Vasculitis - Henoch Schonlein Purpura

Question 1

What is Henoch-Schonlein Purpura?

Answer 1

An IgA Vasculitis presenting with a purpuric rash that affects the lower limbs and buttocks in kids.

Question 2

Pathophysiology of Henoch Schonlein Purpura.

Answer 2

Leakage of blood from small vessels and Inflammation in affected organs IgA Deposits in blood vessels, affecting the skin, kidneys and GI tract.

Question 3

Aetiology of Henoch Schonlein Purpura.

Answer 3

Triggered by URTI or Gastroenteritis - in kids below 10.

Question 4

What are Purpura?

Answer 4

Palpable Non-Blanching Red-purple lumps under the skin containing blood .

Question 5

What can happen to Purpura in severe cases?

Answer 5

Skin ulceration and necrosis.

Question 6

Clinical Presentation of Henoch Schonlein Purpura (4).

Answer 6

1. Purpura.
2. Joint Pain - knees/ankles.
3. Abdominal Pain - indicating GI involvement.
4. Renal Involvement - HSP IgA Nephritis.

Question 7

How can GI involvement manifest in severe cases? (3)

Answer 7

1. GI Haemorrhage.
2. Intussusception.
3. Bowel Infarction.

Question 8

How can Renal Involvement manifest?

Answer 8

Haematuria and Proteinuria.

Question 9

Important Differentials to Exclude.

Answer 9

1. Meningococcal Septicaemia.
2. Leukaemia.
3. ITP.
4. HUS.

Question 10

Diagnostic Criteria of Henoch Schonlein Pupura (4).

Answer 10

EULAR/PRINTO/PRES Criteria :
PALPABLE PURPURA + 1 of :
1. Diffuse Abdominal Pain.
2. Arthritis/Arthralgia.
3. IgA Deposits.
4. Proteinuria/Haematuria.

Question 11

Management of Henoch Schonlein Purpura (3).

Answer 11

1. Supportive - Analgesia, Rest, Hydration.
2. Steroids - GI/Renal Involvement.
3. Close Monitoring : Urine Dipstick (Renal Involvement) and BP (Hypertension).

Question 12

Prognosis of Henoch Schonlein Purpura (3).

Answer 12

1. Abdominal Pain - few days.
2. Without Renal Involvement - 4-6 weeks.
3. 1/3 recurrence within 6 months.