HSP

Question 1

What is HSP?

Answer 1

Hence Schonlein Purpura

Is an IgA mediated autoimmune vasculitis which occurs in childhood.

It is characterised by:

• skin purpura
• arthritis
• nephritis (in 40%)

May also have:

• abdominal pain
• gastrointestinal bleeding
• orchitis

Question 2

What is the proposed aetiology of HSP?

Answer 2

It is thought to be a post infective automimmune reaction to:

• Group A strep
• Mycoplasma
• Epstein Barr Virus

Question 3

Which condition is associated with HSP?

Answer 3

Intussusception (occurs in 2-3% of patients)

Question 4

How should suspected HSP be investigated?

Answer 4

Bedside:
Urine dip (proteinuria)

Bloods:*
FBC if worried about infection
U/e’s
ESR (will be raised)

Abdominal US (if symptoms)
-Rule out intussusception

*Bloods may not be necessary as it is usually a clinical diagnosis

Question 5

How should HSP be managed?

Answer 5

Patient education.

Self limiting disorder usually full recovery in a few months.

NSAIDs for joint pain but use in caution in those with renal involvement.

Steroids are sometimes used for renal involvement but efficacy is disputed, should be admitted for renal involvement.

Monitoring for renal involvement is needed with urine dips and BP.

If no proteinuria monitor on days 7 and 14 and at 1, 3, 6 and 12 months.

If proteinuria monthly between 1-6months.

Question 6

What are the long term complications associated with HSP?

Answer 6

Renal involvement is in approximately 40% of children.

Serious renal involvement is present i approximately 10% of children.

Less than 1% of children progress to end stage kidney disease.

Question 7

Which disease shares the same histopathological appearance as HSP nephritis?

Answer 7

IgA nephropathy.

Thought it could be a manifestation of the same disease.