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Child Health Guideline Identifier

Henoch-Schonlein Purpura

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Introduction

Henoch-Schonlein Purpura (HSP) is the most common systemic vasculitis in childhood. Its cause is unknown.

  • The mean age at presentation is 6 years and it generally affects children aged 2-10 years. 
  • There is a similar incidence in males and females.
  • An upper respiratory tract infection precedes most cases*

* Group A Streptococcus (GAS) has been found in around one third of patients with HSP. It may be worth screening for GAS in individuals with HSP with a throat swab and treating if confirmed, particularly those at high risk of developing other complications (Rheumatic fever, acute post-streptococcal glomerulonephritis).

Definition

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

  • arthritis/arthralgia,
  • abdominal pain,
  • nephritis

Positive histopathologic findings confirm a diagnosis of HSP however diagnosis is usually made on clinical grounds, and biopsy is usually not required.

Rash
  • A palpable purpuric or petechial rash is compulsory for diagnosis. This generally affects the extensor surfaces of the lower limb, buttocks or forearms and is symmetrical. It may spread to involve the arms, trunk and face.
  • Occasionally HSP presents with a non-specific rash (erythematous, macular, urticarial, bullous).
  • Can have associated oedema.
  • Rash does not always precede other symptoms and can take weeks to resolve.
HSP photo 1 HSP photo 2
 HSP photo 3  HSP photo 4
Arthralgia/arthritis (75%)
  • Often oligoarticular and involves large joints of the lower limbs (knee, ankle, hip).
  • Generalised swelling, tenderness and pain is most common. Effusions and erythema are rare.
  • Self-limited and resolves without permanent damage.
Abdominal pain (60%)
  • Generally diffuse colicky abdominal pain (caused by submucosal oedema and haemorrhage of the bowel wall).
  • Nausea and vomiting.
  • Gastrointestinal haemorrhage.
  • Intussusception in 3-4%.
  • Rarely perforation, pancreatitis, protein-losing enteropathy and massive haemorrhage.
Renal involvement/nephritis (25-50%)
  • Wide range of findings: often isolated microscopic haematuria, proteinuria.
  • Can present with or develop macroscopic haematuria, nephrotic syndrome, nephritic syndrome or even isolated hypertension.
  • Typically develops within the first eight weeks from onset of symptoms.
Positive histopathologic findings on skin (leukocytoclastic vasculitis) or renal biopsy (proliferative glomerulonephritis with predominant IgA deposition).

Other organ systems may be involved: genital (scrotal/testicular pain which may mimic torsion and haemorrhage), cerebral (encephalitis, headaches, seizures), respiratory (interstitial disease, pulmonary haemorrhage).

Investigations

Minimal investigations are required and are aimed at identifying nephritis and ruling out other diagnoses.

  • Height (for determining blood pressure centile)
  • Blood pressure (see Starship guideline on Hypertension)
  • Urinalysis and microscopy: protein, blood, dysmorphic red cells and casts, protein:creatinine ratio if positive for protein.
  • Bloods: there is no specific requirement for blood tests.
    • Renal function and associated tests (albumin, calcium and phosphate) should be checked if clinically indicated (if urine positive for both protein and blood, or proteinuria 2+ or more).
    • Other blood tests may be required to rule out other causes: full blood count (Idiopathic Thrombocytopenic Purpura, leukaemia), coagulation studies, blood culture (bacteraemia).
    • If there is significant renal involvement and uncertainty about the cause consider Streptococcal titres, Complement C3 and C4, immunoglobulins and an autoimmune profile - ANCA, ANA, dsDNA
  • Consider intussusception (and abdominal USS) if severe abdominal pain is a feature or there is blood in the stool

General management

Management of HSP is largely supportive.

Analgesia

Paracetamol and non-steroidal anti-inflammatory drugs (NSAIDs) can be given for arthralgia/arthritis. NSAIDs should be used in caution in renal impairment or those with gastrointestinal manifestations.

Steroids

There is no evidence for steroids in preventing renal disease/nephritis.

Steroids may be of benefit for improving the duration of pain in those with severe abdominal pain. Suggested dose 1-2mg/kg prednisone/prednisolone daily for one week with weaning course over 4-8 weeks (due to possible risk of recurrent disease with short courses). Steroids do not change the underlying disease process and there is some concern that they may mask the symptoms of intussusception or other acute abdominal complications. The decision to give a course of steroids is a clinical judgement based on the risks and benefits.

