Paediatric Clinical Guidelines

Renal 6.5

October 2001

Guideline for the Management of

Henoch Schönlein Purpura (HSP)

Background:HSP is the most common vasculitic disease of childhood

Incidence: 18 per 100,000 children

Affects pre-school children predominantly (50% < 5yrs)

Males > females

Commoner during winter to early spring

Clinically:HSP is a multi-system disorder affecting:

  • Skinpurpuric rash (may initially be urticarial)

On limbs (extensor surfaces) and buttocks

  • Joints60-80%

Ankles and knees most commonly

Transient

  • G.I 50-70%

Colicky abdominal pain

Nausea & vomiting

Intestinal haemorrhage

Intussusception

(Pancreatitis)

  • Renal20-100%

Haematuria (microscopic +/- macroscopic)

Proteinuria

Hypertension (may occur without urine abnormalities)

Impaired renal function

  • Cerebralheadaches

Seizures

Coma

  • OthersScrotum (<30%) ΔΔ testicular torsion

Lung

Diagnosis:Usually clinical

Differential diagnosis:

  • Any cause of a non-blanching rash
  • SLE
  • Wegeners
  • Microscopic polyarteritis

Investigations:No specific diagnostic test

ALL children require:

  • Urine dipstick
  • Blood pressure

ONLY consider the following if uncertainty about diagnosis

  • FBC
  • Clotting
  • U&E
  • Autoantibodies

Admit if:

  • Severe symptoms of joint pain
  • Severe abdominal pain
  • G I haemorrhage
  • Neurological symptoms
  • Evidence of acute glomerulonephritis, nephrotic syndrome or abnormal renal function

Clinical course:

  • HSP is usually self-limiting (most remit within 6 weeks). A small minority may relapse
  • Mortality < 1%
  • Long term morbidity is related to renal involvement
  • If the kidneys are involved, this usually manifests within 3 months from the date of onset – but can present up to 12 months later

Management:

  • Joint symptoms:simple analgesia:

Paracetamol, NSAIDS – avoid if hypertensive or

significant proteinuria

  • Abdominal pain:simple analgesia

Severe gut involvement; oral Prednisolone 1mg/kg/day (max 60mgs) for 1 – 2 weeks

  • Renal involvement:See flow sheet for summary

If initial urinalysis shows 2+

proteinuia or more, check U&E’s

Fortnightly early morning urine

Dipstick by parents for 3 months

Monthly check for a total of 12 months from onset. (Can be done by GP)

  • Review on ward or early clinic if symptomatic
  • Provide parents with information leaflet

EARLYreferral to Paediatric Nephrology:

Children with:

  • Acute glomerulonephritis
  • Nephrotic syndrome
  • Impaired renal function

The aim is for early detection of those with severe renal involvement, for treatment with immunosuppression, prior to the development of scarring

FOLLOW UP referral to Paediatric Nephrology: (see flow sheet)

  • Any child with persistent proteinuria (2+ or more) beyond 3 months from the onset.
  • Persistent haematuria beyond 12 months
  • Hypertension
  • Abnormal renal function

Outcomes (with renal involvement)

  • Overall 1% progress to end-stage renal failure
  • HSP accounts for 5-15% of patients entering end-stage renal failure
  • Persistent purpura (> 1/12), severe abdominal pain are all significant risk factors for renal involvement
  • Related to clinical presentation
  1. Microscopic haematuria only
  2. Proteinuria (without nephrotic syndrome) Poorer

+ mico/macroscopic haematuriaoutcome

  1. Acute nephritis (haematuria + ↑BP + ↑creatinine)
  2. Nephrotic syndrome
  3. Mixed nephritic/nephrotic syndrome

ALL patients with renal involvement need long-term follow-up

References:

  1. Tizard, EJ. Henoch Schönlein Purpura. Arch Dis Child 1999;80: 380-383
  1. Kaku Y, Nohara K, Honda S. Renal Involvement in Henoch Schönlein Purpura: A Multivariate Analysis of Prognostic Factors. Kidney Int 1998;53: 1755-1759
  1. Scharer K, Kumar R, Querfeld U, Ruder H, Waldherr R, Schaefer F. Clinical Outcome of Schönlein-Henoch Purpura Nephritis in Children. Clin Nephrol. 1999;13:816-823

PAEDIATRIC CLINICAL GUIDELINES

ISSUE:VERSION: FINAL

Title:

Author: Dr Farida Hussain

Job Title:Consultant Paediatric Nephrologist

First Issued:Jan 05Date Revised: Review Date: Jan 2008

Document Derivation:Consultation Process:

i.e. References: Dr Jonathan Evans – Consultant Paediatric

Included in document Nephrologist Dr Stephanie Smith – Consultant Emergency

Paediatrician

Ratified By: Paediatric Clinical Guidelines Committee

Chaired By:

Consultant with Responsibility: Dr Stephanie Smith

Distribution:All wards QMC and CHN

Training issues: Included in Induction Programme

Audit:

This guideline has been registered with Nottingham City Hospital NHS Trust and QMC Clinical Guidelines Committee. However, clinical guidelines are ’guidelines’ only. The interpretation and application of clinical guidelines will remain the responsibility of the individual clinician. If in doubt contact a senior colleague or expert. Caution is advised when using guidelines after the review date.

MANUAL AMENDMENTS RECORD
(please complete when making any hand-written changes/ amendments to guideline and not processed through guideline committee)
Date / Author / Description

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