Histopathology 10: Renal Disease Flashcards
List the triad of symptoms that define Nephrotic syndrome?
breakdown in selectivity of glomerular filtration barrier leading to protein leak
-Proteinuria (>3g/24h)
-Hypoalbuminaemia
-Oedema
(hyperlipidaemia)
What is the most common cause of nephrotic syndrome in children ?
Minimal change disease
Electron microscopy shows loss of podocyte foot processes With no signs of immune deposits on immunofluorescence. This suggests ………. a cause of Nephrotic syndrome.
Minimal change disease
glomeruli look normal on light microscopy
effacement of foot processes on electron microscopy
responds to immunosuppression
Microscopy shows ‘Spikey” subepithelial deposits and diffuse glomerular membrane thickening. This is most likely ………… a cause of Nephrotic syndrome with a poor prognosis.
Membranous glomerular disease
membrane deposits on the outside of GBM
common cause of nephrotic syndrome in adults
primary disease is AI - antibody against phospholipase A2 type M receptor
Light microscopy shows focal and segmental glomerular consolidation and scarring. The Afro-Caribbean lady is most likely suffering with ……… a cause of Nephrotic syndrome.
Focal segmental glomerulosclerosis (FSGS)
some glomeruli are partly scarred
less likely to respond to immunosuppression
What causes Apple green birefringence with Congo red stain ?
Amyloidosis
deposition of EC proteinaceous material exhibiting B sheet structure
Does multiple myeloma cause AL or AA amyloidosis ?
AL
List 2 causes of AA amyloidosis?
Arthritis
TB
(Cause by chronic inflammation)
A mnemonic for the characteristic features of Nephritic syndrome is PHAROH. List the characteristic features?
Proteinuria (less than Nephrotic syndrome) Haematuria Azootemia (high urea and creatinine) Red cell casts Oliguria Hypertension
List 3 causers of Nephrotic syndrome ?
Minimal change disease
Membranous glomerular disease
Focal segmental glomerulosclerosis (FSGS)
Post streptococcal glomerulonephitis usually occurs 1-3 weeks post throat infection with ………………. or after Impetigo. The bloods show increased ……. and decreased …….. . Immunoflouresence shows …………………………. in the ……….. and this is a cause of nephritic syndrome.
group A alpha haemolytic streptococcus (S.pyogenes) ASOT (antistreptolysin O titre) C3 Granular deposits of IgG and C3 Basement membrane
Which cause of Nephritic syndrome characteristically occurs 1-2 days after a URTI with frank haematuria?
IgA nephropathy
Which cause of Nephritic syndrome is most aggressive and presents with particularly pronounced renal failure and oliguria?
Rapidly progressive Glomerulonephritis
What is the characteristic feature of all types of rapidly progressive glomerulonephritis?
Crescents in the glomeruli
A patient has pulmonary haemorrhage and haematuria. ImmunoFluorescence shows IgG deposition in the GBM. This is most likely ……………….
Goodpasture’s syndrome
Which HLA type is associated with Goodpasteures syndrome ?
HLA-DRB1
The main pathogenesis of Goodpasteures syndrome is ………
Anti-GMB antibodies
SLE causes Rapidly progressive glomerulonephritis. Immunoflourescence shows ……… deposition on the GMB.
Granular IgG immune complex
List 2 Pauci-immune causes of Rapidly progressive glomerulonephritis?
Granulomatosis with polyangitis (Wegener’s granulomatosis)
Microscopic polyangitis
Match these
Granulomatosis with polyangitis
Microscopic Polyangitis.
p-ANCA
c-ANCA
ANA
AMA
Granulomatosis with polyangitis c-ANCA
Microscopic Polyangitis. p-ANCA
Immunoflourescence shows scanty immune complex deposition. This suggests a ………… cause off rapidly progressive glomerulonephritis
Pauci-immune (granulomatosis with polyangitis, Microscopic polyangitis)
List the triad of characteristic features of Alport syndrome?
Nephritic syndrome
Sensorineural deafness
Eye disorders (cataracts, lens dislocation)
Benign familial haematuria is caused by ………… and shows asymptomatic haematuria which has ………….. inheritance.
Thinning of GBM
Autosomal dominant
List 3 diseases that cause asymptomatic haematuria ?
Alport syndrome
IgA nephropathy
Benign familial haematuria (thin basement membrane syndrome)
List 3 causes of ATN ?
Hypovolaemia
Drugs (NSAIDs)
Contrast for radiological imaging
heavy metals
What is the most common cause of acute pyelonephritis ?
