Histopathology - Renal disease Flashcards
Which part of the nephron is impermeable to water?
Distal convuluted tubule
thick ascending loop
What is a “horse shoe kidney”?
Congenital renal fusion
What is the inheritance pattern of adult polycystic kidney disease?
Dominant
What is the triad of symptoms/signs associated with adult PCKD?
Hypertension
Flank pain
Haematuria
2 mutations in PCKD?
PKD 1 on Chr 16 (polysystin 1)
PKD 2 on Ch 4 (polycystic 2)
What is the most strongly associated aneurysm with PCKD?
Berry aneurysm
In which patients are kidney cysts most likely to form?
End stage renal failure and on dialysis
How can the causes of acute renal failure be classified?
Pre-renal/renal/post-renal
What is the most common cause of acute renal failure?
Acute tubular injury
How does acute tubular injury affect glomerular filtration?
Blockage of tubules by casts –> leakage into interstitial space –> secondary haemodynamic changes that affect GFR
What is acute tubulo-interstitial nephritis, and what causes it?
Injury to tubules and interstitium that is usually immune but can also be caused by infection/drugs
What is the most common cause of acute tubulo-interstitial nephritis?
Drugs (especially NSAIDs)
How does acute glomerulonephritis present?
Oligouria
red cell casts
Crescents present when it is bad enough to cause acute renal failure
What is rapidly progressive (crescentic) glomerulonephritis? what are the types
Immune mediated inflammation of glomerulonephritis with crescents as main histopathological finding
Includes:
type 1 - Anti-GBM disease
type 2- immune complex mediated
type 3 - ANCA associated
Which 5 aetiologies might cause immune complex associated crescentic glomerulonephritis?
SLE
IgA nephropathy
Post-infectious glomerulonephritis
alport
HSP
What is anti-GBM disease?
Rare and severe disease caused by Ig directed against the GBM
How can anti-GBM disease affect the lung?
Cross-reaction with alveolar basement membrane leading to pulmonary haemorrhage
What are 3 features of Pauci-immune crescentic glomerulonephritis?
lack of/ scanty immune complex deposition
ANCA associated ( c- wegengers, p- MPA)
Vasculitis everywhere
outline the PHAROH of nephritic syndrome
proteinuria (less than nephrotic)
hamateuria
azootemia (high ureas & creatinine)
red cell casts in utrine
oliguria
hypertension
What is nephrotic syndrome, and what are is its four diagnostic requirements?
Breakdown in selectivity of glomerular filtration barrier leading to protein leak
Proteinuria (>3.5g/day)
Hypoalbuminaemia
Oedema
Hyperlipidaemia
Recall a pre-renal cause of acute renal failure
Failure of perfusion of kidney
Recall 3 renal causes of acute renal failure
Acute tubular injury
Acute glomerulonephritis
vasculitis
How can acute renal failure be caused post-renally?
Obstruction
How can trauma cause acute renal failure?
Release of myoglobin damages tubular epithelial cells, causing acute tubular injury (rhabodmyolysis)
Which antibodies are present in pauci-immune acute crescentic glomerulonephritis?
Anti-neutrophil cytoplasm Ig aka ANCA
How can immune complexes in the glomerulus be identified?
Immunohistochemistry
Electron microscopy
Which syndromes are associated with Pauci-immune Crescentic Glomerulonephritis?
Wegener’s
Churg Strauss
Microscopic polyangiopathy
Which kidney disease is associated with E coli diarrhoea?
HUS
Recall 2 examples of non-immune complex related, primary nephrotic syndrome
Minimal change disease
Focal segmental glomerulosclerosis
Recall an example of a primary cause of nephrotic syndrome that is immune mediated?
Membranous glomerulonephritis
Recall 3 systemic diseases that can cause nephrotic syndrome
Diabetes mellitus
Amyloidosis
SLE
in all the 3 primary nephrotic sydnromes, how do glomeruli appear under electron microscopy?
loss of foot processes
What is a common cause of the nephrotic syndrome in children?
