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Henoch Schonlein Purpura (HSP)

Guideline GL604
Approval
Approval Group Job Title, Chair of Committee Date
Paediatric Governance Policy and Dr Ann Gordon, Chair September
Procedure Subcommittee. Paediatric Governance 2012

Change History
Version Date Author, job title Reason
1 09-07-2012 Kate Hunter (SpR) New Guideline

Contents

Introduction to HSP Incidence, aetiology, diagnostic criteria


Presentation
Differential diagnosis
Investigation
Treatment
Criteria for admission
Prognosis
Follow up
Appendix 1 Letter for GP
Appendix 2 Follow up record sheet for parent/GP
Appendix 3 Parent/patient information sheet
Appendix 4 BP Centiles Girls
Appendix 5 BP Centiles - Boys

Author: Kate Hunter Date: September 2012


Job Title: Specialist Registrar Review Date: September 2014
Policy Lead: Group Director, Urgent Care Group Version: 1
Location: Corporate Governance shared drive GL604
Page 1 of 12
CG001 Procedural Documents Policy

Clinical Guideline: Henoch-Scholein Purpura (HSP)

Introduction
HSP is the commonest vasculitis of childhood and is self-limiting in the majority of cases. It
is immune mediated. The aetiology is unknown but the history often identifies a preceeding
throat/URTI infection and some bacteria and viruses have been implicated.

It has an incidence of 10-20 per 100,000. It affects Males>Females in a ratio of 2:1. It is


more common over autumn, winter and spring.
Peak age incidence is 2-11yrs. (50% under 5yrs, 90% under 10yrs)

The agreed criteria for HSP diagnosis is:


Palpable purpura (mandatory) in the presence of at least 1 of the following;
-Diffuse abdominal pain
-Acute arthirits or arthralgia
-Renal involvement (haematuria +/or proteinuria)
-Biopsy showing predominant IgA deposition

Presentation
Skin (100%):
The classical skin rash often precipitates clinical presentation. Palpable purpura distributed
symmetrically over the lower limbs is typical, commonly extensor surfaces and buttocks. It
may also involve arms, face and ears but usually spares the trunk. Purpuric lesions range
from petechiae to larger bruises and may be preceeded by urticaria or erythematous
maculopapular lesions. Bullous lesions are rare. There may also be a painful
subcutaneous oedema (hands, feet, sacrum, scrotum).

Joints (60-80%):
Typically affects the larger joints (knees, ankles) with pain, swelling and reduced range of
movement. Swelling is usually periarticular oedema, synovial effusions are usually absent.
Joint involvement can be debilitating but does not result in permanent damage.

Gastrointestinal (60%)
Varies from colicky abdominal pain, nausea and vomiting to GI haemorrhage,
intussusception and rarely, pancreatitis, hydrops of the gallbladder and protein losing
enteropathy.

Renal (20-60%)
Of those with renal involvement 76% present within 4 weeks of disease onset and 97%
present within 3 months of disease onset. Renal involvement has a wide range;
microscopic haematuria, macroscopic haematuria, proteinura, nephritic/nephrotic syndrome
and renal impairment. Hypertension may be associated with nephritis or be an isolated
finding. Most patients with renal involvement will have mild disease that recovers well. 5%
will have long term morbidity.

Author: Kate Hunter Date: September 2012


Job Title: Specialist Registrar Review Date: September 2014
Policy Lead: Group Director, Urgent Care Group Version: 1
Location: Corporate Governance shared drive GL604
Page 2 of 12
CG001 Procedural Documents Policy

Others;
Cerebral - Headache, seizures, encephalopathy, coma, stroke, GBS
Lung - Interstitial disease, Haemorrhage
Cardiac - Carditis
Genital - Testicular pain/orchitis/necrosis, cord haematoma

Differential Diagnosis
Sepsis, especially meningococcal disease
ITP, Leukaemia and other causes of a thrombocytopenia.
Connective tissue disease; SLE, Wegeners granulomatosis, Polyarteritis Nodosa

Investigations
Diagnosis is usually on clinical grounds. Bloods are needed to rule out other diagnoses
and in assessing the extent of organ involvement.

