Paediatric Acute care Guidelines PMH Emergency Department

Background

  • Henoch-Schönlein Purpura (HSP) is the most common cause of non-thrombocytopenic purpura in children.
  • The cause is unknown, but it is an IgA-mediated vasculitis of small vessels, usually following an upper respiratory tract infection (URTI).
  • It is more common in children than in adults, with a peak incidence in the 2-8 year age group, and a seasonal peak in winter.
  • The onset of the illness may be acute, with simultaneous involvement of several organ groups, or insidious, with sequential occurrence of features over weeks to months.
  • It is usually a self-limiting disease.
  • Many children will need only a urine test and can be sent home with GP follow up. 

Assessment

Clinical Features

  • The child is generally well-looking
  • There is often a history of a recent URTI
  • Small-vessel vasculitis may involve several organ groups, in particular the skin, joints, gastrointestinal tract and kidneys
  • Rarely, there may be CNS or pulmonary involvement
Rash 
  • Initially blanching pink maculopapules which may be discrete or confluent
  • Progresses to petechiae or purpura, which are often raised (“palpable purpura”)
  • The distribution of the purpura typically involves gravity-dependent body areas, particularly the buttocks, legs and extensor surfaces of the arms. 
Oedema 
  • Vasculitis of dermal vessels also results in angio-oedema
  • Non-pitting, often painful, oedema of dependent areas (hands and feet) as well as the eyelids, lips or scrotum. 
Arthritis
  • Occurs in two-thirds of patients
  • Self-limiting serous joint effusions which resolve over several days
  • Typically involving the gravity-dependent joints (elbows, wrists, knees, ankles) – swollen, painful joints; may cause difficulty in weight-bearing 
GIT
  • Abdominal pain is common, and is generally intermittent and colicky in nature
  • Diarrhoea (with occult blood) is common
  • Occasionally, frank haematemesis or melaena may occur
  • More serious, but infrequent, complications include spontaneous bowel perforation or intussusception 
Renal 
  • 90% of patients have microscopic haematuria, but this is persistent or recurrent in only 5% of cases
  • Serious complications may include acute glomerulonephritis, nephrotic syndrome, acute renal failure (<1%), or isolated hypertension
  • Renal involvement may be a late manifestation, up to 6 months after initial presentation. 

 Differential Diagnosis 

  • Meningococcal septicaemia
  • Thrombocytopenia
  • Other vasculitides
  • Patients initially presenting with only with abdominal pain or arthralgia may pose a diagnostic challenge
  • Patients initially presenting with haematuria only should have other causes of haematuria excluded

Investigations

  • There is no diagnostic investigation for HSP. Investigations are used to detect complications or other causes of purpura  

Urine

  • Microscopic haematuria is common (90%)
  • If macroscopic haematuria is present, urine should be checked for RBC casts (nephritis) and protein (nephrotic syndrome)

Consider 

  • FBC to exclude thromocytopaenia as cause of purpura if diagnosis is uncertain
  • Blood culture, white cell count, CRP, meningococcal PCR if meningococcal septicaemia is suspected
  • Urea, creatinine and electrolytes if renal impairment is suspected
  • Complement levels and ASO-titres if nephritis is present 

Management

Most patients with HSP will be managed as an outpatient with symptomatic treatment (analgesia) and follow up.

  • Document BP
  • Surgical consult for abdominal complications or testicular pain (testicular pain from vasculitis may be difficult to differentiate from testicular torsion)
  • Abdominal X-Ray or ultrasound may be needed to exclude abdominal complications
  • There is some evidence to suggest that prednisolone 1 mg/kg/day for 2 weeks benefits in abdominal and joint pain (and oedema)
    • There is very limited evidence in preventing renal complications
    • The decision whether to start prednisolone should be made by the admitting Paediatric Consultant

Admission criteria

  • Abdominal complications (intussusception, perforation, haematemesis or bloody stools)
  • Renal complications (nephritis, nephrotic syndrome, hypertension, renal failure)
  • Symptomatic relief (severe joint pain or abdominal pain, painful oedema) 

Referrals and follow-up

  • Patients should receive follow up with their GP or Paediatrician for at least 6 months to ensure symptom relief and disease resolution
  • Check BP and urinalysis weekly for one month, then monthly for 6 months after presentation as renal disease may present late
    • If still present at 6 months refer to the Renal team 
  • Provide Henoch-Schonlein Purpura Health Facts 

Nursing

Routine nursing care.

References

  1. Dudley J, Smith G, Llewelyn-Edwards A, Bayliss K, Pike K and Tizard J. Randomised, double-blind, placebo-controlled trial to determine whether steroids reduce the incidence and severity of nephropathy in Henoch-Schonlein Purpura (HSP). Archives of Disease in Childhood 2013, July. Accessed online: http://adc.bmj.com/content/early/2013/07/10/archdischild-2013-303642.abstract
  2. Shin JI and Lee JS. Steroids in Henoch-Schonlein purpura and abdominal pain. Archives of Disease in Childhood. 2006 Aug; 91(8):714. Accessed online: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2083041/
  3. Weiss PF, Klink AJ, Localio R, Hall M, Hexam K, Burnham JM, Keren R and Feudtner C. Corticosteroids may improve clinical outcomes during hospitalisation for Henoch-Schonlein purpura. Paediatrics. 2010 Oct, 126 (4): 627-81. Accessed online: http://www.ncbi.nlm.nih.gov/pubmed/20855386

Tags

We want your feedback!

Help us provide guidelines that are useful to you, the clinician.

Give feedback here