Clinical Practice Guidelines

Henoch-schonlein purpura


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

  • Key Points

    • Urinalysis is the only investigation required in a classic presentation of HSP. Further investigations may be required if the diagnosis is unclear, abdominal symptoms are severe or where there is evidence of significant renal involvement (hypertension, macroscopic haematuria or proteinuria)
    • Most cases are self-limiting and require only symptomatic management
    • Close follow-up is critical to identify significant renal involvement requiring intervention. Such renal involvement can be asymptomatic  

    Background

    • HSP is the most common vasculitis of childhood
    • It is most commonly seen in children 2-8 years of age
    • In ~50% of cases there is a history of a recent upper respiratory tract infection  

    Assessment

    • HSP is characterised by palpable purpura with arthritis/arthralgia (~50-75%), abdominal pain (~50%) and/or renal involvement (~25-50%) (haematuria/proteinuria/hypertension)
    • 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
    • Significant abdominal and renal complications can occur and need exclusion
    • Pulmonary and neurological involvement are both rare but may be life-threatening if present
    Examination Assess for Features
    Vitals
    • Hypertension
     
    Skin
    • Palpable purpura, petechiae and ecchymoses
    • Usually symmetrical
    • Gravity/pressure-dependent areas (buttocks and lower limbs in ambulatory children)
    • Painful subcutaneous oedema
    • Periorbital area
    • Dependent areas (hands, feet, scrotum)
    Joints
    • Arthritis/arthralgia
    • Usually affects large joints of lower limbs
    • Occasionally upper limbs
    • Usually no significant effusion or warmth
    Abdominal
    • Signs of bowel obstruction
    • Peritonism
    • Gastrointestinal haemorrhage
    • Exclude testicular torsion if presents with significant groin pain
    • Most common complication is intussussception
    • Others – GI haemorrhage, bowel ischaemia, necrosis or perforation, protein-losing enteropathy and pancreatitis
    Respiratory
    • Respiratory distress
    • Diffuse alveolar haemorrhage
    Neurological
    • Changes in mental status
    • Labile mood, apathy, hyperactivity, encephalopathy
    • Focal neurological signs
    • Consider intracranial haemorrhage
    Urinalysis
    • Macroscopic haematuria
    • Proteinuria (more than trace on urinalysis)
    • Proteinuria/haematuria, nephrotic syndrome, acute nephritic syndrome, hypertension, renal impairment and in some, renal failure

    Typical rash distribution

    Typical rash distribution

    Investigations

    • Urinalysis is usually the only investigation needed in a classic presentation of HSP
    • If there is hypertension, macroscopic haematuria or significant proteinuria, send urine for formal microscopy and protein-creatinine ratio (UPCR), and bloods for urea/electrolytes/creatinine (UEC) and albumin
    • In some instances further investigations are requested to (a) rule out differentials if the diagnosis is unclear, or (b) to identify potential complications of HSP. These may include
      • FBE, UEC, albumin
      • Blood and urine culture
      • Abdominal imaging
      • ANA, dsDNA, ANCA, C3/C4 if significant renal involvement with an unclear diagnosis  

    Acute Management

    • Mild pain – subcutaneous oedema is managed with bed rest and elevation of the affected area. Regular paracetamol +/- a short course of NSAIDs such as ibuprofen (10 mg/kg TDS) or naproxen (10 mg/kg BD) can be used if not otherwise contraindicated  
    • Moderate-severe pain - The use of steroids (glucocorticoids) has been shown to reduce the duration of abdominal and joint pain. Note that steroids do not impact the rate of long term renal complications of HSP. Oral prednisolone 1-2 mg/kg/day (maximum 60 mg/day) or IV methylprednisolone 0.8-1.6 mg/kg/day (maximum 1g/day) can be used while symptoms persist. Once symptoms have resolved, an appropriate weaning regimen should be used  

    Consider consultation with local paediatric or surgical team

    • Serious abdominal complications
    • Significant renal involvement – see Follow-Up below
    • Pulmonary involvement
    • Neurological involvement  

    Indications for Admission

    • Serious abdominal complications
    • Severe debilitating pain
    • Severe renal involvement (see ‘discussion with Nephrology’ under ‘Follow-Up’ below)
    • Neurological involvement

    Discharge Advice

    Prognosis

    • A first episode of HSP, in the absence of significant renal disease, usually resolves within 4 weeks. The rash is usually 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, usually within 4 months of the initial presentation. Subsequent episodes are usually milder and 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.

    Follow-Up

    • Regular GP or paediatrician review is critical to identify subsequent renal involvement which rarely requires a renal biopsy +/- immunosuppression
    • If the initial urinalysis is normal or only reveals microscopic haematuria, review clinically and check BP/early morning urinalysis at these recommended time intervals:
      • Weekly for the first month after disease onset
      • Fortnightly from weeks 5-12
      • Single reviews at 6 and 12 months
      • Return to 1. if there is a clinical disease flare
    • The development of hypertension, proteinuria or macroscopic haematuria at any point should prompt paediatric review with investigations (outlined above) and ongoing follow-up based on results
    • Discussion with a Renal specialist is recommended if there is:
      • Hypertension
      • Abnormal renal function
      • Macroscopic haematuria for 5 days
      • Nephrotic syndrome
      • Acute nephritic syndrome
      • Persistent proteinuria
        • UPCR >250mg/mmol for 4 weeks
        • UPCR >100mg/mmol for 3 months
        • UPCR >50mg/mmol for 6 months
    • If there is no significant renal involvement plus normal urinalysis at 12 months, no further follow-up is required

    Last revised November, 2016