henoch scholein purpura (hsp) - sheffield children's hospital€¦ · incidence of...

Neme leton & Grace Ehidiamhen Review date: April 2022 © SC(NHS)FT 2019. Not for use outside the Trust. of 14

HENOCH-SCHOLEIN PURPURA (HSP)

Reference: CG1567

Written by: Neme Leton & Grace Ehidiamhen

Peer reviewer Noreen West

Approved: May 2019

Review Due: April 2022

Purpose

It is aimed at providing clinical staff who are presented with a child with HSP

evidenced based information to help guide management. This ensures consistency

in the management of every case avoiding unnecessary investigations, inpatient

admissions while being able to identify children at risk of complications and arrange

adequate follow up.

Intended Audience

All clinical staff providing care to children who present to the Paediatric team

CAEC Registration Identifier: CG1567 Sheffield Children’s (NHS) Foundation Trust

Henoch Scholein Purpura (HSP)


Table of Contents

1. Introduction 2. Guideline Content 3. References 4. Appendix – Blood Pressure Centile charts 2017

1. Introduction

HSP is the most common vasculitis in childhood.

It is a multi-systemic vasculitis affecting the small vessels of the skin, GIT, kidneys, joints and rarely the brain and lungs

It has an incidence of 20.4/100,000 children. It is more common in Asian and Caucasian children and less common in children of Afro-Caribbean heritage.

There is a slight male predominance; male to female ratio 1.2-1.8: 1.0

It is least common in the summer compared to other seasons.

The dominant features include palpable purpura without thrombocytopenia, abdominal pain, arthralgia and / or arthritis and nephritis

Typically HSP is self-limiting with spontaneous resolution within 4 weeks (94%).However there is a 30 -40% recurrence in the first year.

2. Guideline Content

DEFINITION

CLINICAL ASSESSMENT

INVESTIGATIONS

MANAGEMENT

FOLLOW UP

DEFINITION

The diagnosis of Henoch Scholein Purpura (HSP) is largely a clinical one.

The following criteria have been agreed for the diagnosis of HSP 1:

Purpura or petechiae are present (mandatory criterion) with lower limb predominance plus

at least one of four criteria:

1. Abdominal pain

2. Arthritis (acute) or arthralgia 3. Renal involvement such as:

Haematuria Proteinuria




Nephrotic syndrome/nephritis Renal impairment Hypertension

3. IgA deposition on renal biopsy

CLINICAL ASSESSMENT The aetiology of HSP remains unclear; however it is thought to be multifactorial with genetic, environmental and antigenic components. There is usually an antecedent history of URTI, GI or pharyngeal infection in approximately 75% of cases. There have also been reported cases after drug ingestions and vaccinations. History should include history of the rash, likely precipitants and enquire about specific symptoms including abdominal pain, vomiting, haematemesis, melaena, arthralgia, joint swelling and related symptoms, macroscopic haematuria, oedema and past history of HSP. Clinical examination should be detailed specifically looking for clinical features, assessment for oedema and should include measurement of blood pressure. CLINICAL FEATURES SKIN

Palpable purpura on the extensor surface of the dependant parts of the lower limbs and buttocks is classically described in HSP (95 -100% of cases).

Though it can occur elsewhere, the trunk is usually spared.

The rash can initially present as petechiae, maculopapular erythema or urticarial lesions. It typically appears in crops ,with new crops appearing in waves

Severe bullous lesions are seen in 2%. GASTROINTESTINAL

50-75% of patients will have gastrointestinal features which most commonly are diffuse abdominal pain, vomiting and haemorrhage.

Less commonly, it can present as intussusception and a protein losing enteropathy or pancreatitis can occur.

May precede rash

JOINTS

There is joint involvement in 60 -85% of cases which can either be arthralgia or arthritis. It can be the first symptom in 15% of cases.

Major joints of the lower limb are more affected compared to the upper limb, commonly affecting the knees and ankles




It is transient and often migratory and usually resolves in 2-3 days

Joints that are warm, erythematous and with effusion are not typical of HSP

RENAL

20-60% will have renal involvement.

Of these, 70-80% will present in the first 4 weeks but 97-100% by up to 12 weeks from the initial presentation.

