HSP Knowledge Flashcards
Henoch-Schonlein Purpura diagnostic criteria?
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)
Henoch-Schonlein Purpura Epidemiology?
Pathophysiology?
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
Etiology of Henoch-Schonlein Purpura?
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
- Genetic
Alpha 1 antitrysin - Parasites
Clinical features of Henoch-Schonlein Purpura?
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
HSP ddx?
Crohns Wegerners Granulomatosis Infective endocarditis IgA nephropathy Haemolytic uraemic syndrome
HSP Rx?
Prognosis?
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
What is Henoch-Schonlein Purpura and the Tetrad?
Bx - Leukocytoclastic Vasculitis in skin and GI tract
- Palpable Purpura (non blanching)
- Arthritis
- GI involvement
- Renal involvement