Henoch-Schönlein purpura (IgA Vasculitis) - SMALL CELL

Question 1

Henoch-Schonlein purpura (HSP), also known or IgA vasculitis, is the most common form of systemic vasculitis in children. What is the incidence of HSP in the UK?

1 - 3 to 27 per 100,000
2 - 30 to 60 per 100,000
3 - 100 to 200 per 100,000
4 - 200 to 400 per 100,000

Answer 1

1 - 3 to 27 per 100,000
- more common in males

Question 2

Henoch-Schonlein purpura (HSP), also known or IgA vasculitis, is the most common form of systemic vasculitis in children. What age does this typically affect?

1 - 1-5 y/o
2 - 3-15 y/o
3 - 10-20 y/o
4 - 1-20 y/o

Answer 2

2 - 3-15 y/o

Question 3

Although the exact cause of Henoch-Schonlein purpura (HSP) is unknown, there are some triggers that have been identified as causing HSP. Which if the following is NOT one of these?

1 - upper airway infections
2 - gastrointestinal infections
3 - drugs and vaccinations
4 - contraception

Answer 3

4 - contraception

• 50% of cases of HSP have previously had an upper airway infection. Streptococcal infection is common.
• IgA is in high concentration on mucosal surfaces, hence respiratory and GIT involvement

Question 4

Which immunoglobulin is linked with causing Henoch-Schonlein purpura (HSP)?

1 - IgA
2 - IgG
3 - IgM
4 - IgE

Answer 4

1 - IgA

Question 5

Which type of hypersensitivity is Henoch-Schonlein purpura (HSP)?

1 - IgE mediated hypersensitivity (type 1)
2 - IgG mediated cytotoxic hypersensitivity (type 2)
3 - Immune complex mediated hypersensitivity (type 3)
4 - Cell mediated hypersensitivity (type 4)

Answer 5

3 - Immune complex mediated hypersensitivity (type 3)

• IgA antibody immune complexes deposits in vascular walls, stimulating complement activation and neutrophil recruitment
  Immune complex deposition causes vessel necrosis which results in organ-specific symptoms.

Question 6

What makes Henoch-Schonlein purpura (HSP) unique when compared to other small vessel vasculitis?

1 - only affects boys
2 - no ANCA
3 - does not affect adults
4 - all immunoglobulins are raised

Answer 6

2 - no ANCA

Question 7

Which of the following is NOT part of the tetrad in Henoch-Schonlein purpura (HSP)?

1 - Palpable purpura without coagulopathy or thrombocytopenia
2 - Pericarditis with chest pain
3 - Arthritis / arthralgia – transient, migratory, non-deforming mono arthritis
4 - Abdominal Pain
5 - Kidney involvement

Answer 7

2 - Pericarditis with chest pain

purpura = red/purple lumps under the skin

Question 8

Which parts of the body does the palpable purpura without coagulopathy or thrombocytopenia in patients with Henoch-Schonlein purpura (HSP) NOT occur in?

1 - arms
2 - lower legs
3 - bum
4 - thighs

Answer 8

1 - arms

Question 9

Palpable purpura without coagulopathy or thrombocytopenia occurs in 75-100% of patients with Henoch-Schonlein purpura (HSP). What causes the Palpable purpura?

1 - IgA, C3 proteins and neutrophil recruitment collect in small blood vessels
2 - small vessels become inflamed
3 - small blood vessels under the skin leak blood
4 - blood collections lead to red/purple lumps on the skin
5 - all of the above

Answer 9

5 - all of the above

Question 10

Although the diagnosis of Henoch-Schonlein purpura (HSP) is typically clinical, what immunoglobulin is commonly raised in serum?

1 - IgA
2 - IgG
3 - IgM
4 - IgE

Answer 10

1 - IgA

Question 11

Although the diagnosis of Henoch-Schonlein purpura (HSP) is typically clinical, which of the following may we see in the urine?

1 - proteinuria >3-5 and haematuria with casts
2 - minimal proteinuria, microscopic haematuria without casts
3 - proteinuria <3-5 and fatty casts
4 - minimal proteinuria and haematuria with casts

Answer 11

2 - minimal proteinuria, microscopic haematuria without casts

• due to kidney damage
• creatinine may also be high

Question 12

Although the diagnosis of Henoch-Schonlein purpura (HSP) is typically clinical, skin biopsies can also be taken. Which of the following would they typically show?

1 - leucocytoclastic (neutrophil debris) vasculitis in postcapillary venules with IgA deposition
2 - leucocytoclastic (neutrophil debris) vasculitis in postcapillary venules with IgM deposition
3 - lymphcytoclastic (neutrophil debris) vasculitis in postcapillary venules with IgA deposition
4 - lymphcytoclastic (neutrophil debris) vasculitis in postcapillary venules with IgG deposition

Answer 12

1 - leucocytoclastic (neutrophil debris) vasculitis in postcapillary venules with IgA deposition

Question 13

Which inflammatory marker is high in 75% of patients in Henoch-Schonlein purpura (HSP)?

1 - CRP
2 - IL-6
3 - ESR
4 - TNF-a

Answer 13

3 - ESR

Question 14

What is typically the first line treatment in Henoch-Schonlein purpura (HSP)?

1 - steroids
2 - conservative approach
3 - NSAIDs
4 - immunosuppressants

Answer 14

2 - conservative approach
- majority of patients with HSP recover spontaneously

• NSAIDs can help with arthralgia and abdominal pain

Question 15

Steroids are rarely used in Henoch-Schonlein purpura (HSP), except in which case?

1 - older patients
2 - younger patients
3 - severe severity
4 - pregnant women

Answer 15

3 - severe severity

Question 16

Steroids are rarely used in Henoch-Schonlein purpura (HSP), except in severe cases. Which 2 groups of patients should NOT be given NSAIDs?

1 - abdominal bleeds
2 - older patients
3 - glomerulonephritis
4 - history of stroke

Answer 16

1 - abdominal bleeds
3 - glomerulonephritis

Question 17

The prognosis of Henoch-Schonlein purpura (HSP) is typically excellent with the majority of patients symptoms disappearing after how long?

1 - 1 week
2 - 4 weeks
3 - 12 weeks
4 - 24 weeks

Answer 17

2 - 4 weeks