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Henoch-Schönlein purpura


  • Statewide logo

    This guideline has been adapted for statewide use with the support of the Victorian Paediatric Clinical Network

  • See also

    Fever and petechiae
    The acutely swollen joint
    Hypertension
    Haematuria

    Key Points

    1. Urinalysis and blood pressure measurement must be done when Henoch-Schönlein purpura (HSP) is suspected
    2. Most cases are self-limiting and only require symptomatic management
    3. Close follow-up is critical to identify significant renal involvement requiring intervention. Renal involvement is usually asymptomatic

    Background

    HSP is the most common vasculitis of childhood. It most commonly affects children 2-8 years of age 

    Assessment

    HSP is a clinical diagnosis
    The features include rash, and one or more of:

    • Arthritis/arthralgia (50-75%)
    • Abdominal pain (50%)
    • Nephritis (25-50%)

    History

    • The clinical manifestations of HSP can take days to weeks to fully develop and may vary in order of appearance
    • Purpura occurs in all patients but is the presenting complaint in only 75% of cases
    • Recent upper respiratory tract infection is present in 50% of HSP cases, commonly viral or Group A streptococcus infections

    Examination

    Assess for

    Features

    General observations

    Hypertension

    Blood pressure must be measured at initial presentation and at follow-up

    Skin 

    Palpable purpura, petechiae and ecchymoses
    May be preceded by urticarial, erythematous,  maculopapular or bullous skin lesions

    Usually symmetrical
    Located on gravity/pressure-dependent areas (eg buttocks & lower limbs in ambulatory children)

    Painful non-pitting subcutaneous oedema

    Commonly periorbital (in non-ambulant child), and/or dependent areas (hands, feet, scrotum)

    Joints 

    Arthralgia +/- arthritis

    Usually affects large joints of lower limbs. Rarely upper limbs.
    Usually no significant effusion or warmth

    Abdominal 

    Diffuse abdominal pain

    Generalised tenderness
    Signs of bowel obstruction
    Peritonism

    Most common complication is intussusception
    Others include GI haemorrhage, bowel ischaemia, necrosis or perforation, protein-losing enteropathy, pancreatitis

    Genital

    Testicular pain +/-
    orchitis +/- necrosis
    Cord haematoma

    Exclude testicular torsion

    Respiratory (rare) 

    Respiratory distress

    Diffuse alveolar haemorrhage

    Neurological (rare)

    Changes in mental status

    Labile mood, apathy, hyperactivity, encephalopathy

    Focal neurological signs

    Consider intracranial haemorrhage

    Urinalysis 

    Haematuria (microscopic or macroscopic)
    Proteinuria (mild to severe)

    Proteinuria/haematuria, nephrotic syndrome, acute nephritic syndrome, hypertension, renal impairment, renal failure

    Typical rash distribution

    Typical rash distribution

    Management

    Investigations

    Urinalysis is usually the only investigation needed in a classic presentation of HSP
    If there is hypertension, macroscopic haematuria or significant proteinuria, also perform: 

    • Formal urine microscopy and urinary protein-creatinine ratio (UPCR)
    • UEC and albumin

    Additional investigations may be required to rule out differentials if the diagnosis is unclear (eg ITP, leukaemia, meningococcal infection) or to identify potential complications of HSP:

    • FBE, UEC, albumin
    • Blood and urine cultures
    • Abdominal imaging
    • ASOT and anti-DNAse B
    • ANA, dsDNA, ANCA, C3, C4 if significant renal involvement with an unclear diagnosis  

    Treatment

    Mild pain

    • Subcutaneous oedema is managed with bed rest and elevation of the affected area
    • Regular paracetamol and a short course of NSAIDs such as ibuprofen (10 mg/kg TDS) or naproxen (10 mg/kg BD) can be used if not contraindicated (eg GI bleeding or renal impairment)

    Moderate-severe pain

    • Consider the use of steroids (glucocorticoids) in children with moderate to severe abdominal and joint pain (may reduce duration of symptoms)
    • Steroids do not impact the rate of long-term renal complications of HSP
    • If prescribing oral prednisolone, give 1-2 mg/kg/day (maximum 60 mg/day) or IV methylprednisolone 0.8-1.6 mg/kg/day (maximum 1 g/day) while symptoms persist. Once symptoms have resolved, an appropriate weaning regimen should be used

    Follow-up

    • Regular GP or paediatrician review is critical to identify subsequent renal involvement which rarely requires a renal biopsy +/- immunosuppression
    • If initial urinalysis is normal or only reveals microscopic haematuria, review clinically and check BP/urinalysis:
      • Weekly for the first month after disease onset
      • Fortnightly from weeks 5-12
      • Single reviews at 6 and 12 months
      • Return to weekly reviews if there is a clinical disease flare or additional signs of renal involvement
    • If there is no significant renal involvement and normal urinalysis at 12 months, no further follow-up is required

