Henoch Schonlein Purpura

Question 1

What is HSP

Answer 1

acute immunoglobulin A (IgA)–mediated disorder generalized vasculitis involving the small vessels of the skin, the gastrointestinal (GI) tract, the kidneys, the joints, and, rarely, the lungs and the central nervous system (CNS).

Question 2

Pathophysiology and cause

Answer 2

IgA aggregates or IgA complexes with complement deposited in target organs, resulting in elaboration of inflammatory mediators

The cause is unknown but there may be a recent history of an upper respiratory tract infection.

Question 3

Classical presentation

Answer 3

HSP typically presents with the triad of

purpuric rash on the extensor surfaces of limbs (mainly lower) and buttocks,
joint pain/swelling and
abdominal pain.

Question 4

Assessment

Answer 4

Rash
Joint pain
Abdominal pain
Renal disease
SC edema
Pulmonary and CNS complications

Question 5

If child unwell or rash atypical, what to consider

Answer 5

Consider meningicocemia, thrombocytopenia or other rare vasculitic disease

Question 6

When does the joint pain usually resolve

Answer 6

Usually in 24-48 hours

Question 7

When does the uncomplicated abdominal pain usually resolve

Answer 7

Usually 72 hours

Question 8

What are possible GIT complications (5)

Answer 8

Hematemesis
Pancreatitis
Intussusception
Perforation
Bloody stools

Question 9

How common is hematuria, how often persistent/recurrent

Answer 9

Hematuria in 90%, 5% persistent/recurrent

Question 10

Less common renal complications

Answer 10

Proteinuria
Nephrotic syndrome
Renal failure
Hypertension
Renal insufficiency

Question 11

Rare complications

Answer 11

Pulmonary and CNS complications

Question 12

Investigations

Answer 12

Urinalysis
FBE, PLT count
UEC
Blood culture
Consider coags? ->rule out differentials

Question 13

Management

Answer 13

Get senior help due to rash
Document BP
Consider surgical consult if abdominal features are prominent (?testicular torsion)
Consider steroids in some cases
Rest and analgesia

Question 14

When to consider steroids

Answer 14

More than mild joint or abdominal pain. Consider prednisolone 1mg/kg while symptoms persist.

Question 15

Indications for admission (3)

Answer 15

Abdominal complication->arrange early surgical consultatioin
Renal complication->nephritis, nephrotic syndrome
Symptomatic management for severe joint/abdominal/edema

Question 16

Follow up

Answer 16

See GP
Blood pressure measurement
Urinalysis

Question 17

When does the rash go away, when is it made worse

Answer 17

Last clinical manifestation to remit

Usually worse when + activity

Question 18

Advice to parents

Answer 18

• > No specific treatment
• > Rest and analgesia
• > HSP usually gets better on its own without causing lasting problems.
• > About half of people who have had HSP once will get it again.
• > A few people will have kidney damage because of HSP. This may occur in the first week or so of illness, but there may be a delay of weeks or months before it appears.
• > Your child’s doctor will want to test urine samples and blood pressure several times after the HSP goes away to check for kidney problems. These checks should go on for at least six months and some doctors recommend a blood pressure and urine check every year for life.

Question 19

When to return

Answer 19

Return to your doctor or the hospital if your child has increasing stomach pains or swelling, blood in the stools or urine, or if you are worried for any other reason.