Renal: Glomerulonephritis

Question 1

What is the difference between nephrotic and nephritis syndrome?

Answer 1

Nephritic: Haematuria, hypertension

Nephrotic: Proteinuria and oedema

Question 2

What is minimal change disease?

Answer 2

Damage to the glomerular basement membrane causing nephrotic syndrome (leakage of intermediate sized proteins such as albumin and transferrin. It is most commonly seen in children.

Question 3

What is the treatment for minimal change disease?

Answer 3

Steroids (80% are responsive)

Cyclophosphamide

Question 4

If you suspect someone has minimal change disease do you need to do a renal biopsy?

Answer 4

No. Only if the response to steroids is poor.

Question 5

What is the difference between IgA glomerolonephritis and post streptoccal glomerulonephritis?

Answer 5

Post-streptococcal glomerulonephritis occurs 1-2 weeks after an infection and presents with a nephritic syndrome picture.

IgA glomerulonephritis is similar in presentation but develops 1-2 days after a viral infection

Question 6

What is henoch - schonlein purpura?

Answer 6

An IgA mediated small vessel vasculitis which is usually seen in children following an infection. Presents with a purpuric rash over the buttocks, arms and legs, abdominal pain, joint pain and features of IgA nephropathy such as haematuria

Question 7

What is the treatment for henoch schonlein purpura?

Answer 7

Analgesia for athralgia
Supportive.
Self limiting condition with a good prognosis.

Question 8

What are the four main categories of disease that affect the glomerulus?

Answer 8

Diabetic nephropathy
Glomerulonephritis
Amyloid/Light chain nephropathy
Transplant glomerulopathy

Question 9

What is glomerulonephritis?

Answer 9

An immune mediated set of disease of the kidneys affecting the glomeruli (this then results in secondary interstitial damage. The presentation depends on the specific disease entity (eg can present as nephrotic or nephritic syndrome. They are often broadly based into either proliferative (damage to endothelial or mesangial cells) or non proliferative (damage to podocytes).

Question 10

What is meant by nephrotic syndrome?

Answer 10

Oedema, proteinuria and decreased protein levels in the blood as well as increased fat in the blood. This is due to imflammation in the podocytes which increases the permeability to proteins, causing excessive secretion of protein in the urine.

Question 11

What is meant by nephritic syndrome?

Answer 11

Blood in the urine, oliguria and hypertension. Inflammation results in destruction of the epithelial cells and the damage to menagnial cells results in decreased kidney blood flow which leads to activation of the RAAS system and hypertension.

Question 12

List some causes of non proliferative glomerulonephritis which result in the nephrotic syndrome?

Answer 12

1. Minimal change disease
2. Focal segmental glomeurolsclerosis
3. Membranous glomerulonephritis
4. Basement membrane disease

Question 13

What is minimal change disease?

Answer 13

Nephrotic syndrome (commonly seen in children)  due to cytokines damaging the foot processes of the podocytes, causing them to flatten and allow more protein through (selective) 
Mostly idiopathic but can be associated with hodgekins lymphoma.  This does not cause renal failure.

Question 14

What are the 4 hallmarks of nephrotic syndrome?

Answer 14

1. Proteinuria (more than 3.5g/day)
2. Hypoalbuminemia
3. Oedema
4. Hyperlipidaemia

Question 15

What is the diagnosis test for minimal change disease?

Answer 15

Electrron microscopy of kidney biopsy (cannot see the effacement on light microscopy alone)

Question 16

How do you treat minimal change disease

Answer 16

Corticosteroids (children respond better than adults)

Question 17

What is focal segmental glomerulosclerosis

Answer 17

Sclerosis/scarring of segments of golmerules. This presents as a nephrotic syndrome.
This can be primary FSGS (idiopathic) or secondary and associated associated with HIV and heroin use and sickle cell disease.

Question 18

How is focal segmental glomerulosclerosis treated?

Answer 18

Steroids (not that good a response) can lead to CKD.

Question 19

What are some general treatments of glomerulonephritis?

Answer 19

1. Blood pressure control (usually with an ACEi)
2. Immunosupression
3. Plasmaphoresis

Question 20

What is the general treatment of nephrotic syndrome?

Answer 20

Fluid restriction 
Salt restriction 
Diuretics 
ACEi
IV albumin if volume deplete
Immunosupression

Question 21

What is membranous glomerulonephritis?

Answer 21

Inflammation of the basement membrane by immune complexes. This form as a result on autoantibodies to the GBM.. They then get stuck between the podocytes and GBM and activate the complement system. These then damage the podocytes and the GBM and mesangial cells.
The GBM appears thickened on histology. On immunofluoresence you see IgG autoantibodies.
Most often this is idiopathic but can be secondary to infection (hep B, parasites) , malignancy, drugs (gold/penicillamine) or autoimmune disease (SLE).
Risk of renal vein thrombosis.

Question 22

What is the treatment of membranous glomerulonephritis?

Answer 22

Steroids +/- cyclophosphamide

Question 23

What is IgA nephropathy(Bergers disease)?

Answer 23

Episodic macroscopic haematuria after a respiratory/GI infection.
Due to IgA depositing in the mesangial cells. Assoictaed with henoch schonlein purpura. 25% progress to end stage renal failure.

Question 24

What is rapidly progressive glomerulonephritis?

Answer 24

A treatable cause of renal failure which causes a rapid deterioration in renal function over days /weeks. It is associated with glomerular crescents on biopsy. This is broadly divided into ANCA +ve and ANCA -ve

Question 25

What are ANCA +ve causes of rapidly progressive glomerulonephritis?

Answer 25

• Vasculitis

- Wegners grunulomatosis - Microscopic polyangitis

Question 26

What are ANCA -ve causes of rapidly progressive glomerulonephritis?

Answer 26

Goodpastures disease
Anti GBM
HSP
SLE

Question 27

What is goodpastures syndrome (Anti GBM disease)?

Answer 27

Development of autoantibodies to type 4 collagen and it is characterised by haemoaturia and hameoptyisu.. If plasma exchange and corticosteroids are given early the prognosis is much better.

Question 28

How do you treat a rapidly progressive glomerulonephritis?

Answer 28

• Immunosupression with steroids and cyclophosphamid

Plasmapheresis