About 5-7% of those children with renal features may eventually develop severe renal disease (nephritic syndrome, nephrotic syndrome or renal impairment).

Hypertension though commonly associated with renal features has been known to present without other evidence of renal involvement

OTHERS

About 24% of the boys diagnosed with HSP, will develop orchitis. Surgical exploration may be required if the diagnosis is in doubt and torsion of the testis cannot be excluded on clinical grounds.

Rarely, neurological features in the form of headache or encephalopathy

Pulmonary involvement in the form of diffuse alveolar haemorrhage has been reported.

INVESTIGATIONS

1. Urinalysis

a. Inspect for macroscopic haematuria

b. Dipstick to look for presence of blood and protein

c. Send for Urine protein: creatinine ratio (uPCR) if proteinuria present (preferably early morning sample)

2. Blood

Blood investigation not required if the diagnosis of HSP is certain. However investigations should be undertaken if any of the following apply:

a. There is any doubt about the diagnosis

b. The child is in any way systemically unwell

c. The urine analysis is abnormal or there is macroscopic haematuria, hypertension or oedema

d. The symptoms of HSP are severe

Investigations should include FBC, coagulation screen, U+E, creatinine, bone profile, protein/albumin +/- blood culture and other tests as per meningococcal guideline.

MANAGEMENT




At presentation, most children with HSP are systemically well and admission is not required. Uncomplicated cases of HSP can be discharged from A&E with a clear plan for review at the GP practice and written information

However, if there is any concern that there is systemic illness, investigations and management must reflect this and antibiotics as per the Trust guidelines should be given pending at least culture results.

In most patients, the disease and symptoms will spontaneously resolve and only simple measures and monitoring will be required. However, in some patients referral to General Paediatrics and sometimes admission early in the illness will be needed for symptomatic relief of severe joint or abdominal pain or observation and management of gastrointestinal or other significant complications.

Measures include

1. Simple analgesia for joint and abdominal pain

a. Paracetamol

b. NSAIDs – avoid in the presence of renal disease

2. Steroids

a) There is no strong evidence supporting the routine use of steroids to reduce the long term renal outcome in children with HSP

b) A course of prednisolone 1mg/kg daily for 1 -2 weeks may be considered in children with severe abdominal or joint pain not responding to simple analgesia. This should be a consultant decision.

FOLLOW UP

If the patient is fit for discharge, there should be a hospital review in 1 week following first symptoms. Further follow up should be as below depending on the results of the urinalysis and blood pressure measurement.

All patients should have follow up for at least 6 months by:

o GP (normal urinalysis and normotensive) at 2 weeks, 4 weeks, 3 months and 6 months

o General Paediatrician (microscopic haematuria, proteinuria <200mg/mmol)

o SPIN Paediatrician /Nephrologist ( macroscopic haematuria, proteinuria >200mg/mmol, hypertension, oedema)

On discharge letter to GP, the 95th centile for blood pressure should be included and a clear plan on reason for review (urine dipstick and BP measurement) and how to refer back to hospital

Families should be given HSP leaflet and signposted to the website (www.infoKID.org.uk)

Reasons for further assessment

Further paediatric assessment should be advised if at presentation or on monitoring there is:

http://www.infokid.org.uk/




o Severe rash

o Significant abdominal pain or other gastrointestinal symptoms

o Persisting joint pain or arthritis

o Concern about other complications

Macroscopic haematuria

Proteinuria or Hypertension

Generalised oedema

Impaired renal function (i.e. estimated GFR (40 x ht (cm)/creatinine) < 90 mls/min/1.73m2 )

Other specialist referral

o Dermatology advice for severe rash

o Rheumatology advice may be needed for persisting joint symptoms

o Surgical and gastroenterology opinion may be required for severe abdominal and gastrointestinal symptoms.