Minimal change disease
What treatment does minimal change glomerulonephritis usually respond to?
Immunosuppression
1st line = steroids
2nd = cyclosporine
Does focal segmental glomerulonephritis produce the nephrotic or nephritic syndrome?
Nephrotic
What is the broad pathophysiology of membranous glomerular disease
Immune deposits are subepithelial, outside of GBM
also thickening of BM
What age group does membranous glomerular disease sually affect?
Adults
what antibody is commonly seen in membranous glomerular disease?
anti- phospholipase A2 type M receptor
Does diabetic nephropathy cause nephrotic or nephritic syndrome?
Nephrotic
What is amyloidosis?
Deposition of extracellular proteinaceous material exhibiting β-sheet structure
What is the inheritance pattern of Alport’s syndrome? what mutation
X-linked
type IV collagen alpha 5 chain
What are the triad of symptoms of Alport’s syndrome?
nephritic syndrome
sensorineural deafness
eye disease
What is the most common form of glomerulonephritis?
IgA nephropathy
What eGFR is indicative of end-stage renal failure?
<15
What is the most common cause of chronic renal failure?
Diabetes
What are anti-GBM antibodies directed against?
The C terminal domain of type IV collagen
What kidney disease is characterised by shrunken kidneys with granular cortices?
Hypertensive nephropathy
What stain is used to detect amyloidosis?
Congo red (–> apple green birefringeance)
in whom is FSGS most cmonnon
afro carribean adults
What might histopathology show in hypertensive nephropathy?
Nephrosclerosis
What is the commonest cause of kidney failure requiring renal replacement therapy?
Diabetes
How does SLE affect the kidney?
Anti-nuclear anti-dsDNA Ig directed against a broad range of intracellular and extracellular antigens
Does SLE cause the nephrotic or nephritic syndrome?
Nephrotic
What would be the histological appearance of acute tubulo-interstitial nephritis?
Eosinophils and granulomas
Describe each stage of diabetic nephropathy
Stage 1: Basement Membrane thickening
Stage 2: Mesangial matrix expansion
Stage 3: Kimmelstiel-Wilson nodules
Stage 4: Advanced glomerulosclerosis (scarring)
How is IgA nephropathy graded?
Oxford classification (MEST-C)
muddy brown casts in urine =
pathognomonic for ATN
what bacteria is implicated in post strep GN
usually 1-3 WEEKS after thoroat infection or impetigo so
group A haemolytic strep = strep pyogenes
2 bloods for post strep GN
C3 down
ASOT titre up
what’s seen on microscopy in post strep GN
granular despots of IgG and C3 in GBM
most common GN worldwide?
IgA nephropathy = BERGER disease
IgA nephropathy is more common in what race
east/south asiana
when does IgA nephropathy tend to present after an UTRI
1-2 DAYS (much earlier than post strep GN)
PRESENTS W FRANK HAEMATURIA
blood results in IgA nephropathy
IgA up
note than 1/3rd will progress to CKD
give a rare dominant inherited cause of nephritic syndrome. what mutation
thin membrane disease (benign familial haematuria)
type IV collagen alphan 4 chain
usually asymtomatic
give 3 conditions that typically cause asymptomatic haematuria
alport
thin membrane disease
IgA nephropathy
what is lupus nephritis. give a histo finding
GN caused by lupus
immune complex deposition-
in capillaries = wire loop capillaries
in GBM = lumpy-bumpy granular fashion
give 3 priamry causes of nephrotic syndrome
minimal change disease
membranous gloemrualr disease
focal segmental sloemrulosclerosis
give 5 diseases that cause a nrphritic syndrome
post strep GN
IgA nephropathy
rapidly progressive/crescenteric
alport’s syndrome
thin basement memebran disease
microscopy findings of membranous glomerular disease
loss of poocytes for all three nephrotics
ALSO spikey subepithelial deposits
WHAT NEPHRTOIC SYDROM SHOWS DIFFUSE THICKENING OF GBM
MEMBRANOUS
