Guess the rash…

Henoch-Schönlein 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

Johann Lukas Schönlein 1793-1864

Eduard Heinrich Henoch 1820-1910

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 cont’d

• 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?

ReferencesWebsites: Mayo clinic

Up-to-dateKidney Foundation (NZ)Patient.co.ukDermNet NZCleveland ClinicJournal of American Medical AssociationMedline PlusMedscapeKidsHealth.org.nzWikipedia (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, 2009Weiss, 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 Henoch-Schonlein purpura: A retrospective cohort study”, The Lancet, 2002