Case 23 - Glomerulonephritis and PKD Flashcards
What are glomerular diseases?
Can be due to a variety of reasons
Diseases in which the function of the glomerulus has been reduced
As what syndromes can glomerular disease present?
Isolated haematuria/proteinuria/both RPGN Acute nephritic syndrome Nephrotic syndrome CKD - and eventually ESRD
Investigations for glomerular diseases?
Urine dip - for protein and blood (dysmorphic on film)
Quantigfy protein with ACR
FBC U and E Bone profile Clotting HbA1c CRP Immunology Lipid profile LFTs
USS kidney
Kidney biopsy
What is nephritic syndrome?
Combination of:
- Haematuria
- HTN
- Proteinuria
- Oligouria
Can be caused by a variety of diseases
What diseases can cause nephritic syndrome?
IgA nephropathy
PSGN
RPGN
What is IgA nephropathy?
Classically seen 1-3 days after a throat infection (synpharyngitic GN)
The antibodies made to combat the throat infection start to attack the kidney
IgA deposited in the kidney
In biopsy will see clumps of IgA in the mesangium
May present with Henoch Schonlein Purpura which is systemic vasculitis
What is PSGN?
Post-streptococcal GN
Presents 1-2 weeks after a throat infection
Either the antibodies get lodged in GBM, or the antigens do
Inflammatory cells cause damage to the glomerulus
What is RPGN?
Rapidly progressive GN
Can present for a number of reasons
Sudden deterioration in kidney function due to GN
Crescentic GN
What are the types of vasculitis?
Categorised based on vessel size - large, medium and small
Smalls vessels : ANCA positive -Wegeners granulomatosis -Microscopic polyangitis -Churg Strauss syndrome -Pauci-immune GN
Induce remission with cyclophosphamide and steroids and maintain with azothioprine and steroids
How can vasculitis present?
Nasal crusting RPGN Epistaxis Rashes e.g. Henoch schonlein purpura Joint pain Myalgia Haemoptysis
What is nephrotic syndrome?
Combination of:
- Proteinuria >3.5g/day
- Oedema
- Hypercholesterolaemia
- Hypoalbuminaemia
What are the types of nephrotic syndrome on biopsy?
Minimal change
Focal segmental glomerular sclerosis
Membranous GN
Membranoproliferative GN
Causes of nephrotic syndrome
Primary is idiopathic
Secondary - can be a variety of conditions that can cause nephrotic syndrome: Infections Drugs Tumours Autoimmune Heavy metals
Treatment of nephrotic syndrome
Salt restriction
Fluid restriction
Diuretics for the oedema
ACE-i/ARB
Biopsy to check for histology which may help direct serology for the cause
Treat the cause
Prophylaxis of renal vein thrombosis due to urinary loss of AT-III
Causes of RPGN?
Type 1 -anti-GBM mediated
Ab to GBM are formed and attack the GBM
Can present as part of Good-Pastures syndrome - anti-GBM + Haemoptysis + haematuria
Type 2 - immune complex mediated
Antibodies and antigens clog up the glomerulus and cause damage
e.g. IgA nephropathy, PSGN
Pauci immune - when there is no immune complex deposition
This is triggered by ANCA instead
Could be Wegeners or microscopic polyangitis etc.
What are some genetic cystic kidney diseases?
Autosomal domininat:
ADPKD
Von Hippel Lindau syndrome
Autosomal recessive:
ARPKD
X-linked:
Chromosomal disorders
What are some non-genetic cystic kidney diseases?
Developmental:
Medullary sponge kidney
Multicystic dysplastic kidney
Acquired:
Acquired cystic kidney disease - dialysis
Simple cysts
Hypokalaemia-related cysts
What is von Hippel Lindau disease?
Autosomal dominant cystic kidney disease
Mutated gene has a role as a TSG
Increased risk of renal Ca
Can treat with surgery so should monitor with USS and CT
What is PKD?
Makes up 10% of dialysis population
Most common cystic kidney disease
Adult PKD has triad of liver cysts, renal cysts and berry aneurysms
What is ADPKD?
Less severe form
More common
Mutations in PKD1 and PKD2 genes
What is ARPKD?
More severe form
Diagnosed in childhood
Liver fibrosis essential to diagnose
Imaging for PKD?
USS - to assess cyst size and number on each kidney
CT - may be able to see liver cysts too
MSU - proteinuria, the higher the risk this is, the higher the risk of progressing to CKD
Management for PKD
Fluids and monitor CKD Control HTN from childhood Family screening CV risk control Treat CKD when it comes Tolvaptan can slow the progression - but need to drink high levels of fluids Nephrectomy for severe pain
Treatment for RPGN
Anti-coagulants to reduce fibrin deposition
Plasmaphoresis and immunosuppressants in combination
RRT