HSP Knowledge

Question 1

Henoch-Schonlein Purpura diagnostic criteria?

Answer 1

It’s a clinical Dx

The new European league against Rheumatism 2006
Mandatory criteria: Palpable Purpura
Plus at least 1 of following:
- diffuse abdo pain
- IgA deposition in any Bx
- Arthritis / Arthralgias
- Renal involvement (haematuria / proteinuria)

Question 2

Henoch-Schonlein Purpura Epidemiology?

Pathophysiology?

Answer 2

peak age incidence: 4-6
90% before age 10

Antigen and antibody complexes - mainly IgA
As a result of bacterial and viral infections, vaccines, drugs and autoimmune mechanisms. Complexes deposit in small vessel walls and activate the alternate complement pathway

Question 3

Etiology of Henoch-Schonlein Purpura?

Answer 3

1. Bacterial - 
group A beta haemolytic
Strep 
Staph
Helicobacter 
Mycoplasma 

2. Viral
Hep A/ B / E
Herpes Simplex
B19 
Varicella
Adenovirus 
CMV
HIV 

3. Vaccinations 
MMR
Pneumococcal 
Influenza
Meningococcal
Hep B

4. Drugs
Quinolones
Clarithromycin
Codeine
Etanercept 

5. Tumors
NSCLC
Prostate
Lymphoma
Multiple myeloma

6. Genetic
   Alpha 1 antitrysin
7. Parasites

Question 4

Clinical features of Henoch-Schonlein Purpura?

Answer 4

Palpable Purpura with petechiae and ecchymoses

Colicky abdo pain and worse with food (rare Cx is intusseception)

Symmetrical knees and ankles joint pains

Renal - proteinuria and haematuria
5% ESRD in 5 yrs

Question 5

HSP ddx?

Answer 5

Crohns 
Wegerners Granulomatosis 
Infective endocarditis
IgA nephropathy 
Haemolytic uraemic syndrome

Question 6

HSP Rx?

Prognosis?

Answer 6

Self limiting - NSAIDs

Severe rash / colicky abdo pain / renal / rectal involvement - Oral Steroids (1-2 mg/Kg / day - prevent Cx

Nephrotic range of proteinuria and mesenteric Vasculitis - high dose IV pulse steroids
Refer for renal Bx
If no benefits from steroids alone then add immunosuppressants
Can lead to rapidly progressive glomerulonephritis

High dose IVIG for worsening renal function for refractory cases

Prognosis usually good.
1/3 relapses

Worse prognostic factors: 
 > 8 yrs
Greater no of relapses
High Cr at onset 
Proteinuria > 1g/ day
Haematuria
Hypertension 
Persistent purpura
Increase ESR
Low Factor VIII

Question 7

What is Henoch-Schonlein Purpura and the Tetrad?

Answer 7

Bx - Leukocytoclastic Vasculitis in skin and GI tract

1. Palpable Purpura (non blanching)
2. Arthritis
3. GI involvement
4. Renal involvement