Glomerulonephritis (2) Flashcards

1
Q

Supportive treatment of glomerulonephritis

A

Supportive:

  • lifestyle modification
  • salt and water restriction
  • control BP
  • nutrition -> not to give too much potassium/phosphate (as it comes out mostly in urine, so if oliguria then potassium build up)
  • prevention of thromboembolism -> risk of clots increase when low plasma protein (as anticoagulants are proteins - lost with proteinuria)
  • treat infections
  • electrolytes and acid balance
  • renal replacement therapy
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Definitive treatment of glomerulonephritis

A

Definitive treatment - to treat the underlying cause of glomerulonephritis

  • treat the underlying cause -> infection, cancer etc.
  • immunosuppression -> steroids, alkylating agents etc.
  • plasmapheresis -> plasma and abnormal antibodies in it are replaces
  • renal transplant -> if all the other treatment fails and kidney cannot be saved
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q
A

Answer:

C. IgA vasculitis (HSP)

This is because it happens 2 days after the sore throat (post-strep would happen 2-3 weeks post infection)

Henoch Schonlein Purpura: presents with abdominal pain, and blood in the urine, rash

Other investigations: biopsy to confirm diagnosis and how bad it has become

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Henoch-Schonlein Purpura

  • another name
  • how common is it
  • population profile
A

Henoch- Schonlein Purpura

  • aka IgA nephropathy
  • the commonest cause of primary glomerulonephritis
  • 20-30 years old / children following an infection
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Henoch Schonlein Purpura

  • what happens (pathophysiology)
A

Henoch-Schonlein purpura (HSP)

  • IgA mediated small vessel vasculitis
  • abnormal IgA deposition in the glomerulus
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Features/ presentation of Henoch-Schonlein Purpura

A

Features

  • palpable purpuric rash (with localized oedema) over buttocks and extensor surfaces of arms and legs
  • abdominal pain
  • polyarthritis
  • features of IgA nephropathy may occur e.g. haematuria, renal failure, proteinuria
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Management and prognosis of Henoch-Scholein** purpura

A

Treatment

  • analgesia for arthralgia
  • treatment of nephropathy is generally supportive.
  • inconsistent evidence for the use of steroids and immunosuppressants

Prognosis

  • usually excellent, HSP is a self-limiting condition, especially in children without renal involvement
  • around 1/3rd of patients have a relapse
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q
A

Answer

1. Diagnosis: Nephrotic syndrome -> minimal change glomerulonephritis

*we do not biopsy children - 90% will have minimal change

* treat with the steroids

  1. Possible complications are: pulmonary embolism, cellulitis, cerebral sinus thrombosis, peritonitis

* cellulitis and peritonitis (as immunoglobulins are lost as proteins in the urine -> prone to infection)

*not pulmonary oedema -> it depends on hydrostatic pressure; here it is lost (pt has low BP, less likely to get pulmonary oedema)-> so cannot force fluid into the lungs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What’s the commonest cause of nephrotic syndrome in the child?

A

Minimal change glomerulonephritis

  • the underlying cause of glomerulonephritis in a child - unless proven otherwise
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What’s the prevalence of minimal change disease?

A

Minimal change disease

  • nearly always presents as nephrotic syndrome
  • 75% of cases in children
  • 25% in adults.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Possible causes of Minimal Change glomerulonephritis

A

The majority of cases are idiopathic, but in around 10-20% a cause is found:

  • drugs: NSAIDs, rifampicin
  • Hodgkin’s lymphoma, thymoma
  • infectious mononucleosis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Pathophysiology of minimal change glomerulonephritis

A

Pathophysiology of minimal change glomerulonephritis

It is immuno- mediated but we do not know what in immune system causes it (according to the lecture)

PassMedicine notes:

  • T-cell and cytokine mediated damage to the glomerular basement membrane → polyanion loss
  • the resultant reduction of electrostatic charge → increased glomerular permeability to serum albumin
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Features of minimal change glomerulonephritis

