Flashcards in Glomerulonephritis Deck (50):
What is glomerulonephritis?
Inflammatory disorder of the kidney
What percentage of end stage kidney disease is glomerulonephritis responsible for?
Up to 30%
Give some of the many ways glomerulonephritis can present
How is glomerulonephritis classified?
What do we mean by proliferative glomerulonephritis?
Characterised by excessive numbers of cells in glomeruli
These include infiltrating leucocytes
What do we mean by non-proliferative glomerulonephritis?
Glomeruli look normal or have areas of scarring.
They have normal numbers of cells
Tubules and interstitium may be damaged
What are the different types of proliferative glomerular nephritis?
-Post infective nephritis
-Mesangial IgA disease
Focal necrotising (crescentic) nephritis
How do you differentiate diffuse and focal proliferative glomerulonephritis?
Decided on percentage of glomeruli effected
How do you diagnose diffuse proliferative (post infective) glomerulonephritis?
When does diffuse proliferative glomerulonephritis present?
Follows 10-21 days after infection typically of throat or skin
What organism is diffuse proliferative glomerulonephritis most commonly associated with?
Lancefield group A Streptococci
How does acute nephritis present?
(typical of post infective glomerulonephritis)
Fluid retention with oedema
Normal serum albumin
What is the treatment for Post infective glomerulonephritis?
Antibiotics for infection
Loop diuretics such as frusemide for oedema
Vasodilator drugs (e.g. amlodipine) for hypertension
Consider immunosupression for severe disease
If someone presents with what looks like post infective glomerulonephritis when would you do a biopsy?
Test urine and bloods
If it looks like post infective treat as such
If they get better then great
If not biopsy
What is the commonest cause of glomerulonephritis?
How does IgA nephropathy present?
Typically occurs in the young
Presents with macroscopic haematuria
-Very occassionally microscopic
Provoked by inter current infection
Usually not hypertensive
What are the investigative tests like in IgA nephropathy?
Laboratory tests reflect renal function
No characteristic serology
Diagnosed by renal biopsy
How does the renal biopsy in IgA nephropathy appear?
-IgA deposits seen
how does IgA nephropathy present in adults?
Often have an insidious onset
Prognosis of IgA nephropathy is worse in adults - 25% develop renal failure
How do you treat IgA nephropathy?
Non-specific with ACE inhibitors and other hypotensives
What is segmental necrosis?
Such an intense proliferation that the capillaries rupture
(morphological appearance -> not diagnosis)
How does crescentic glomerulonephritis present?
Rapidly progressive glomerulonephritis
May occur in isolation or complicate other diseases e.g. chronic nephritis, vasculitis
What 3 main settings does focal necrotising (crescentic) glomerulonephritis occur?
In the presence of anti-glomerular basement membrae antibodies (Goodpastures syndrome)
Associated with systemic vasculitis (e.g. Wegener's granulomatosis, Microscopic polyangiitis)
As a complication of other types of glomerulonephritis including post-streptococcal glomerulonephrotos and mesangial IgA disease
What is goodpasture's syndrome?
Rare disease caused by autoimmunity to the Glomerular basement membrane
Typically presents as nephritis with or without lung haemorrhage
Diagnosed by demonstrating anti-GBM antibodies in serum and kidney
Treated by plasma exchange and immunosupression
How do you treat crescentic glomerulonephritis?
-Cytotoxic drugs (e.g. cyclophosphamide)
Summerise Proliferative glomerulonephritis
Presents with nephritic syndrome
Blood on dipstix
-None or minimal proteinuria
Can be medical emergency with rapidly progressive glomerulonephritis
What are the different types of non-proliferative glomerulonephritis?
Minimal change disease
CAUSE NEPHROTIC SYNDROME
What is the clinical triad of nephrotic syndrome?
What is the definition of nephrotic syndrome?
A clinical syndrome in which severe oedema is caused by hypoalbuminia due to loss of protein in the urine.
Specifically there is:
-Serum albumin (5G/25hrs
What additional features may be seen in nephrotic syndrome?
Increased risk of infection, especially with pneumococcus
What is the differential diagnosis of nephrotic syndrome?
Congestive Heart Failure
What are the principles of management of nephrotic syndrome?
Treatment of oedema
Prophylaxis against complications
What is the specific management of nephrotic syndrome?
1. Make specific diagnosis, e.g. by renal biopsy
2. Specific treatment when indicated
3. Consider Rx with prednisolone if severe
How do you treat oedema in nephrotic syndrome?
1. Fluid and sodium restriction
2. Normal to high protein diet
3. Rx " loop diuretics" such as frusemide
4. Infusion of albumin with frusemide IV for resistant oedema
What prophylaxis against complications do you use in nephrotic syndrome?
1. Penicillin V (250mg qds) as prophylaxis against infection
2. Consider heparin as prophylaxis against thrombosis
3. Treat hyperlipidaemia in those with persistent nephrotic syndrome
What is minimal change nephrotic syndrome?
Commonest form in children
Sudden onset of oedema - days
Complete loss of proteinuria with steroids
Two thirds of patients relapse
Explain the morphology of minimal change nephrotic syndrome
Only see small change on EM
-Slight change to podocytes
What is the treatment of minimal change nephrotic syndrome?
-60mg/M2 per day with rapid tapering dose after a max of 8 weeks
Initial relapse treated in the same way
Subsequent relapses treated with 8 week course on cyclophosphamide or with continuous cyclosporin
What are the complications of steroid responsive (minimal change) nephrotic syndrome?
Bacterial infection, especially with pneumococcus
Thrombosis, especially in adults
What is the prognosis of steroid responsive (minimal change) nephrotic syndrome?
Resolves eventually and the long term prognosis is good: patients so not develop renal failure
NB in the pre-antibiotic and pre-steroid era, 60% of patients died
What are the clinical features of focal glomerulosclerosis (steroid resistant nephrotic syndrome)?
Severe nephrotic syndrome, especially in men in their 4th decade
Symptoms very disabling
At best an incomplete response to steroids
Progresses to renal failure over 2-3 years
Can recur in renal transplants
What is the morphology of focal segmental sclerosis?
Adhesion seen at edge of bowman's
What is the treatment of focal glomerulosclerosis (steroid resistant nephrotic syndrome)?
Initial trial of steroids
-If responds completely the prognosis as for minimal change; if not poor prognosis
Continue steroids if clinically useful response
Try cyclophosphamide or cyclosporin if steroids fail
Use non-specific drugs to reduce proteinuria
-ACE inhibitors and NNSAIDs (indomethacin)
Give non specific treatment for nephrotic oedema, and prophylaxis against nephrotic complications
What is the commonest cause of nephrotic sundrome in adults?
About half the cases occur in isolation (idiopathic) and about half are associated with another disease
What diseases are associated with membranous nephropathy?
What drugs are associated with membranous nephropathy?
Hypersensitivity to drugs
-e.g. gold and penicillamine
What is the morphology of membranous nephropathy?
IgG deposits with spikes around them
-These spikes are the hallmark and can be seen on EM
What is the management of membranous nephropathy?
Control nephrotic symptoms
Immunosuppression for those with deteriorating renal function
Prednisolone and Chrorambucil alternating monthly for 6 months
Cyclosporin probably ineffective
What is the prognosis of membranous nephropathy?
Resolves spontaneously in 25% over 5-10 years
Prognosis good in treated patients whose proteinuria resolves
About 25% are on dialysis at 10 years
Can recur in renal transplants