HENLOCH – SCHONLEIN PURPURA
Summary statement: How does the document support patient care? / The purpose of this policy is to provide evidence based guidance for staff on Henloch-Schonlein Purpura
Staff/stakeholders involved in development:
Job titles only / Dr L. Ramakrishnan, DR E. Vamvakiti, Dr R. Remorino
Division: / Women & Child Health
Department: / Paediatrics
Responsible Person: / Chief of Service
Author: / DR L. Ramakrishnan
For use by: / Medical and Nursing staff
Purpose: / To aid recognition, diagnosis and management of the Henloch-Schonlein Purpura condition
This document supports:
Standards and legislation
Key related documents: / Management of meningococcal sepsis
Approved by:
Divisional Governance/Management Group / W & C Divisional Governance
Approval date: / February 2013
Ratified by Board of Directors/ Committee of the Board of Directors / No Applicable – Divisional ratification only required
Ratification Date: / No Applicable – Divisional ratification only required.
Expiry Date: / November 2015
Review date: / August 2015
If you require this document in another format such as Braille, large print, audio or another language please contact the Trusts Communications Team
Reference Number: / To be added by the Library
Version / date / Author / Status / Comment
1.0 / October 2012 / Dr L Ramakrishnan / Live
2.0
3.0
4.0

Index

Page No
1.0 / Introduction
2.0 / Clinical Features
3.0 / Management
4.0 / Follow Up
5.0 / Referral Criteria
References

1.0Introduction

Henoch- Schonlein Purpura is the most common vasculitic disease of childhood.

It is a systemic, IgA-mediated, small vessel vasculitis most commonlyaffecting skin, joints, gastrointestinal tract, and kidneys, butother organs may be affected.

The annual incidence is approximately 13.5-18/100000children.

50% of cases occur in childrenunder 5years of age and 75% are under 10years.

It is more common in boys.

This guideline is intended to aid recognition, diagnosis and management of the condition in primary care, A&E and in Hospital.

2.0Clinical Features

The clinical features can be divided into cutaneous manifestations, gastrointestinal and articular problems and other. Renal involvement is an important complication and may be present at diagnosis.

Cutaneous

  • Erythematous macules usually develop into palpable purpura.
  • Usually involves extensor surfaces of the lower limb and buttocks and can also involve upper limbs
  • Rarely haemorrhagic bullae and vesicles may occur and may desquamate
  • Subcutaneous oedema of the scalp, ears, periorbital area, dorsi of the hands, feet and external genitalia may occur in <3 year olds.

Gastrointestinal

  • GI involvement occurs in 2/3 of cases
  • Abdominal pain precede the rash in 14-36% of the patients
  • May be vomiting, diarrhoea, periumbilical pain, bloody stools and/or upper GIT haemorrhage.

 Intussusception may occur in 4-5 % patients, USS is the investigation of choice as 58% is confined to the small bowel.

  • Rarely haemorrhagic pancreatitis, hydrops of the gallbladder or pseuodomembraneous colitis may occur.

Articular

  • Joint involvement in 50-80% of the patients
  • Arthralgia and periarticular oedema
  • Synovial effusions are typically absent
  • Knees and ankles are most commonly affected, less commonly wrists, fingers and elbows

Renal Involvement

  • Reported incidence of renal disease ranges from 20-100%.
  • Important cause of acute nephritis, nephrotic syndrome and renal failure.
  • Most commonly presents with haematuria and/or proteinuria but could present with features of frank nephritis or nephrotic syndrome. (see flow chart)
  • Mainly (80%) becomes apparentwithin the first four weeks of the illness although may be delayed for 2 months or more, hence the need for structured follow up.
  • Hypertension may occur even without nephritis.

