IgA vasculitis / henoch schonlein purpura

Question 1

What is the typical onset time for IgA vasculitis after an upper respiratory tract infection (URTI)?

Answer 1

1-2 weeks

20-50% show ASOT, but no causal role of GAS has been shown

Question 2

What type of immune complexes are involved in the pathogenesis of IgA vasculitis?

Answer 2

IgA immune complexes in vessel walls

Question 3

What percentage of adults and children with IgA vasculitis experience fever?

Answer 3

20% adults, 40% children

Question 4

What are the initial lesions seen in IgA vasculitis?

Answer 4

Urticarial papules or erythematous macules

Question 5

What is the progression of cutaneous lesions in IgA vasculitis?

Answer 5

Progress to palpable purpura

Question 6

How long does it typically take for individual lesions to resolve in IgA vasculitis?

Answer 6

10-14 days

Question 7

List the most common extrcutaneous signs of IgA vasculitis.

Answer 7

• Arthritis (75%)
• Gastrointestinal (50-75%)
• Renal (40-50%)

Question 8

What is the most common renal manifestation in IgA vasculitis?

Answer 8

Microscopic haematuria

Question 9

What are some risk factors for renal disease in pediatric patients with IgA vasculitis?

Answer 9

• Age >8 years
• Abdominal pain
• Recurrent disease

Question 10

What are poor prognostic factors for renal disease in IgA vasculitis?

Answer 10

• Pre-existing renal disease
• Hypertension
• Decreased factor 13 activity

Question 11

What rare manifestation can occur in boys with IgA vasculitis?

Answer 11

Orchitis

Question 12

How does the presentation of IgA vasculitis in adults differ from that in children?

Answer 12

Adults have >60% necrotic lesions, while children have <5%

Question 13

What is a common malignancy found in adults with IgA vasculitis?

Answer 13

Solid malignancy (often lung)

Question 14

What pathology finding is seen in small dermal vessels in IgA vasculitis?

Answer 14

LCCV (Leukocytoclastic vasculitis)

Question 15

What do direct immunofluorescence (DIF) studies show in IgA vasculitis?

Answer 15

Perivascular IgA and C3 deposits

Question 16

What are some differential diagnoses for IgA vasculitis?

Answer 16

• IgA monoclonal gammopathy
• IBD
• Lupus
• Cryoglobulinaemia

Question 17

Is IgA vasculitis generally self-limiting?

Answer 17

True

Question 18

What medications may decrease the duration of cutaneous lesions in IgA vasculitis?

Answer 18

• Dapsone
• Colchicine

Question 19

What is the role of steroids in the treatment of IgA vasculitis?

Answer 19

Do not stop purpura, but reduce duration and treat arthritis, abdominal pain, and GIT complications

Question 20

Should patients with IgA vasculitis be referred for renal evaluation?

Answer 20

Yes

Question 21

What is the current consensus regarding the use of steroids for renal sequelae in IgA vasculitis?

Answer 21

Steroids do not stop renal sequelae but can treat renal nephritis effectively

Question 22

Describe the clinical features of IgA vasculitis?

Answer 22

· Cutaneous as above incl. other lesions of CSVV
○ Begin as urticarial papules or erythematous macules then progress to palpable purpura. Urticaria, vesicles, bullae, targetoid lesions and foci of necrosis can be seen
○ Individual lesions resolve over 10-14/7, which complete resolution in weeks-months