Glomerulonephritis 2

Question 1

Name the 3 causes of nephrotic syndrome

Name 2 secondary causes

Answer 1

1) Minimal change disease
2) Focal & Segmental GN
3) Membranous nephropathy

4) Diabetic nephropathy
5) Amyloidosis: myeloma

Question 2

Underlying pathology of nephrotic syndrome [2]

Answer 2

Non-proliferative structural changes lead to basement membrane thickening and glomerulosclerosis. [1]
Podocytes normally wrap around glomerular capillaries maintaining filtration barrier and prevent large molecules from entering the urine but effacement of the foot processes and podocyte loss [1] leaks protein loss

Question 3

What characterises Minimal change disease? [1]

Who is most affected by minimal change disease?

Answer 3

Sudden onset oedema

Its the most common form in children

Question 4

Minimal change disease
Appearance on renal biopsy [1]
Management: 1st line then 2nd line [4]

Answer 4

Secondary to NSAIDs, Hodgkin’s lymphoma

Electron microscopy: effacement of podocyte foot processes

If they relapse use steroids again, concurrent relapses use other treatments like cyclophosphamide, tacrolimus or rituximab

Question 5

Focal Segmental glomerulosclerosis
Causes [5]
Association with IgA nephropathy [2]
Management [4]

Answer 5

Alport's syndrome
VUR
Sickle cell disease
HIV
Heroin

IgA nephropathy tends to be nephritic but can be complicated by focal segmental glomerulosclerosis causing nephrotic syndrome

Trial steroids (30% respond)
Cyclosporin, cyclophosphamide & Rituximab

Question 6

Whats the commenest cause of nephrotic syndrome? [2]

Answer 6

Membranous nephropathy in adults or Minimal change disease in kids

Question 7

Investigations for nephrotic syndrome
Urine [4]
Bloods [6]
Immunology [3]
Renal biopsy [1]

Answer 7

Urine:
o Urinalysis: protein
o Urine microscopy
o 24h urine collection: for protein and creatinine
o Urinary Bence Jones proteins: excl. myeloma

Bloods: U&E and creatinine, lipids (hyperlipidaemia), clotting (hypercoagulability), serum protein electrophoresis (excl. myeloma), glucose (excl. DM), TFTs (low total T4)

Immunology: anti-dsDNA and ANA (SLE), low immunoglobulins (protein loss in urine), low C3 and C4

Renal biopsy: more difficult in nephrotic syndrome due to gross oedema and hypercoagulability

Question 8

What causes Membranous nephropathy? [4]

Answer 8

Mostly idiopathic

Secondary to Malignancy, SLE, rheumatoid arthritis or drugs (NSAIDs/gold/penicilllamine)

Question 9

Membranous nephropathy
Describe what immunology studies will show [2]
Describe what renal biopsy will show [2]

Answer 9

Primary membranous nephropathy shows serological markers (PLA2R antibodies & THSD7A) [2]

Renal biopsy shows diffusely thickened glomerular basement membrane on light microscopy [1] with IgG and C3 sub epithelial deposits on IF [1] , subepithelial spikes on silver stain [1]

Question 10

How do you treat membranous nephropathy? [3]

Answer 10

Just use general anti-nephrotic syndrome treatments for the 1st 6 months as 1/3rd of cases resolve on their own.

Porcelli regimen (alternates IV steroids with CHLORAMBUCIL and CYCLOPHOSPHAMIDE)

Rituximab if anti-PLA2 positive

Question 11

What general measures do you use to treat nephrotic syndrome? [6]

Answer 11

Na/fluid restriction + loop diuretic for oedema (IV furosemide)
Reduce proteinuria: ACEi/ARB

Statin for hyperlipidaemia
Pneumococcal vaccine for infection risk
Heparin/warfarin for thrombosis risk
RAAS blockage for hypertension