SPIN Paediatrician or tertiary nephrologist discussion or referral is required if at presentation or on monitoring any of the following develop:

o Macroscopic haematuria > 5 days

o Significant proteinuria (> 250 mg/mmol at any point, > 100 mg/mmol for 3 months, > 50 mg/mmol for 6 months)

o Hypertension

o Nephrotic syndrome

o Acute nephritic syndrome

o Impaired renal function




A summary of suggested management is illustrated in the flow chart

Patient pathway

NO YES

HSP cases which have been referred to general paediatrics

Normal urinalysis AND

Normotensive

Any Haematuria or Proteinuria on dipstick AND/OR

Hypertension (BP>95th centile on 3 separate occasions)

FBC,Urea & electrolytes,Albumin

Urine protein: creatinine ratio (uPCR)

Any indication for admission

Hypertension

Oedema

Abnormal blood or urine results

Severe joint pain

Severe abdominal pain

GI haemorrhage

Neurological symptoms

Other acute complications eg. orchitis

Follow up in AAU/MDC at 1 week

Normal

Abnormal

Give HSP leaflet GP review at 2 & 4 weeks, 3 and 6 months. GP to rerefer if needed

General Paediatric / SPIN nephrology Follow up

Admit under General paediatrics Discuss/Refer to Paediatric nephrology / SPIN

Hypertension

uPCR>100mg/mmol

Macroscopic haematuria

Albumin <30g/dl

eGFR < 90ml/min/1.73m2




4. References

1. Ozen, S et al. EULAR/PRINTO/PRES criteria for Henoch-Scholein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria. Ann Rheum Dis 2010; 69:798-806.

2. Tizard, EJ et al. Henoch-Schönlein Purpura. Arch Dis Child Educ Pract Ed 2008; 93:1-8.

3. Gardner-Medwin JMM, Dolezalova P, Cummins C, Southwood TR. Incidence of Henoch-Schönlein purpura, Kawasaki disease, and rare vasculitides in children of different ethnic origins. Lancet. 2002; 360: 1197–202.

4. Jauhola O, Ronkainen J, Koskimies O, Ala-Houhala M, Arikoski P, Hölttä T et al. Clinical course of extrarenal symptoms in Henoch-Schönlein purpura: a 6-month prospective study. Arch Dis Child. 2010; 95: 871–876.

5. Chartapisak W, Opastirakul S, Hodson EM, Willis NS, Craig JC. Interventions for preventing and treating kidney disease in Henoch-Schönlein Purpura (HSP). Cochrane Database of Systematic Reviews. 2009, Issue 3. Art. No.: CD005128.

6. Dudley J, Smith G, Llewelyn-Edwards A et al. Rabndomised, double blind, placebo-controlled trial to determine whether steroids reduce the incidence and severity of nephropathy in Henoch-Schonlein Purpura (HSP). Arch Dis Child 2013; 98: 756-763

7. Trapani S, Micheli A, Francesca G, Resti M, Chiappini E, Falcini F et al. Henoch Schönlein purpura in childhood: epidemiological and clinical analysis of 150 cases over a 5-year period and review of literature. Semin Arthritis Rheum. 2005; 35: 143– 53.

8. Weiss PF, Feinstein JA, Luan X, Burnham JM, Feudtner C. Corticosteroids May Improve Clinical Outcomes During Hospitalisation for Henoch-Schönlein Purpura. Pediatrics. 2010; 126(4): 674-681

9. Narchi H. Risk of long term renal impairment and duration of follow up recommended for Henoch-Schönlein purpura with normal or minimal urinary findings: a systematic review. Arch Dis Child. 2005; 90: 916–920.

10. Nottingham University Hospital Clinical guideline for the Management of Henoch Scholein Purpura. Revised June 2016

11. Flynn, J.T., Kaelber, D.C., Baker-Smith, C.M., Blowey, D., Carroll, A.E., Daniels, S.R., de Ferranti, S.D., Dionne, J.D, Falkner, B., Flinn, S.K., Gidding, S.S., Goodwin, C., Leu, M.G., Powers, M.E., Rea, C., Samuels, J., Simasek, M., Thaker, V.V., Urbina, E.M. Clinical Practice Guideline for Screening and Management of High Blood Pressure in Children and Adolescents. Pediatrics. 2017; 140, 3: 1-72




5. Appendix 1: BP Levels for Boys by Age and Height Percentile (taken from Flynn et al. 2017: 9-11)




6. Appendix 2: BP Levels for Girls by Age and Height Percentile (taken from Flynn et al. 2017: 12-14)

henoch scholein purpura (hsp) - sheffield children's hospital€¦ · incidence of...

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