Henoch Scholein Purpura

Question 1

What is Henoch-Schönlein Purpura (HSP)?

Answer 1

A small-vessel vasculitis characterized by IgA deposition in vessel walls, leading to a tetrad of clinical features: palpable purpura, arthritis/arthralgia, abdominal pain, and renal involvement.

Question 2

What is the hallmark feature of HSP?

Answer 2

Palpable purpura on the lower extremities and buttocks.

Question 3

Who is most commonly affected by HSP?

Answer 3

Children aged 3–15 years, with a slight male predominance.

Question 4

What causes HSP?

Answer 4

The exact cause is unknown, but it is often triggered by infections or environmental factors.

Question 5

What are common triggers for HSP?

Answer 5

Upper respiratory infections (e.g., Streptococcus).

Viral infections (e.g., Epstein-Barr virus, adenovirus).

Vaccinations.

Certain medications.

Question 6

What is the underlying mechanism of HSP?

Answer 6

Immune complex deposition of IgA in the small vessel walls leads to inflammation, vessel damage, and organ involvement.

Question 7

Which organs are primarily affected in HSP?

Answer 7

Skin.

Joints.

Gastrointestinal tract.

Kidneys.

Question 8

What are the four key clinical features of HSP?

Answer 8

Palpable purpura: Non-blanching, raised, and commonly on the lower limbs and buttocks.

Arthritis/arthralgia: Often affecting knees and ankles, typically non-erosive.

Abdominal pain: Colicky, may be associated with gastrointestinal bleeding.

Renal involvement: Hematuria, proteinuria, and in severe cases, nephritis.

Question 9

What are rare complications of HSP?

Answer 9

Intussusception (GI tract).

Severe glomerulonephritis leading to renal failure.

Orchitis or scrotal swelling in boys.

Question 10

How is HSP diagnosed?

Answer 10

Clinically based on the presence of palpable purpura and at least one of the following:

• Arthritis or arthralgia.
• Abdominal pain.
• Renal involvement (hematuria, proteinuria).

Question 11

What laboratory findings support the diagnosis of HSP?

Answer 11

Elevated IgA levels in some patients.

Normal platelet count and coagulation studies (rule out thrombocytopenic causes of purpura).

Evidence of hematuria or proteinuria on urinalysis in renal involvement.

Question 12

What imaging studies are used in HSP?

Answer 12

Ultrasound: To detect intussusception in patients with severe abdominal pain.

Renal biopsy: In cases of severe renal involvement, showing IgA deposition on immunofluorescence.

Question 13

What histopathological findings are characteristic of HSP?

Answer 13

Small-vessel leukocytoclastic vasculitis with IgA deposition.

Question 14

What is the primary treatment for mild HSP?

Answer 14

Supportive care, including:

• Adequate hydration
• Pain relief with NSAIDs or acetaminophen (if no renal involvement).

Question 15

When are corticosteroids used in HSP?

Answer 15

For severe symptoms such as:

• Persistent abdominal pain.
• Renal involvement (e.g., nephritis).
• Severe arthritis.

Question 16

What is the role of immunosuppressive agents in HSP?

Answer 16

Used in severe renal disease (e.g., azathioprine, cyclophosphamide).

Question 17

How is intussusception in HSP managed?

Answer 17

Surgical intervention may be required for confirmed cases.

Question 18

What is the long-term risk of renal complications in HSP?

Answer 18

Approximately 1–5% of children may develop chronic kidney disease or end-stage renal disease.

Question 19

What are the main complications of HSP?

Answer 19

Chronic renal impairment.

Intussusception or bowel perforation.

Persistent arthritis.