All patients need weight, height, BP, Urine dipstick


-FBC & film - to rule out thrombocytopenia/leukaemia
-BC, CRP, clotting - if concerned about sepsis
-U&E, Albumin, calcium, phosphate, urine Protein:Creatinine ratio if urine
dipstick has 2+ or more of proteinuria
-FBC and Clotting if macroscopic haematuria

If the diagnosis is in doubt, consider the following additional investigations after discussion
with the on call consultant (+/- tertiary nephrology)
ASOT, Anti-DNAse B
Complement (C3,C4)
Immunoglobulins
Autoimmune profile; ANA, dsDNA, pANCA, cANCA
Renal USS

Treatment
Generally supportive measures and simple analgesia is all that is required.

Joint symptoms usually respond to rest and NSAIDS (avoid NSAIDS if renal disease
present). Steroids have also been used in severe cases (avoid use of NSAIDS and steroids
together).

Abdominal pain usually settles within 72hrs and simple analgesia is adequate. If
abdominal pain is severe, consider using steroids:
Prednisolone discuss dose with Consultant
For more severe GI disease, systemic steroids have been used after discussion with, and
direction by, Paediatric Gastroenterology.
If abdominal pain is acute, severe and/or associated with blood in the stool, then consider
Intussusception - USS and surgical opinion.

Author: Kate Hunter Date: September 2012


Job Title: Specialist Registrar Review Date: September 2014
Policy Lead: Group Director, Urgent Care Group Version: 1
Location: Corporate Governance shared drive GL604
Page 3 of 12
CG001 Procedural Documents Policy

Skin manifestations are generally benign. Severe cases have been treated with
Colchicine or dapsone. Seek Dermatology opinion in these cases.

Treatment in HSP Nephritis is debatable and in cases of significant findings the opinion of a
Paediatric Nephrologist is required. Please see prognosis and follow up section..

Criteria for admission


Severe joint pain/swelling limiting ability to weight bear and mobilize
Severe abdominal pain
GI Haemorrhage
Evidence of nephritis/nephritic syndrome or renal impairment
Neurological symptoms

Prognosis
HSP is usually self-limiting and symptoms resolve within 6 weeks in most cases. 33% will
have relapses/recurrence of symptoms, more likely in patients with renal disease. Mortality
is very low (<1%).
Long term morbidity is usually related to the renal complications and occurs in 5%. Overall,
1% progress to end-stage renal failure. HSP accounts for 3% of all patients with end-stage
renal failure (previously 15%). Persistent purpura (>1month), severe abdominal pain and
bloody stools are risk factors for significant renal involvement. Renal manifestations
generally present in the first 6 months but occasional cases take up to 12 months.
Therefore, prolonged monitoring is required. Urinalysis which is normal at presentation,
and remains so at 6 months is unlikely to progress and the patient can be discharged.
Otherwise ongoing follow up is required until urinalysis is normal on 2 occasions, 6 months
apart.

Referral to a Paediatric Nephrologist is warranted if there is;


-Macroscopic haematuria >5days
-Persistent microscopic hematuria beyond 12 months
-Persistent proteinuria (2+ or more) beyond 3 months
-Hypertension
-Abnormal renal function

Author: Kate Hunter Date: September 2012


Job Title: Specialist Registrar Review Date: September 2014
Policy Lead: Group Director, Urgent Care Group Version: 1
Location: Corporate Governance shared drive GL604
Page 4 of 12
CG001 Procedural Documents Policy

Follow up
Straightforward cases should be shared between GP(see algorithm) or Kempton Ward (if
GP unable) and Outpatients clinic (3months). Note. In cases of HSP relapse, follow up
should occur from the most recent symptoms, not the initial presentation. Refer to
algorithm:

Give Parent/child info/handout (Appendix 3 - pages 8&9)


Contact GP and ensure they are happy to provide follow up. If GP unable to do BP,
organise follow up via Kempton Day Care Unit.
Give Parent/child GP letter and follow-up record sheet (Appendix 1 and 2 - pages 6&7).
Include the systolic BP 95th centile appropriate to the child on the record sheet (Refer to
Appendix 4 Girls, Appendix 5 Boys)

Author: Kate Hunter Date: September 2012


Job Title: Specialist Registrar Review Date: September 2014
Policy Lead: Group Director, Urgent Care Group Version: 1
Location: Corporate Governance shared drive GL604
Page 5 of 12
CG001 Procedural Documents Policy

Appendix 1

Dear Doctor

Your patient has been diagnosed with Henoch Scholein Purpura (HSP) and requires
monitoring of the following: Blood pressure
Early morning urine dipstick for proteinuria and haematuria
This monitoring needs to occur at defined intervals as directed on the patient record sheet.
Essentially this will be weekly for the first month, 2 weekly for the next 2 months and if all
remains well, at 3, 6 and 12 months. Your patient will be seen routinely in paediatric
outpatients at 3 months.

