Guess the rash

Henoch-Schnlein Purpura

Overview
History Patient presentation Aetiology Epidemiology Symptoms Diagnosis Investigations Duration Renal implications Management Complications Long term follow up Other long term effects Summary & questions

History

Eduard Heinrich Henoch 1820-1910

Johann Lukas Schnlein 1793-1864

Patient presentation

Aetiology
HSP is an immune-mediated vasculitis
Usually linked to a trigger, ie: Infection: Group A streptococcus, H.pylori, Staph aureus, Hib, HepB Antibiotics: Clarithromycin, Ampicillin Vaccination: Influenza, HepB, Measles Insect bites Susceptibility has genetic link

Aetiology

contd

The trigger stimulates the immune system to produce an antibody in response to the antigen - IgA immunoglobulin is the antibody The combined antigen and antibody complexes get deposited in small blood vessels and cause inflammation
complexes can be deposited in the skin (purpura), joints, GI tract, and in kidneys

Epidemiology
Most common form of vasculitis in children Incidence thought to be approximately 20 cases per 100,000 children (under 17 years) Peak prevalence children aged 4-7 years Peak incidence in late autumn and winter with 50-90% having a URTI in the preceding 1-3 weeks Male to female ratio in UK 1.5 : 1 Caucasian and Asian ethnicities more commonly affected

4 key Symptoms
Rash Swollen, sore joints GI symptoms Kidney involvement

Other Symptoms
Low grade fever +/- headache Swelling of testicles Seizures

Diagnosis
Palpable purpura in the presence of one or more of the following:
Diffuse abdominal pain Any biopsy showing predominant immunoglobulin A deposition Arthritis (acute, any joint) or arthralgia Renal involvement (any haematuria or proteinuria)
International Consensus Convention 2006

Differential diagnosis
Acute abdomen Inflammatory bowel disease Juvenile rheumatoid arthritis Kawasaki disease Leukaemia Meningococcemia Thrombocytopenic purpura Acute haemorrhagic edema

Investigations
Urinalysis Blood testing: Blood count Metabolic panel Coagulation studies ESR Serum IgA Autoantibody screen Ultrasound or imaging (GI and testicular) Barium enema Renal biopsy: if there is persistent nephrotic syndrome

Duration
Usually a mild illness Duration of illness is usually 4-6 weeks 30-40% of children will have at least one recurrence within the first year

Renal implications
10 40% paediatric patients with HSP have renal involvement 12% will be left with chronic renal damage 3-4 years after onset 1% will progress to kidney failure and require dialysis

Management
Supportive therapy HSP spontaneously resolves in 94% of children Naproxen, paracetamol, NSAIDs Rest and elevation of extremities Regular urinalysis

Management
Hospitalisation if:
Inability to maintain adequate hydration Severe abdo pain Significant GI bleed Changes in mental status Severe joint involvement Renal insufficiency, hypertension, and/or nephrotic syndrome

Management
For patients with severe symptoms and renal involvement
Frequent urinalysis, pain management, hydration assessment, be aware of complications (ie intussusception) Steroids Immunosuppressants - azathioprine Plasmapheresis High-dose immunoglobulin G Cyclophosphamide

Complications
Renal
Glomerulonephritis, haemorrhagic cystitis, nephrotic syndrome, ureteral obstruction, renal failure

Gastrointestinal
Intussusception, intestinal stricture, bowel/duodenal infarction, bowel perforation, GI haemorrhage

Complications (rare)
Pulmonary
Alveolar haemorrhage, interstitial infiltrate, pulmonary effusion

CNS
Aphasia, ataxia, cerebral haemorrhage, seizure, paresis, neuropathy, cortical blindness, chorea

Other
Myocarditis, orchitis, scrotal edema, testicular torsion

Long term follow-up

Children with normal urinalysis f/u urine testing for 6 months
isolated haematuria +/- non nephrotic range proteinuria that persists after 6 months should have periodic serum creatinine

Long term follow-up

Children with renal involvement
Weekly/bi-weekly urinalysis and blood pressure for 1-2 months (can use home dipstick) Then monthly urinalysis and BP monthly, then every other month until 12 months following presentation

Long term follow-up

Children with renal involvement
Renal biopsy is a good predictor of severity of renal disease:
Pts showing crescents involving 50% of glomeruli have a 37% risk of progressing to end-stage renal disease, and 18% had chronic renal disease

Other long term effects

Women tend to have poorer outcome with ongoing complications Higher probability (three-fold) of pregnant women developing hypertension, pre-eclampsia, proteinuria

Summary
HSP is an uncommon condition that can be either transient or have ongoing serious implications Diagnosis key to identifying condition and managing appropriately Supportive treatment to manage symptoms Intensive follow up of patients with renal involvement

Any questions?

References
Websites: Mayo clinic Up-to-date Kidney Foundation (NZ) Patient.co.uk DermNet NZ Cleveland Clinic Journal of American Medical Association Medline Plus Medscape KidsHealth.org.nz Wikipedia (for history section) YouTube Harpers Textbook of Dermatology, 3rd edition, 2011 (Chapter 160 - online) Gonzalea, Janniger, Schwartz: Pediatric Henoch-Schonlein purpura, International Society of Dermatology, 2009 Weiss, Klink, Localio, Hall, Hexem, Burnham, Keren, Feudtner: Corticosteroids may improve clinical outcomes during hospitalisation for Henoch-Schonlein purpura, Journal of American Academy of Paediatrics, 2010

Chartapisak, Opastirakul, Hodson, Willis, Craig: Interventions for preventing and treating kidney disease in Henoch-Schonlein Purpura (HSP) (Review), Cochrane Review, 2010 Watson, Richardson, Holt, Hones, Beresford: Henoch Schonlein Purpura A 5-year review and proposed pathway, PLoS ONE (online article) Lau, Suzaki, Novak: Pathogenesis of Henoch Schonlein Purpura nephritis, Paediatric Nephrology, 2010 Rai, Nast, Adler: Henoch-Schonlein Purpura nephritis, Journal of American Society of Nephrologists, 1999 Reamy, Williams, Lindsay: Henoch-Schonlein Purpura, Journal of American Academy of Family Physicians, 2009 McCarthy, Tizard: Clinical practice: Diagnosis and management of Henoch Schonlein Purpura, European Journal of Pediatrics, 2010 Penny: An epidemiological study of Henoch-Schonlein purpura, Paediatric Nursing, 2010 Ronkainen, Nuutinen, Koskimies: The adult kidney 24 years after childhood HenochSchonlein purpura: A retrospective cohort study, The Lancet, 2002