Henoch-Schonlein Purpura (HSP)

Question 1

Tell me basic intro to Henoch-Schonlein Purpura?

Answer 1

Immune complex small vessel vasculitis
- aka IgA vasculitis

Common in childhood, often Hx of recent URTI

Typically presents:

• palpable purpura
• GI disturbance
• arthritis
• Glomerulonephritis

Most cases are self limiting (need follow up urinalysis in 12months to check renal function).

Question 2

Epidemiology and aetiology of HSP?

Answer 2

HSP is from IgA complexes depositing in small arteries and activating complement.

• renal lesions give a FSGS
• platelets are normal
• M:F 2:1
• 6-20 per 100k annually
• children 2-8years
• preceding URTI
• caucasian
• typically in winter

Risks:

• recent infection: group A strep, mycoplasma, EBV
• vaccination
• drugs, allergens

Question 3

Presentation of HSP?

Answer 3

PALPABLE PURPURA - 50-75%, often on extensor surfaces of feet, legs arms, sometimes buttocks.

GI upset - 50% colicky abdo pain, tenderness, malaenia

ARTHRITIS - and joint swelling in 75% (large joints, lower limb, no effusion - not warm or red)

GLOMERULONEPHRITIS - 50% of patients (haematuria, proteinuria, RBC casts, hypertension, could be nephritic or nephrotic, occurs in first few months and need follow up 12months later).

rare respiratory

scrotal involvement

neurological involvement

Progression:
rashes appear for a period of days at different sites.
- relapsing and remitting with 4 week episodes
- 20% of patients have end stage renal failure
- prognosis worse in adults

Question 4

Diagnosis of Henoch-Schonlein Purpura?

Answer 4

Often clinical diagnosis in children.
Urinalysis should be done.
Proteinuria
raised ESR
Could do urine microscopy and protein/creatinine. U&Es.

Diagnosis can be confirmed with skin biopsy showing IgA deposits in small vessels - rarely done.

Renal biopsy is signs of renal disease.

DIFFERENTIALS:

• idiopathic thrombocytopenia (ITP)
• leukaemias (can cause petechial rash and fever)

Question 5

Treatment of HSP

Answer 5

• medication an be a cause

Analgesia helps with joint and abdo pain:

• paracetamol 10mg/kg QID
• NSAIDs eg ibuprofen 15mg/kg TDS if there isn’t renal failure

Oral steroids if more severe

Dapsone

Colchicine

Significant kidney involvement combine steroids with immunosuppression medication or IV immunoglobulin.

Hospital admission:

• severe pain
• abdo complications
• renal involvement
• neuro signs and symptoms

Question 6

Complications and prognosis of HSP?

Answer 6

Complications:

• GI bleed (can be massive and life threatening)
• chronic renal failure 5%
• acute renal failure (rare)
• Haemoptysis (rare)

Prognosis:

• most cases resolve within 4 weeks
• joint pain subsides in 72 hours
• abdo pain resolves in 48hrs
• HSP recurs in 1/3rd within 4 months

Question 7

Follow up in HSP?

Answer 7

Regular repeats of urinalysis and blood pressure.

• weekly for first month
• every 2 weeks for weeks 5-12
• at 6 and 12 months

Refer to paediatrics / renal if:

• any HTN
• any abnormal renal fucntion
• macroscopic haematuria
• nephritic syndrome
• persistent proteinuria