Guess the rash…
Oct 22, 2015
Overview• History• Patient presentation• Aetiology• Epidemiology• Symptoms• Diagnosis• Investigations• Duration• Renal implications• Management• Complications• Long term follow up• Other long term effects• Summary & questions
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
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
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