Henoch-Schonlein purpura (HSP)

Question 1

What is henoch-schonlein purpura?

Answer 1

• An inflammatory disorder
• Characterized by generalized vasculitis involving the small vessels of the:
  1) Skin
  2) GI tract
  3) Kidneys
  4) Joints
  5) Rarely lungs or CNS
• Most common vasculitis of childhood

Question 2

True or false: If purpura is caused by vasculitis is will be palpable

Answer 2

• True

- Palpable purpura suggests vasculitis

Question 3

Does HSP occur more frequently in males or females?

Answer 3

More common in boys (2:1)

Question 4

What is the classic triad associated with HSP?

Answer 4

1) Palpable purpuric rash on the LOWER extremities
2) Abdominal pain or renal involvement (Nephritis)
3) Arthritis

Question 5

What are the signs of HSP?

Answer 5

1) Rash (100%)
- lower extremity
- Palpable purpura
2) Abdo pain (85%)
- Due to G.I. involvement
3) Joint involvement (70%)
- Knee and ankle pain/swelling
- ++ pain typically refusal to weight bare
- Resolves spontaneosly with no permanent joint damage
4) Renal disease (25-50%)
- Ranges from mild hematuria or proteinuria to renal failure
- Perminant renal failure in 20% of those diagnosed with nephritic or nephrotic syndrome **HOWEVER - that only represents 0.1% of those with HSP

Question 6

What laboratory studies would you order in a patient with suspected HSP?

Answer 6

• ESR, CRP and WBC count will all be elevated
• Platelet count important to differentiate from purpura seen in thrombocytopenia
• Urinalysis to evaluate for hematuria or protein
• Renal function testing to assess for failure
• Stool for blood to evaluate for intestinal ischemia

Question 7

What are the diagnostic criteria for HSP?

Answer 7

Two+ of the following four criteria:

1) Palpable purpura
2) Bowel angina
- diffuse abdo pain
- or bowel ischemia
3) Diagnostic biopsy
- granulocytes in walls of arterioles
- IgA deposits in vessel wall
4) Age under 20 at onset of symptoms

Question 8

How is HSP managed?

Answer 8

Supportive therapy

1) Arthritis
- Treat with NSAIDS
2) Abdominal pain
- Systemic corticosteroids
- 1mg/kg/day prednisone for 1-2 weeks + taper
3) Nephritis
- Corticosteroids or other immunosuppressives

Question 9

What imaging studies might you perform in a patient with HSP?

Answer 9

• Often none
  1) Abdo ultrasound
• If abdominal symptoms, rule out intussiception
  2) Abdo X-ray
• If presentation suggests possible perforation of bowel