Henoch-Schonlein Purpura (HSP)

Question 1

What is Henoch-Schonlein Purpura (HSP)? What are four main characteristics?

Answer 1

Systemic, small vessel, IgA immune complex mediated leukocytoclastic vasculitis. Tetrad of palpable purpura, renal failure, abdominal pain, arthritis/arthalgias.

Question 2

Causes of HSP?

Answer 2

Exposure to trigger that causes antibody formation. Antigen-antibody complex deposits in arteriole, capillary walls of skin, renal mesangium, GI tract. Possible trigger: Grp A strep, viral, drugs, parovirus B19

Question 3

What are some clinical presentations of HSP?

Answer 3

Palpable purpura (BLE and areas under pressure), GI symptoms (nausea, vomiting, cramping, hematochezia, GI bleeding), arthalgias/arthritis, renal involvement, subcutaneous involvment

Question 4

Diagnosis of HSP?

Answer 4

Finding IgA in small vessels. Biopsy shows leukocytoclastic vasculitis (inflammation of small blood vessels of the body that manifest as purpura) These need to be unrelated to thrombocytopenia.

Question 5

Work up of HSP?

Answer 5

No specific results for HSP but can rule out other dz. Run a CBC, CMP, clotting factors, PT, blood cultures, urinalysis, IgA levels elevated in 50% of pts

Question 6

Treatment for acute HSP?

Answer 6

Prednisone 1mg/kg PO QD for 2 weeks for severe arthritis or abdominal pain. Corticosteroids + azathioprine (ISA) can help prevent rapidly progressive glomerulonephritis. Combo steroids + cyclosporin A can help cause remission of nephrotic syndrome in adults.

Question 7

What should you check every 2-4 weeks from first 6 months after first episode of HSP?

Answer 7

Check urinalysis and BP to detect progression of renal dz.