Concerning findings which warrant referral to the paediatric team acutely, include:
Urine dipstick showing 2+ or more proteinuria
Macroscopic haematuria
Blood pressure >95th centile for age/sex this value has been recorded on the
patient record sheet.
Patients with microscopic haematuria are less concerning but if their findings persist at 12
months this will need referral to childrens outpatients.

Should you have any other concerns, or would like to discuss this further, please contact
the paediatric team at Royal Berkshire Hospital.

Weeks 1, 2, 3, 4, 6, 8, 10,
Early am urinanalysis Refer to paediatric team if:
Blood pressure
Proteinuria 2+ or more
Macroscopic haematuria
th
Systolic BP >95 centile

3 months
Childrens Outpatient Appointment
RBH

6 months, 12 months Refer to outpatients if:


Early am urinanalysis Persistent microscopic
Blood pressure haematuria at 12months

One third of patients with HSP will have relapse/recurrent of symptoms. In these instances,
the follow up from a renal point of view should start again at week 1.

Appendix 2
Author: Kate Hunter Date: September 2012
Job Title: Specialist Registrar Review Date: September 2014
Policy Lead: Group Director, Urgent Care Group Version: 1
Location: Corporate Governance shared drive GL604
Page 6 of 12
CG001 Procedural Documents Policy

Henoch-Scholein Purpura (HSP) Follow-Up Record


th
Name. Systolic Blood Pressure 95
.. centile:
DOB
..
Hosp. No. .
Address. Refer to Paediatrics if recorded
th
BP exceeds 95 centile value
...

You/your child has been diagnosed with HSP. It is important that we continue to monitor the kidney
involvement of this disease. You will need to have your blood pressure measured and an early morning
sample of urine (ie. the first urine passed that day) tested at regular intervals as suggested by the table below.
This follow up will take place with your GP and you will be seen in the childrens outpatients at 3 months (or
as required). If in the meantime you or your GP have any concerns, they can be discussed with the
Paediatric Registrar on call.
(Switchboard 0118 322 5111 ask them to bleep the Paediatric Registrar)

Please keep this document safe and fully completed at each stage.

Week Place of Review Date of Urine Urine Systollic


Review dipstick dipstick BP
(Protein) (Blood) (mmHg)
1 GP

2 GP

3 GP

4 GP

6 GP

8 GP

10 GP

12 Childrens
outpatients (RBH)
6 GP/Outpatients
months (RBH)
12 GP/Outpatients
months (RBH)

Author: Kate Hunter Date: September 2012


Job Title: Specialist Registrar Review Date: September 2014
Policy Lead: Group Director, Urgent Care Group Version: 1
Location: Corporate Governance shared drive GL604
Page 7 of 12
Appendix 3
Parent information sheet: Henoch Scholein Purpura (HSP)
This leaflet explains what HSP is, how it will affect your child and how it is managed. If you
have any questions or concerns, please ask your doctor or nurse.

What is HSP?
HSP is a condition which causes the small blood vessels to become inflamed. This
inflammation is called vasculitis. It particularly affects the blood vessels in the skin to cause
a rash called purpura. It also commonly affects the blood vessels in the kidneys, intestines
and the joints to cause the common symptoms of HSP.

What are the causes?


Why these blood vessels get inflamed is not known but it may be triggered by a recent viral
or bacterial infection. HSP can happen at any age but is most common in the 2-10yrs age
group. It affects boys more than girls.
HSP is not contagious.

Signs and Symptoms of HSP.


Skin HSP causes a typical rash. This looks like bleeding points or bruises under the skin
and they dont disappear when they are pressed with a glass. It is usually worse on the
legs and buttocks. Sometimes when the rash first starts it can look like urticaria (nettle
sting rash).

Joints HSP causes swelling and pain of the joints, usually the knees and ankles.
Sometimes the skin on the back of the hands and feet can also get swollen and tender.