    Consider consultation with local paediatric team when

    Consider admission

    • Serious abdominal complications
    • Severe debilitating pain
    • Severe renal involvement (see UPCR values below)
    • Neurological or pulmonary involvement
    • If treatment with prednisolone is considered

    The development of hypertension, proteinuria or macroscopic haematuria at any point should prompt a paediatric review and the investigations as outlined above

    Consider consultation with a renal specialist when

      • Hypertension
      • Abnormal renal function
      • Macroscopic haematuria for 5 days
      • Nephrotic syndrome
      • Acute nephritic syndrome
      • Persistent proteinuria 
        • UPCR >250 mg/mmol for 4 weeks
        • UPCR >100 mg/mmol for 3 months
        • UPCR >50 mg/mmol for 6 months

    Consider transfer when

    The care required for the child is beyond the comfort level of the health care service

    For emergency advice and paediatric or neonatal ICU transfers, see Retrieval services

    Consider discharge when

    Appropriate follow up has been arranged

    Discharge Advice

    Prognosis

    • A first episode of HSP, in the absence of significant renal disease, usually resolves within 4 weeks.  Rash is often the last symptom to remit
    • Joint pain usually resolves spontaneously within 72 hours
    • Uncomplicated abdominal pain usually resolves spontaneously within 24-48 hours
    • In 25-35% of patients, HSP recurs at least once, within 4 months of the initial presentation. Subsequent episodes are usually associated with milder symptoms that are shorter in duration than previous episodes
    • 90% of those who develop renal complications do so within 2 months of the onset, and 97% within 6 months

    Parent information sheet

    Henoch-Schönlein purpura
    Pain relief for children
    Urine samples
    Starship HSP Record

    Last Updated January 2021

  • Reference List

    1. Bluman, J. and Goldman, R.D., 2014. Henoch-Schönlein purpura in children: Limited benefit of corticosteroids. Canadian Family Physician60(11), pp.1007-1010.
    2. Dudley, J., Smith, G., Llewelyn-Edwards, A., Bayliss, K., Pike, K. and Tizard, J., 2013. Randomised, double-blind, placebo-controlled trial to determine whether steroids reduce the incidence and severity of nephropathy in Henoch-Schönlein Purpura (HSP). Archives of disease in childhood98(10), pp.756-763.
    3. Feng, D., Huang, W.Y., Hao, S., Niu, X.L., Wang, P., Wu, Y. and Zhu, G.H., 2017. A single-center analysis of Henoch-Schonlein purpura nephritis with nephrotic proteinuria in children. Pediatric Rheumatology15(1), p.15.
    4. Gardner-Medwin JM, Dolezalova P, Cummins C, Southwood TR. Incidence of Henoch-Schonlein purpura, Kawasaki disease, and rare vasculitides in children of different ethnic origins. Lancet 2002;360:1197
    5. Hahn, D., Hodson, E.M., Willis, N.S. and Craig, J.C., 2015. Interventions for preventing and treating kidney disease in Henoch‐Schönlein Purpura (HSP). Cochrane Database of Systematic Reviews, (8).
    6. Hunter, K 2006, Henoch Schonlein Pupura guidelines – v1 – GL604, viewed 10th December 2019 , <https://www.royalberkshire.nhs.uk/Downloads/GPs/GP%20protocols%20and%20guidelines/Children%20and%20adolescents/Paeds%20Henoch%20Schonlein%20Purpura%20guideline.pdf>
    7. Jauhola, O., Ronkainen, J., Koskimies, O., Ala-Houhala, M., Arikoski, P., Hölttä, T., Jahnukainen, T., Rajantie, J., Örmälä, T., Turtinen, J. and Nuutinen, M., 2010. Renal manifestations of Henoch–Schönlein purpura in a 6-month prospective study of 223 children. Archives of disease in childhood95(11), pp.877-882.
    8. Lei, W.T., Tsai, P.L., Chu, S.H., Kao, Y.H., Lin, C.Y., Fang, L.C., Shyur, S.D., Lin, Y.W. and Wu, S.I., 2018. Incidence and risk factors for recurrent Henoch-Schönlein purpura in children from a 16-year nationwide database. Pediatric Rheumatology16(1), p.25.
    9. McCarthy H, Tizard J. Clinical practice: Diagnosis and management of Henoch-Schonlein purpura. European Journal of Paediatrics. 2010; 169:643-650.
    10. Starship 2018. Henoch-Schonlein Purpura, viewed 8th December 2019. <https://www.starship.org.nz/for-health-professionals/starship-clinical-guidelines/h/henoch-schonlein-purpura/>
    11. Trnka P. Henoch-Schonlein purpura in children. Journal of Paediatrics and Child Health. 2013; 49:955-1003.
    12. Watson, L., Richardson, A.R., Holt, R.C., Jones, C.A. and Beresford, M.W., 2012. Henoch schonlein purpura–a 5-year review and proposed pathway. PLoS One7(1), p.e29512.
    13. Weiss PF, Feinstein JA, Luan X, et al. Effects of corticosteroid on HenochSchonlein purpura: A systematic review. Pediatrics 2007;120:1079.