Histopathology 8 - Renal disease Flashcards
Which part of the nephron is impermeable to water?
Distal convuluted tubule
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
What are the genetic associations of PCKD?
PDK1 PDK2
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
Urine casts containing erthrocytes and leucocytes
Crescents present when it is bad enough to cause acute renal failure
What is acute crescentic glomerulonephritis?
Immune mediated inflammation of glomerulonephritis with crescents as main histopathological finding
Includes:
Anti-GBM disease
Pauci-immune disease
Which aetiologies might cause immune complex associated crescentic glomerulonephritis?
SLE
IgA nephropathy
Post-infectious glomerulonephritis
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 the features of Pauci-immune crescentic glomerulonephritis?
Only scanty glomerular Ig deposits
Usually ANCA associated
Vasculitis everywhere
What on earth is thrombotic microangiopathy? (no seriously if someone works this out pls pm me)
Damage to endothelium in glomeruli, arterioles and arteries leading to thrombosis??
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
Thrombotic microangiopathy
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
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?
Thrombotic microangiopathy
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 minimal change glomerulonephritis, how do glomeruli appear under electron microscopy?
Effacement 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
Does focal segmental glomerulonephritis produce the nephrotic or nephritic syndrome?
Nephrotic
What is the broad pathophysiology of primary membranous glomerulonephritis?
Immune complex related
Immune deposits are subepithelial, outside GBM
What age group does membranous glomerulonephritis usually affect?
Adults
What is the most common cause of membranous glomerulonephritis?
75% are immune
Antipody against phospholipase A2 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?
X-linked dominant
Affects alpha 5 subunit
What are the symptoms of Alport’s syndrome?
Renal failure in middle age
Deafness
Ocular 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)
What socring system is used to score IgA renal disease?
Oxford classification
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
What is the usual cause of non-diarrhoea-associated thrombotic microangiopathy?
ADAMT313 deficiency (eg scleroderma and anti-phospholipid syndrome)
Describe each stage of diabetic nephropathy
Stage 1: Basement Membrane thickening
Stage 2: Mesangial matrix expansion
Stage 3: Nodular lesions (Kimmelstiel-Wilson)
Stage 4: Advanced glomerulosclerosis
How is IgA nephropathy graded?
Oxford classification (MEST-C)
