group A streptococcus
What age does post-streptococcal glomerulonephritis affect
Children: 3-12 years-old
What time frame after infection does post-streptococcal glomerulonephritis occur
1-2 Weeks
Name two streptococcal infections that can lead to post-streptococcal glomerulonephritis
- Pharyngitis
- Impetigo
What are the symptoms of post-streptococcal glomerulonephritis
- Coca-cola urine (haematuria)
- Proteinuria (>3.5g/day)
- Oliguria
What investigations can be ordered in post-streptococcal glomerulonephritis
Antistreptolysin O titre (ASOT)
Explain prognosis of post-streptococcal glomerulonephritis
Self-resolving in one month
What is IgA neprhopathy also known as
Berger disease
What is the most common cause of nephropathy worldwide
IgA nephropathy
What is the stereotype for Bergers disease
Young male, presents with episodes of recurrent haematuria following recurrent URTIs
How does Bergers disease present
1-2 days after URTI with macroscopic haematuria
NO proteinuria
What is a way to remember time frame that IgA neprhopathy is 1-2 days and post-streptococcal is 1-2W
A comes before S
What blood-test can be ordered in IgA nephropathy to confirm diagnosis
Raised total serum IgA
What is a good prognostic factor in IgA nephropathy
Frank Haematuria
What are poor prognostic factors in IgA nephropathy
Male Smoker HTN Hyperlipidaemia ACEi
What is anti-GBM disease also known as
Goodpasture’s syndrome
When should good pasture’s be suspected
Haemoptysis and haematuria
What age does goodpasture’s tend to occur
20-30 or 60-70 years-old
What gene is goodpasture’s associated with
HLADR2
What environmental factors increase risk of good pastures
Smoking
Infection
Oxidative-stress
Hydocarbon-based solvent
How does goodpasture’s present clinically
Haemoptysis and haematuria
Do lung or kidney symptoms start first in good pastures
Lung symptoms occur before kidney symptoms
What type of collagen are autoantibodies raised to in good pasture’s syndrome
Collagen type IV
What happens when autoantibodies bind to collagen type IV
Activate the complement system
How is good pastures syndrome managed
- Plasmapheresis
- Glucocorticoids
- Cyclophosphamide
What can good pastures syndrome lead to
- Rapidly progressive glomerulonephritis
What is alports syndrome
Genetic condition
What is the inheritance pattern of Alport syndrome
X Linked Dominance
Given Alport syndrome is an x-linked dominant condition, which gender are affected more
Males
What age does Alport Syndrome tend to occur
Haematuria may be present during infancy, however severe symptoms manifest during adolescence
Outline the clinical presentation of Alport Syndrome
- Intermittent gross haemaruia
- ESRD: as glomerular damage progresses causes decrease in GFR
- SNHL
- Eye involvement
What collagen is defective in Alport syndrome
IV Collagen
What is the confirmatory test for Alport syndrome
Skin Biopsy - absence of type IV collagen a5 chain fibres
What will be seen on skin biopsy in Alport Syndrome
Absence of type IV collagen a5 chain fibres
What ocular deficit may be seen in Alport Syndrome
Anterior lenticonus
Perimacular flecks
Explain management of Alport Syndrome
- ACEi
- Hearing aids
- Surgery for anterior lenticonus
RRT = only definitive management
What is rapidly progressive glomerulonephritis also known as
Cresenteric glomerulonephritis
What is rapidly progressive glomerulonephritis
Inflammatory disease causing rapid destruction of glomeruli leading to ESRD
What is type I RPGN
anti-GBM mediated
What is type II RPGN
Due to immune complex deposition
Give examples of immune complex mediated type II RGPN
- SLE
- Post-streptococcal glomerulonephritis
- IgA neprhopathy
What is type 3 RPGN
Pauci-immune
What is pauci immune
No immune complex deposition
What are two types of type 3 RPGN
- Granulomatosis with polyangitis
- Eosinophillis granulomatosis with polyangitis
What antibody is present in granulomatosis with polyangitiis
C-ANCA
What antibody is present in eosinophillic granulomatosis with polyangitis
P-ANCA
How does rapidly progressive glomerulonephritis present
Nephritis syndrome: haematuria, oliguria, oedema
What will be seen on light-microscopy in rapidly progressive glomerulonephritis
Cresenteric cells
How is cresenteric glomerulonephritis treated
- Anticoagulants
- Glucocorticoids
- Cyclophosphamide
- RRT