A

Features

  • nephrotic syndrome
  • normotension - hypertension is rare
  • highly selective proteinuria*
  • renal biopsy: electron microscopy shows fusion of podocytes

* only albumin and transferrin will leak through (due to their size)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Treatment of minimal change glomerulonephritis

A

Management

  • majority of cases (80%) are steroid responsive
  • cyclophosphamide is the next step for steroid resistant cases

Prognosis is overall good, although relapse is common. Roughly:

  • 1/3 have just one episode
  • 1/3 have infrequent relapses
  • 1/3 have frequent relapses which stop before adulthood
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q
A

Answer:

B. Lupus nephritis -> clue is in the Hx (e.g. Reynolds), discoid lupus - hair loss, bloods are low

*lupus affects lots of body system: joint pain, rashes, blood problems, psychiatric problems

Other investigation: look for ANA (90% of pt with lupus but not really lupus specific); look for dsDNA -> more positive for lupus

*also do kidney biopsy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Treatment for Lupus nephritis

A

Immunosuppression

  • steroids
  • cyclophosphamide
  • Rituximab
  • hydroxychloroquine)
17
Q
A

Answer

  • Diagnosis: Acute Post Strep glomerulonephritis (as 2 weeks after infections)
  • Tests: suggestive of post strep
  • glomerulonephritis -> serum complement & anti Dnase (anti - streptolysin anti-body -> as compotents of strep wall)

*some antigens in kidney look at streptococcus wall -> molecular mimicry

18
Q

What’s the most likely diagnosis?

a) post strep glomerulonephritis
b) Lupus nephritis
c) IgA nephropathy
d) microscopic polyangitis
e) Good Pasture’s disease

A

Answer:

Good Pasture’s -> because it is positive for an anti-glomerular basement membrane antibody

X-ray: pulmonary haemorrhage

19
Q

Goodpasture’s disease

  • what is this (2 main features)
A

Pulmonary haemorrhage + rapidly progressive glomerulonephritis

20
Q

What antibody causes Goodpasture’s syndrome? (pathophysiology)

A

Good Pasture’s syndrome

  • caused by anti-glomerular basement membrane (anti-GBM) antibodies against type IV collagen
  • this breaks basement membrane -> blood get into it and irritates it

*a lot of ‘crescents’ in the Bowman’s capsule forms -> nephron is gone

21
Q

Goodpasture’s syndrome

  • gender prevalence
  • age distribution
  • genetic association
A

Goodpasture’s syndrome

  • more common in men (sex ratio 2:1)
  • (peaks in 20-30 and 60-70 age bracket)
  • associated with HLA DR2
22
Q

Investigations for Goodpasture syndrome

A

Investigations

  • renal biopsy: linear IgG deposits along basement membrane
  • raised transfer factor secondary to pulmonary haemorrhages
23
Q

Management of Goodpasture’s syndrome

A

Management

  • plasma exchange (plasmapheresis)
  • steroids
  • cyclophosphamide -> alkylating agent -> immunosuppressant
24
Q

What ocular abnormality is it connected with?

A

Diagnosis: Alport’s syndrome

Ocular abnormality: anterior lenticonus (protrusion of the lens surface into anterior chamber)

25
Q

Alport’s syndrome

  • gene
  • defects (pathophysiology)
A

Alport’s syndrome

  • 85% inherited in an X-linked dominant pattern
  • 15% autosomal recessive
  • defect in the gene coding for type IV collagen

Result: abnormal glomerular-basement membrane (GBM)

26
Q

What features may be seen in Alport’s syndrome?

A

Alport’s syndrome usually presents in childhood. The following features may be seen:

  • microscopic haematuria
  • progressive renal failure
  • bilateral sensorineural deafness
  • lenticonus: protrusion of the lens surface into the anterior chamber
  • retinitis pigmentosa
  • renal biopsy: splitting of lamina densa seen on electron microscopy
27
Q

What may happen with renal transplant in a patient with Alport’s syndrome?

A

Alport’s patient -> may fail renal transplant

  • this may be caused by the presence of anti-GBM antibodies leading to a Goodpasture’s syndrome like picture