Management of Henoch-Schonlein Purpura in Patients Under 16 Years

• Aims
• History, Examination and Severity
• Pathways - Mild, Moderate and Severe
• Discharge Pathway
• Appendix 1
• Appendix 2

Aims

To improve the diagnosis and management of Henoch-Schonlein Purpura in patients under the age of 16 years.

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History, Examination and Severity

Diagnosis: in the absence of an obvious cause, palpable purpura on extensor surfaces of lower limbs (rash can start as urticaria) in a stable child plus one or more of the following:
Diffuse abdominal pain, arthritis/arthralgia, renal involvement.
Less common complications includes intussusception, orchitis, pulmonary haemorrhage or CNS vasculitis.
The consultant may consider requesting a rheumatology opinion if diagnostic uncertainty.
If the child is unwell or there is any doubts take cultures/ give antibiotics as for possible meningococcal sepsis!
Record weight, urine dipstick and blood pressure
Record observations/ PAWS
Enter pathway corresponding to History & Examination

Overview of pathways

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Pathways

 
Severe HSP is present with any of the following

Severe abdominal pain, blood in stool Severe debilitating joint pain Deranged U&Es or hypertension

1
Does this patient need immediate resuscitation?

Discuss with senior if any uncertainty
If yes call CRASH 2222
IF THERE IS ANY CONCERN OR PATIENT IS UNWELL TREAT AS POSSIBLE MENINGOCOCCAL SEPSIS
If none of the above, progress as follows

2
Cannulate (consider urgency vs use of topical anaesthesia/ sucrose)

If unable to secure access discuss with senior/ consultant.

3
Investigations

CRP FBC U&E Clotting
LFT Glucose Bone profile (Ca²⁺, PO₄, Mg²⁺)

Blood pressure

Urine dip

Early morning protein/creatinine ratio

If severe intermittent abdominal pain, for urgent ultrasound scan to exclude intussusception
Consider requesting secondary investigations (appendix 2) if acute kidney injury e.g. deranged U&Es or hypertension

Analgesia
Avoid NSAIDs if concerns regarding renal involvement.
Prescribe regular paracetamol and consider morphine for severe abdominal pain / joint pains. Please refer to cBNF for doses of morphine and titrate according to pain.
Do not use codeine / dihydrocodeine due to variable conversion in children.

4
Admit
Commence treatment
If shocked (i.e due to GI bleeding) give fluid bolus 0.9% Sodium Chloride
20mL/kg =__mLs / 10mins
If severe GI pain or bleeding for urgent surgical review.
Exclude intussuception and consider appendicitis/ perforationIf severe abdominal pain prescribe prednisolone 1-2 mg/kg (max 40 mg) for 2 weeks with subsequent weaning for 2 weeks.
Patients with severe gut wall oedema/ protein losing enteropathy are likely to require, IV methylprednisolone (10 mg/kg max 500mg OD) for 3/7, then 2 mg/kg oral prednisolone (maximum dose 40mg) weaning dose and elemental diet. This would be associated with bloody diarrhoea and severe abdominal pain after intussusception / other ‘surgical cause’ e.g. appendicitis had been excluded.
Refer to gastroenterology.

If deranged U&Es or hypertension arrange for urgent renal review and carry out secondary investigations (appendix 2).

Moderate HSP is present with any of the following

More severe joint pain (refractory to NSAIDs or incapacitating) Mild – moderate abdominal pain Macroscopic/Microscopic haematuria plus proteinuria on dipstick but normal BP and U&Es

2
Investigations
CRP FBC U&E Clotting
LFT Glucose Bone profile (Ca²⁺, PO₄, Mg²⁺)

Blood pressure

Urine dip

Early morning protein/creatinine ratio

Review with results and observations

observations satisfactory
Investigations satisfactory
No progression of rash

3
Discharge- enter final common pathway
Consider secondary investigations (appendix 2) if significant urine PCR > 100
Consider high dose ibuprofen with ranitidine cover on TTO

• Avoid ibuprofen if any abnormalities on dip/ deranged U&Es, hypertension NSAIDS contraindicated for pain (e.g. renal involvement)
• Else admit, consider need for treatment (Discuss with senior if any uncertainty)

HSP is mild with all of the following

Respiratory rate < 40/minute Normal pulse rate/ capillary refill time Normal temperature Mild joint pain/swelling No abdominal pain Microscopic haematuria with no proteinuria or negative urine dip

1
Observe for 4 hours, in line with PAWS chart
SpR review
If any deterioration in PAWS score, inform Dr immediately
Any concerns please treat as per meningococcal sepsis

2
Investigations
Blood pressure

Urine dip

Review with results and observations

3
Review with observations

Observations satisfactory
Investigations satisfactory
No progression of rash

Discharge- enter final common pathway

• Ibuprofen on TTO standard dose (Note ibuprofen and other NSAIDs are contraindicated if macroscopic haematuria or proteinuria on dip, acute kidney injury with deranged U&Es or hypertension due to renal involvement)

Else admit

consider need for treatment or further investigations
If any concerns treat as possible meningococcal sepsis
(discuss with senior if any uncertainty)

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Discharge Pathway

Repeat set of observations satisfactory
Workbook completed
Name
D.o.B.
Investigation to chase & action to do with this
Telephone contact information written in workbook

Patient advice leaflet given to parents

Discharge letter
given to parents
electronically sent to GP
Contact number to CAT (children’s advice and treatment unit ward L9) given
Rapid access appointment in 3-4 weeks

Please inform parents:
Parents to dip urine daily (until follow up in 3-4 weeks in RAC where BP measured)
Then urine dip 3 times a week for 6/12 unless >1+ of protein on two consecutive days.
Parents to call 0113 392 7409 to speak to CAT consultant or registrar for advice within 24 hours if any of the following.

1. Urine dipstick shows > 1+ protein for 3 consecutive days
2. There is any visible blood (red or cola coloured) in the urine.
3. There is any significant swelling/puffiness with protein in the urine. 

HSP may have the following symptoms as well as the rash

1. Swollen painful joints in up to 80%
2. Gut symptoms in 75%, tummy ache being the most common, but look for warning signs of vomiting bile (dark green vomits) and blood passed at the back passage - these both need urgent review.
3. Testicular pain and swelling in boys - this needs to be reviewed urgently to exclude torsion.
4. Leaky kidneys in 80%
5. Headaches in 30%

Investigation

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Appendix 1

Follow up

After review 1-2 weeks

• If ≤1+ proteinuria review at 1, 3, 6 months

• If > 1+ proteinuria review at 1, 2, 3, 4, 5, 6 months

Primary investigations include urine PCR, U&Es, Albumin, BP
If abnormal i.e. urine PCR > 100 or rapidly increasing urine PCR < 100,r abnormal U&Es and/ or hypertension carry out secondary investigations and refer to renal.

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Appendix 2

Secondary investigations
Anti streptococcal titre (ASOT)
Antinuclear antibody (ANA)
Double stranded DNA (dsDNA)
Anti-neutrophil cytoplasmic antibody (ANCA)
Complement – C3 C4
FBC
Clotting
ESR
CRP
Immunoglobulin profile
Renal Ultrasound