Henoch-Schönlein Purpura

Disclaimer

These guidelines have been produced to guide clinical decision making for the medical, nursing and allied health staff of Perth Children’s Hospital. They are not strict protocols, and they do not replace the judgement of a senior clinician. Clinical common-sense should be applied at all times. These clinical guidelines should never be relied on as a substitute for proper assessment with respect to the particular circumstances of each case and the needs of each patient. Clinicians should also consider the local skill level available and their local area policies before following any guideline.

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Aim

To guide staff with the assessment and management of Henoch-Schönlein purpura.

Background

Henoch-Schönlein Purpura (HSP) is the most common cause of non-thrombocytopenic purpura in children.
The cause is unknown, but it is an IgA-mediated vasculitis of small vessels, usually following an upper respiratory tract infection (URTI).
It is more common in children than in adults, with a peak incidence in the 2-8 year age group, and a seasonal peak in winter^.1 It is slightly more common in males than females.¹
The onset of the illness may be acute, with simultaneous involvement of several organ groups, or insidious, with sequential occurrence of features over weeks to months.
It is usually a self-limiting disease.

Assessment

Clinical Features

The child is generally well-looking
There is often a history of a recent URTI
Small-vessel vasculitis may involve several organ groups, in particular the skin, joints, gastrointestinal tract and kidneys
Rarely, there may be central nervous system or pulmonary involvement.

Rash	Initially blanching pink maculopapules which may be discrete or confluent Progresses to petechiae or purpura, which are often raised ('palpable purpura'). Present in 75% of cases.¹ The distribution of the purpura typically involves gravity-dependent body areas, particularly the buttocks, legs and extensor surfaces of the arms.
Oedema	Vasculitis of dermal vessels also results in angio-oedema Non-pitting, often painful, oedema of dependent areas (hands and feet) as well as the eyelids, lips or scrotum.
Arthritis	Occurs in 50-75% of patients. Usually 1-4 joints involved.¹ Self-limiting serous joint effusions which resolve over several days Typically involving the gravity-dependent joints (elbows, wrists, knees, ankles) - swollen, painful joints; may cause difficulty in weight-bearing
Gastrointestinal tract	Abdominal pain is present in 50% of cases¹, and is generally intermittent and colicky in nature Diarrhoea (with occult blood) is common Occasionally, frank haematemesis or melaena may occur More serious, but infrequent, complications include spontaneous bowel perforation or intussusception(2-3%)¹
Renal	90% of patients have microscopic haematuria, but this is persistent or recurrent in only 5% of cases Serious complications may include acute glomerulonephritis, nephrotic syndrome, acute renal failure (<1%), or isolated hypertension Renal involvement may be a late manifestation, up to 6 months after initial presentation.

Differential diagnosis

Meningococcal septicaemia disease
Thrombocytopenia
Other vasculitides
Patients initially presenting with only with abdominal pain or arthralgia may pose a diagnostic challenge
Patients initially presenting with haematuria only should have other causes of haematuria excluded.

Investigations

There is no diagnostic investigation for HSP - it is a clinical diagnosis.
Investigations are used to exclude other diagnoses or detect complications of HSP.

Urine

Microscopic haematuria is common (90%)¹
If macroscopic haematuria is present, urine should be checked for red blood cell casts (nephritis) and protein (nephrotic syndrome).

Consider

Full blood count to exclude thrombocytopaenia
Blood culture, white cell count, CRP, meningococcal PCR if infection is suspected
Urea, creatinine and electrolytes if renal impairment is suspected
Complement levels and ASO-titres if nephritis is present.

Management

Most patients with HSP will be managed as an outpatient with symptomatic treatment (NSAIDs for analgesia) and follow up.

Document blood pressure
Surgical consult for abdominal complications or testicular pain (testicular pain from vasculitis may be difficult to differentiate from testicular torsion)
Abdominal X-Ray or ultrasound may be needed to exclude abdominal complications
There is some evidence to suggest that prednisolone 1 mg/kg/day for 2 weeks benefits in abdominal and joint pain (and oedema) ²
- There is very limited evidence in preventing renal complications.

Admission criteria

Abdominal complications (intussusception, perforation, haematemesis or bloody stools)
Renal complications (nephritis, nephrotic syndrome, hypertension, renal failure)
Symptomatic relief (severe joint pain or abdominal pain, painful oedema).

Referrals and follow-up

Patients should receive follow up with their GP or paediatrician for at least 6 months to ensure symptom relief and disease resolution
Check blood pressyre and urinalysis weekly for one month, then monthly for 6 months after presentation as renal disease may present late
- If still present at 6 months refer to the Renal team
Provide Henoch-Schonlein Purpura - Health Fact sheet.

References

Dedeoglu F & Kim S. IgA vasculitis (Henoch-Schönlein purpura): Clinical manifestations and diagnosis. In: UpToDate, Post TW (Ed), UpToDate, Waltham MA. 2019. Accessed at www.uptodate.com.
Dedeoglu F & Kim S (2021) IgA vasculitis (Henoch-Schönlein purpura): Management. In: UpToDate, Post TW (Ed), UpToDate, Waltham MA. 2021. Accessed at www.uptodate.com.
Dudley J, Smith G, Llewelyn-Edwards A, Bayliss K, Pike K and Tizard J. Randomised, double-blind, placebo-controlled trial to determine whether steroids reduce the incidence and severity of nephropathy in Henoch-Schonlein Purpura (HSP). Archives of Disease in Childhood, 2013, 98 (10), p756-763.
Weiss PF, Klink AJ, Localio R, Hall M, Hexam K, Burnham JM, Keren R and Feudtner C. Corticosteroids may improve clinical outcomes during hospitalisation for Henoch-Schonlein purpura, Paediatrics, 2010, 126 (4), p627-81.

Endorsed by:	Executive Director, Medical Services	Date:	Oct 2021
		Review date:	Sep 2022

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