E.coli
Which 2 scans are used to investigate VUR in children ?
MCUG (micturition cystourogram)- identifies the reflux
DMSA - identifies renal scarring
How does acute interstitial nephritis present?
Fever, skin rash, haematuria, anaemia, proteinuria caused by a hypersensitivity reaction after exposure to a drug such as NSAIDs or antibiotics
List the triad for HUS ?
MAHA
Thrombocytopaenia (after renal thrombosis)
renal failure
List pentad for TTP
MAHA Thrombocytopaenia (after widespread thrombosis) renal failure Fever Neurological signs
List 2 causes of secondary Nephrotic syndrome ?
- Amyloidosis
- Diabetes
Match the type of RPGN with the immunofluorescence
Type
Type 1 (Goodpastures) Type 2 (Immune complex e.g SLE) Type 3 (Pauci-immune)
Immunoflourescence
Linear
Scanty
Granular
Type 1 (Goodpastures)- Linear Type 2 (Immune complex e.g SLE)- Granular Type 3 (Pauci-immune)- Scanty
List 5 causes of nephritic syndrome ?
Post strep infection IgA nephropathy RPGN Alport's syndrome Benign familial haematuria
What causes chronic interstitial nephritis ?
Long term analgesic consumption e.g NSAIDS or paracetamol
List 3 pre-renal causes of renal failure ?
- Hypovolaemia
- Sepsis
- Renal artery stenosis
List 3 renal causes of renal failure
- ATN
- Acute glomerulonephritis
- Thrombotic microangiopathy
List 3 post-renal causes of renal failure
- stones
- tumours
- prostatic hyperplasia
What GFR defines end stage renal failure (stage 5)
GFR <15
Which autosomal dominant disease is associated with renal failure and berry aneurysms?
APCKD (adult polycystic kidney disease)
List 5 causes of type 2 (immune complex) RPGN ?
SLE Henoch–Schlönein purpura post-streptococcal Alport’s syndrome IgA nephropathy
list clinical features of renal disease
haematuria proteinuria uraemia hypertension oliguria/anuria polyuria oedema colic
list genitourinary malformations involving the kidney
agenesis renal fusion renal dysplasia pelvi-ureteric junction obstruction ureteral duplication posterior urethral valves
features of polycystic kidney disease
presents in adulthood with hypertension, flank pain, haematuria
PKD1, PKD2
berry aneurysm
increased risk of malignancy
causes of renal failure
pre-renal: perfusion failure
renal: acute tubular injury, acute glomerulonephritis, thrombotic microangiopathy
post-renal: obstruction
what is acute tubule-interstitial nephritis
immune injury to tubules and interstitium
also due to infection and drugs (NSAIDs/antibiotics/diuretics/allopurinol)
heavy interstitial inflammatory infiltrate with tubular injury
eosinophils and granulomas visible
what is acute glomerulonephritis
acute inflammation of glomeruli
presents with oliguria with urine casts containing erythrocytes + leukocytes
what is acute crescentic glomerulonephritis
immune complex
anti GBM disease
pauci-immune (anti-neutrophil cytoplasm antibodies)
what is immune complex associated crescentic glomerulonephritis
aetiologies include SLE, IgA nephropathy and post-infectious glomerulonephritis
what is anti-GBM disease
disease caused by antibodies directed against the glomerular basement membrane
c-terminal domain of type IV collagen
antibody can be detected with serology
linear deposition of IgG
what is pauci-immune complex glomerulonephritis
only scanty Ig deposits
ANCA associated
vasculitis elsewhere
what is thrombotic microangiopathy
damage to the endothelium in glomeruli, arterioles, arteries leading to thrombosis
red cells can be damaged by fibrin - MAHA, HUS
diarrhoea associated
non-diarrhoea associated
features of diabetic nephropathy
high glucose levels cause direct injury
starts as microalbuminuria, progresses to proteinuria and nephrotic syndrome
causes of thin basement membranes
hereditary defect in type 4 collagen synthesis
<250nm
haematuria
what is alports syndrome
x linked dominant mutations affecting alpha 5 subunit
progressive, renal failure in middle age
deafness, ocular disease
what is IgA nephropathy
commonest glomerulonephritis
IgA predominant mesangial immune complex deposition
30% develop end-stage kidney failure
what is hypertensive nephropathy
narrowing of arteries and arterioles leading to scarring and ischaemia of glomeruli
shrunken kidneys and granular cortices
