Glomerulonephritis

Question 1

Glomerular disease/Glomerulonephritis info:

Answer 1

Glomerulonephritis is a broad term that refers to a group of parenchymal kidney diseases that all result in inflammation of the glomeruli and nephrons.
It can cause:
1) Damage to the filtration mechanism resulting in haematuria and proteinuria.
2) Damage to the glomerulus which restricts blood flow leading to compensatory hypertension.
3) Loss of usual filtration capacity leading to AKI

Question 2

PPx of acute glomerulonephritis/Nephritic syndrome?

Answer 2

Often caused by immune response triggered by an infection of other diseases. Increased glomerular cellularity restricts glomerular blood flow and therefore filtration is decreased.
Characterised by:
1) Haematuria (visible/non-visible - microscopy)
2) Proteinuria
3) Decreased urine output (oliguria) and volume overload
4) Hypertension and Oedema (leg, sacral, periorbital)

Question 3

Post-streptococcal glomerulonephritis (PSGN) aetiology:

Answer 3

PSGN - e.g. Streptococcus pyogenes

• Presents 7-21 days after a streptococcal infection (pharyngitis, impetigo, cellulitis) - usually Group A beta-haemolytic streptococcus.
• Bacterial antigen gets trapped in glomerulus leading to a diffuse proliferative glomerulonephritis.

Question 4

PSGN Dx and Tx?

Answer 4

Dx: 1) Culture of organism
2) Raised ASO/anti-DNAse B titres

Tx: 1) Antibiotics - amoxicillin
2) Supportive

Question 5

Henoch Schonlein Purpura (HSP) Ex and PPx?

Answer 5

Ex: Occurs between 3-10yrs, more common in boys, peaks during winter and often preceded by URTI.

PPx: IgA forms complexes that activate complement and are deposited in affected rogans leading to an inflammatory response with vasculitis.

Question 6

HSP presentation?

Answer 6

Similar presentation to IgA neuropathy (Berger’s syndrome) with a rash.

1) Characteristic skin rash on buttocks, extensors, legs and arms (trunk spared) becomes maculopapular and purpuric.
2) MSK - arthralgia and periarticular oedema
3) Kidney - glomerulonephritis - haematuria and proteinuria (If proteinuria is severe then nephrotic syndrome can occur)
4) Abdominal pain - haematemesis, malaena, intussusception

Question 7

HSP Diagnosis?

Answer 7

1) Clinical
2) Urinalysis - microscopic/macroscopic haematuria and proteinuria
3) Renal biopsy - not usually recommended but will show IgA deposition

Question 8

HSP Treatment?

Answer 8

1) Analgesis: Paracetamol/Ibuprofen
2) Oral corticosteroids: Prednisolone
3) Severe nephritis - immunosuppression: Cyclophosphamide
4) Very severe: Plasmapheresis
Self-limiting condition especially when no renal environment
All children with renal involvement are followed for a year to detect those with persisting urinary abnormalities who require long-term follow-up.

Question 9

IgA neuropathy/Berger’s syndrome info:

Answer 9

• MOST COMMON CAUSE of glomerulonephritis worldwide
• Typically presents with macroscopic haematuria following a URTI.
• IgA deposition in mesangium (provide structural support to glomerulus) of kidney resulting in kidney inflammation = glomerulonephritis

Treatment: 1) Supportive 2) Oral Prednisolone

Question 10

Alport syndrome Ex:

Answer 10

• Familial nephritis
• X-linked recessive disorder - defect in gene coding for collagen resulting in abnormal glomerular basement membrane
• Males affected more severely than females (rarely get renal involvement)

Question 11

Alport syndrome presentation?

Answer 11

1) Gross haematuria
2) Progresses to end-stage renal failure by early adult life
3) Associated with nerve deafness and ocular defects
4) Mother may have haematuria

Question 12

Alport syndrome diagnosis and treatment?

Answer 12

Dx: Renal biopsy shows longitudinal splitting of glomerular basement membrane - ‘basket-weave’ appearance

Tx: Renal disease monitoring and dialysis

Question 13

Systemic Lupus erythmatosus (SLE) Ex and PPx?

Answer 13

PPx: Autoimmune disease characterised by multiple autoantibodies.
Ex: Presents mainly in adolescent GIRLS and young women. More common in Asians and afro-carribeans than Caucasian.

Question 14

SLE presentation, Dx, Tx?

Answer 14

Sx: Rash, arthralgia, haematuria, proteinuria

Dx:

1) ANA positive and double stranded DNA positive.
2) Low complement C3 and C4
3) Renal biopsy - immune deposits - intensity of immunosuppression required will depend on degree of renal impairment assessed by biopsy.

Tx:

1) Immunosuppression (Cyclophosphomide)
2) Oral Prednisolone