Paeds henoch schonlein purpura guideline

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

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Henoch Schonlein Purpura (HSP) Guideline – GL604

Approval

Approval Group Job Title, Chair of Committee Date Paediatric Governance Policy and Procedure Subcommittee.

Dr Ann Gordon, Chair Paediatric Governance

September 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





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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.





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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, Wegener’s 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.





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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





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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)





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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.

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

Weeks 1, 2, 3, 4, 6, 8, 10, Early am urinanalysis Blood pressure

3 months Childrens’ Outpatient Appointment RBH

6 months, 12 months Early am urinanalysis Blood pressure

Refer to paediatric team if: Proteinuria 2+ or more Macroscopic haematuria Systolic BP >95

th centile

Refer to outpatients if: Persistent microscopic haematuria at 12months





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Henoch-Scholein Purpura (HSP) Follow-Up Record

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 children’s 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 Review

Urine dipstick (Protein)

Urine dipstick (Blood)

Systollic BP (mmHg)

1 GP

2 GP

3 GP

4 GP

6 GP

8 GP

10 GP

12 Childrens outpatients (RBH)

6 months

GP/Outpatients (RBH)

12 months

GP/Outpatients (RBH)

Name…………………………………………………………... DOB…………………………………………………………….. Hosp. No. ……………………………………………………. Address………………………………………………………. …………………………………………………………………...

Systolic Blood Pressure 95th

centile: Refer to Paediatrics if recorded BP exceeds 95

th centile value





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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 don’t 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 child’s 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 child’s 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.





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Your doctor will need to monitor the child’s 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 Schönlein Purpura Support Group, c/o Contact a Family, 209-211 City Road, London EC1V 1JN

Tel: 01733 204368 (10am - 2pm) E-mail: [email protected] 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





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





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





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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: Children’s Hospital, Melbourne Children’s Hospital, Bristol Paediatric Dept. Morriston Hospital, Swansea Name, Job title and signature: Kate Hunter SpR Department: Paediatrics Date: August 2012

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Paeds henoch schonlein purpura guideline