Henoch-Schonlein Purpura (HSP)

Question 1

What is HSP?

Answer 1

An IgA vasculitis

Question 2

What is its incidence/prevalence?

Answer 2

75% of cases in children < 10 years

UK incidence = 20 out of every 100,000 children (aged under 17 years)

Question 3

Does it affect boys or girls more?

Answer 3

Uncertain as there is no general consensus

Question 4

What are some of the aetiologies of HSP?

Answer 4

Gastroenteritis

URTI

Question 5

What is the “classic triad” of HSP?

Answer 5

Purpura

Abdominal pain

Arthritis

Question 6

What is the pathogenesis of HSP?

Answer 6

Inflammation in organs due to IgA deposits in blood vessels

Affects kidneys, skin, and GI tract

Question 7

What are the typical symptoms of HSP?

Answer 7

Sudden

Red spotting of the skin

Headaches

Loss of appetite and/or fever

Diffuse erythema

Cramping abdominal pain

Blood in stool/urine

Joint pain and swelling

Question 8

What are the signs?
HINT: remeber the “classic triad”

Answer 8

Palpable purpura (100% of cases)

Joint pain (75% of cases) - arthritis/arthralgia mainly in the knees

Abdominal pain (50-75% of cases) - in severe cases there can be intussusception, haemorrhage, bowel infarction

Kidney impairment (50% of cases) - IgA/HSP nephritis. Causes proteinuria/haematuria

2+ protein = nephrotic syndrome (causes a degree of oedema)

Question 9

What other serious pathologies that causes non-blanching rashes do you need to exclude?

Answer 9

Meningococcal meningitis

Leukaemia

Idiopathic thrombocytopenic purpura

Haemolytic uraemic syndrome

Question 10

What is haemolytic uraemic syndrome?

Answer 10

A condition that can occur when the small blood vessels in your kidneys become damaged and inflamed. This damage can cause clots to form in the vessels. The clots clog the filtering system in the kidneys and lead to kidney failure, which could be life-threatening

characterised by the triad of microangiopathic haemolytic anaemia, thrombocytopenia, and acute kidney injury.

Most cases of HUS occur in children and are diarrhoea-associated (D+ HUS). Diarrhoea-associated HUS is usually caused by Shiga toxin-producing Escherichia coli.

Rarely other organisms, such as Shigella and Streptococcus pneumoniae, are implicated.

Acute kidney injury necessitating dialysis develops in approximately half of children with diarrhoea-associated HUS.

Question 11

Sources

Answer 11

https://en.wikipedia.org/wiki/Henoch%E2%80%93Sch%C3%B6nlein_purpura

https://bestpractice.bmj.com/topics/en-gb/470

Question 12

What investigations would you do for a child with suspected HSP and why?

Answer 12

FBC + film ¬– thrombocytopenia, sepsis, leukaemia

Renal profile

Albumin – nephrotic syndrome

CRP – sepsis

Blood cultures – sepsis

Urine dipstick – proteinuria

Urine protein:creatinine ratio – proteinuria

Blood pressure – hypertension

Question 13

What is the diagnostic criteria for HSP according to Eular/Printo/Pres 2010 criteria?

Answer 13

Palpable purpura

And at least one of the following:
- Diffuse abdominal pain
- Arthritis or arthralgia
- IgA deposits on histology
- Proteinuria/haematuria

Question 14

What is the Mx of HSP?

Answer 14

Supportive – analgesia, rest and hydration

Use of steroids debatable – considered in patients with severe GI pain or renal involvement

Monitoring
- urine dipstick – renal impairment
- blood pressure – HTN

Question 15

What is the prognosis of HSP?

Answer 15

Abdominal pain settles in a few days

Without kidney involvement – full recovery in 4-6 weeks

1/3 have recurrence within 6 months

Very small proportion develop end stage renal failure