Other features

  • Genital:orchitis, cord haematoma, testicular paian, testicular necrosis, painful ecchymotic induration of the scrotum.
  • CNS: seizures, encephalopathy, coma, Gullain –Barre syndrome, cortical blindness, intracerebral haemorrhages, stroke
  • Carditis
  • Parotitis
  • Pulmonary disease with haemorrhage

Differential Diagnosis

Sepsis

  • Systemic vascultides [Wegeners, SLE, hypersensitivity vasculitis, polyarteritis nodosa]
  • Thrombocytopenic purpura

3.0Management (See flow chart)

  • Diagnosis is clinical but blood tests are helpful to exclude differentials. Assessment should include a full history and examination including weight, blood pressure, and urine dipstick. Ensure results of urine recorded in notes
  • A well child, with a clear diagnosis, without renal involvement or microscopic haematuria alone may be able to managed in primary care or on an ambulatory basis.
  • Admission is required where diagnosis is uncertain and/or the child is unwell or where there is indication of significant renal involvement.
  • Take blood for FBC, U&E, LFT, Clotting, CRP if diagnosis unclear as you would for other presentations of purpura or other investigations as indicated by the presenting symptoms. Other investigations may be required depending on system involvement and disease severity if diagnosis is certain. (see flow chart)
  • Monitor weight, blood pressure, urinalysis and renal function.
  • Provide adequate analgesia. Use NSAIDs with caution. Do not use if there is poor hydration, diarrhoea, vomiting or abnormal urine dipstick.

In- patient management:

  • As above but also
  • Careful attention to fluid balance and electrolytes
  • If severe abdominal pain, arthritis, pulmonary haemorrhage: consider prednisolone 1mg/kg od for 2 weeks then reduce over 2 weeks
  • If significant GI disturbance consider nutritional support
  • If chronic or relapsing skin manifestations consider colchicines (1st line) or dapsone (2nd line). Discuss with dermatologist.

4.0Follow Up (See Flow Chart)

Urine of children with recurrent episodes should be followed up with each episode for at least 3 months after the rash resolves. Depending on their disease severity this may be done by the GP or paediatrician.

If requesting GP follow up ensure they have the appropriate guideline to follow. (consider sending copy of flow sheet with the discharge letter)

5.0Referral Criteria

To Paediatrician

Presence of hypertension, macroscopic haematuria, proteinuria (1+ or more) or a child who is unwell or in pain should prompt referral to a paediatrician.

To Paediatric Nephrologist

  • Persistent hypertension
  • Abnormal renal function
  • Urine protein:creatinine ratio persistently raised (see flow chart)
  • Nephrotic syndrome
  • Acute nephritic syndrome: haematuria plus 2 other signs (proteinuria, oedema, hypertensive, oliguria, raised urea and creatinine)
  • Increasing proteinuria or persistent oedema,
  • 2nd episode of macroscopic haematuria
  • 3+ or more protein on dipstick on 3 separate occasions without intercurrent illness at or after 1 year
  • Microscopic haematuria at 1 year.

References

  1. Stewart M, Savage JM, Bell B, McCord B. Long term renal prognosis of Henoch-Schönlein purpura in an unselected childhood population Eur J Pediatr 1988;147:113-115
  1. E J Tizard Henoch Schonlein Purpura. ArchDis Child 1999; 80:380-383 (April)
  1. Choong CK,Beasly SWIntra-abdominal manifestations of Henoch-Schonlein purpura.J Paediatr Child Health. 1998 Oct;34(5):405-9.

4.Ronald. J.Hogg et al Evaluation and management of proteinuria and nephritic syndrome in children. Paediatrics 2000,105; 1242-1249

5.Ronkainen J et al,Outcome of Henoch-Schonlein nephritis with nephritic range proteinuria.Clinical Nephrology2003;60(2):80-84

6.SalsburyFT.Henoch-Sconlein purpura in children.Report of hundred patients and review of literature.Medicine 1999;78:395-409

7.SanoH et al .Risk factors of renal involvement and significant proteinuria in Henoch-Sconlein purpura.Eur J Pediatr 2002;161(4):196-201

  1. Ronkainen J et al, early prednisolone therapy in Henoch-Schonlein Purpura: a randomised double blind, placebo controlled trial. J. Pediatri 2006 Aug;149 (2) 241-7
  1. Sunderkotter C et al: Management of leucocytoclastic vasculitis. J Dermatol Treat. 2005; 16 (4): 193-206

Henloch – Schonlein Purpura Guidelines

Version 1Page 1 of 8Oct 2012

Henloch – Schonlein Purpura Guidelines

Version 1Page 1 of 8Oct 2012