Prognosis

The prognosis in HSP is excellent:

  • Spontaneous resolution in most cases within 4-8 weeks
  • Recurrence occurs in one third of patients usually within four months of initial episode. This can be triggered by intercurrent illness
  • Nephritis occurs in 25-50% of children, most of which resolves
  • A small percentage of children develop significant renal complications and <1% develop end stage renal disease. Factors that increase risk include age (over 8 years), presence of abdominal pain, and recurrent HSP.

Discharge and follow-up plan

Arrange follow up so as not to miss persistent renal disease/nephritis. Nephritis is not always present on initial presentation. 90% of children with nephritis develop this by two months and 97% by six months.  The follow up plan must be clearly documented, including who will oversee this (e.g. GP, community nurse, paediatrician, etc) as per local arrangements.

Provide family with the monitoring record which details the follow up plan for family and GP.  In addition, copy and paste the following advice into the patient's discharge summary under Advice for GP (including the relevant BP)

Your patient has been diagnosed with Henoch Schonlein Purpura (HSP) and requires monitoring of their blood pressure and urine (ideally early morning urine for proteinuria and haematuria).  We have given your patient a record sheet (see below) which details the timing of the required monitoring.  Please refer/discuss with the paediatric team acutely if:

  • Urine shows 2+ or more proteinuria
  • Macroscopic haematuria
  • Blood pressure >95th centile for age, height and sex =

One third of patients with HSP will have a relapse; in this circumstance monitoring should start again at week 1.
No further follow-up is needed for children with normal urine and blood pressure at 12 months.

Patient record sheet: www.starship.org.nz/media/574411/hsp-record-sheet.pdf
For more information: www.starship.org.nz/for-health-professionals/starship-clinical-guidelines/h/henoch-schonlein-purpura/

If HSP fails to improve as expected or is complicated, consider other causes of vasculitis and discussion with Paediatric Rheumatology or Nephrology.


Aim for first morning urine for dipstick testing - this reduces false positive rate from orthostatic proteinuria later in day.

Nephrology referral criteria

Criteria for referral to/discussion with Paediatric Nephrology:

  1. Nephrotic syndrome:
      • Urine protein:creatinine ratio >250mg/mmol
      • Plasma albumin <25g/L
      • Oedema
  2. Hypertension.
  3. Abnormal renal function.
  4. Macroscopic haematuria ≥5 days.
  5. Persistent proteinuria:
    • Urine protein:creatinine ratio >250mg/mmol for 4 weeks
    • Urine protein:creatinine ratio >100mg/mmol for 3 months
    • Urine protein:creatinine ratio >50mg/mmol for 6 months

Persistent microscopic haematuria can be managed as per Haematuria guideline

Information for families

Parent information can be found on the Kidshealth NZ website

References

  1. Trnka, P. Henoch-Schonlein purpura in children. Journal of Paediatrics and Child Health. 2013;49:955-1003.
  2. Jauhola, O., Ronkainen, J., Koskimies, O., Ala-Houhala, M., Arikoski, P., Holtta, T., Jahnukainen, T., Rajantie, J., Ormala, T., Turtinen, J., Nuutinen, M., Renal manifestations of Henoch-Schonlein prupura in a 6 month prospective study of 223 children. Archives of disease in childhood Education and Practice. 2010;95:877-882.
  3. Chartapisak, W., Opastirakul, S., Hodson, E., Willis, N., Craig, J. Interventions for preventing and treating kidney disease in Henoch-Schonlein Purpura (HSP). Cochrane Database of Systematic Reviews. 2009 (3).
  4. Tizard, E., Hamilton-Ayres, M. Henoch-Schonlein purpura. Archives of disease in childhood Education and Practice. 2008;93:1-8.
  5. McCarthy, H., Tizard, J. Clinical practice: Diagnosis and management of Henoch-Schonlein purpura. European Journal of Paediatrics. 2010;169:643-650.
  6. Narchi, H. Risk of long term renal impairment and duration of follow-up recommended for Henoch-Schonlein Purpura with normal or minimal urinary findings: a systematic review. Archives of disease in childhood Education and Practice. 2005;90:916-920.
  7. Bluman, J., Goldman, R. Henoch-Schonlein purpura in children - limited benefit of corticosteroids. Canadian Family Physician. 2004;60:1007-1010.

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Document Control

  • Date last published: 04 July 2018
  • Document type: Clinical Guideline
  • Services responsible: Children’s Emergency Department
  • Owner: Sarah Jamison
  • Editor: Greg Williams
  • Review frequency: 2 years

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