Abdominal Pain the inflamed blood vessels can cause abdominal (tummy) pain.
Occasionally there can be bleeding and you must inform your doctor if there is any blood in
the childs faeces (stool). Abdominal pain is usually mild and settles with simple pain killer
medicine. If the abdominal pain is very severe, the child must see a doctor.

Kidneys Your doctor will know if there is a problem with the kidneys by testing a urine
specimen and looking for blood or protein. The childs blood pressure will also be checked.
Serious problems with the kidneys are uncommon but it is important to monitor this. Your
GP and the hospital, will keep testing this over the next 6-12months.

What is the treatment?


There is no specific treatment for HSP and symptoms usually settle on their own over about
6 weeks. One third of patients will have recurrence of symptoms.

Joint pain and abdominal is managed with simple painkillers like paracetamol. Ibuprofen
can be used if there is no kidney involvement. In very severe cases, steroids can be used.

Author: Kate Hunter Date: September 2012


Job Title: Specialist Registrar Review Date: September 2014
Policy Lead: Group Director, Urgent Care Group Version: 1
Location: Corporate Governance shared drive GL604
Page 8 of 12
CG001 Procedural Documents Policy

Your doctor will need to monitor the childs urine and blood pressure over the next year.
Minor changes are common and usually settle without a lasting problem. Serious kidney
involvement is uncommon. Most patients will show a problem with their kidneys in the first
3 months after being diagnosed with HSP.

Abdominal pain usually settles with paracetamol. In very severe cases your doctor may
recommend using steroid medicines.

Contacting us
If you require any further advice please contact:
Kempton Day Bed Unit: 0118 322 7512 / 8754 (Mon-Fri 7am-7pm)
Lion/Dolphin Wards: 0118 322 7519 / 8075 (outside of these hours)

Further information
Henoch Schnlein Purpura Support Group, c/o Contact a Family, 209-211 City Road,
London EC1V 1JN
Tel: 01733 204368 (10am - 2pm)
E-mail: hsphelp@inbox.com Web: www.cafamily.org.uk/Direct/h36.html

UK National Kidney Federation, The Point, Coach Road, Shireoaks, Worksop S81
8BW
Helpline: 0845 601 0209 Web: www.kidney.org.uk

More information about the Trust can be found on our website www.royalberkshire.nhs.uk

Author: Kate Hunter Date: September 2012


Job Title: Specialist Registrar Review Date: September 2014
Policy Lead: Group Director, Urgent Care Group Version: 1
Location: Corporate Governance shared drive GL604
Page 9 of 12
CG001 Procedural Documents Policy

Appendix 4
Blood Pressure centiles for Girls based on age and height;

Author: Kate Hunter Date: September 2012


Job Title: Specialist Registrar Review Date: September 2014
Policy Lead: Group Director, Urgent Care Group Version: 1
Location: Corporate Governance shared drive GL604
Page 10 of 12
CG001 Procedural Documents Policy

Appendix 5
Blood Pressure centiles for Boys based on age and weight

Author: Kate Hunter Date: September 2012


Job Title: Specialist Registrar Review Date: September 2014
Policy Lead: Group Director, Urgent Care Group Version: 1
Location: Corporate Governance shared drive GL604
Page 11 of 12
CG001 Procedural Documents Policy

References
Henoch-Schonlein Purpura
EJ Tizard, MJJ Hamilton-Ayres
Arch Dis Child Educ Pract Ed. 2008; 93; 1-8

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.
H Narchi
Arch Dis Child 2005:90:916-920

Henoch-Schonlein Purpura
EJ Tizard
Arch Dis Child 1999:80:380-383

Renal Involvement in Henoch-Schonlein Purpura: a multivariate analysis of prognostic


factors.
Y Kaku, K Nohara, S Honda
Kidney International Vol 53, (1998) pp 1755-1759

Also reference made to current guidelines in use at:


Childrens Hospital, Melbourne
Childrens Hospital, Bristol
Paediatric Dept. Morriston Hospital, Swansea

Name, Job title and signature: Kate Hunter SpR

Department: Paediatrics
Date: August 2012

Author: Kate Hunter Date: September 2012


Job Title: Specialist Registrar Review Date: September 2014
Policy Lead: Group Director, Urgent Care Group Version: 1
Location: Corporate Governance shared drive GL604
Page 12 of 12