Henoch-Schönlein purpura (HSP) is an IgA-mediated, autoimmune hypersensitivity vasculitis of childhood. The main clinical features are skin purpura, arthritis, abdominal pain, gastrointestinal bleeding, orchitis and nephritis. The aetiology remains unknown.
In the UK, the estimated annual incidence is 6-20 cases per 100,000 population.
Henoch-Schönlein purpura (HSP) is the most common form of systemic vasculitis in children; 90% of cases occur in childhood.The peak prevalence is in children aged 4-6 years. It is rare in infants and young children.
The male-to-female ratio is 1.5-2:1. Caucasians are more often affected than other ethnic groups.
Associated conditions preceding HSP include:
- Infections: for example, Group A streptococci, mycoplasma, Epstein-Barr virus.
- Environmental exposures: for example, drug and food allergens, pesticides, cold exposure, insect bites.
- The disease occurs mostly in the winter months. About 50-90% of patients have a preceding upper respiratory tract infection (URTI).
- Generally, patients appear to be mildly ill, with low-grade fever.
- There is a symmetrical, erythematous macular rash, especially on the back of the legs, buttocks and ulnar side of the arms.
- Within 24 hours, the macules evolve into purpuric lesions, which may coalesce and resemble bruises. Typically the purpura are slightly raised and palpable.
- Abdominal pain and bloody diarrhoea may precede the typical purpuric rash. HSP may also cause nausea and vomiting.
- Joint pain, especially in the knees and ankles. Joints may also be swollen and tender but permanent deformity does not occur.
- Renal involvement:
- Affects approximately 40% of children affected with HSP.
- Only a small minority progresses to end-stage kidney disease.
- Usually occurs within three months of disease onset.
- There is usually no relationship between the severity of nephritis and the extent of the other manifestations of HSP.
- Microscopic haematuria with mild-to-moderate proteinuria may occur.
- Nephrotic syndrome may also occur.
- Oliguria and hypertension are uncommon.
- Scrotal involvement may mimic testicular torsion.
- Headaches may occur.
- Intussusception should be considered, even if the typical purpuric rash has evolved (intussusception occurs in 2-3% of HSP patients).
- Connective tissue diseases - eg, systemic lupus erythematosus (SLE).
- Other causes of purpuric rash - eg, thrombocytopenia.
- Other causes of glomerulonephritis.
- Acute haemorrhagic oedema of infancy: self-limiting condition presenting with fever, oedema and rosette-shaped, annular-shaped or targetoid-shaped purpura affecting the face, ears and extremities.
Diagnosis of HSP is clinical and not based on laboratory investigations.The following abnormalities may be present:
- Urinalysis: haematuria and/or proteinuria are present in 20-40% of patients.[1, 4]
- FBC: there may be raised white cell count with eosinophilia; normal or increased platelets.
- Raised ESR.
- Serum creatinine may be elevated in renal involvement.
- Serum IgA levels are often increased.
- Autoantibody screen: connective tissue diseases.
- Abdominal ultrasound: if there are gastrointestinal symptoms - for diagnosis of intestinal obstruction.
- Barium enema: may be used to confirm and treat intussusception.
- Testicular ultrasound: assessment of possible torsion.
- Renal biopsy: if there is persistent nephrotic syndrome.
- HSP is usually self-limiting and no form of therapy has been shown appreciably to shorten the duration of disease or prevent complications. Therefore, treatment for most patients remains primarily supportive.
- Non-steroidal anti-inflammatory drugs (NSAIDs) may help joint pain but should be used with caution in patients with renal insufficiency.
- May require admission to hospital for monitoring of abdominal and renal complications.
- Nephropathy: treated supportively. A variety of drugs (steroids, azathioprine, cyclophosphamide) and plasmapheresis have been used to prevent the progression of the renal disease but the results of trials have been inconsistent.
- Corticosteroids can ameliorate associated arthralgia and the symptoms associated with gastrointestinal dysfunction. There was no evidence of benefit of prednisone in preventing serious long-term kidney disease in HSP.[7, 8]
- No controlled clinical trials have been performed with immunosuppressive drugs, although azathioprine or cyclophosphamide may be beneficial.
- Plasma exchange is used in the management of some adults with vasculitis and idiopathic rapidly progressive nephritis.
- Renal involvement occurs in 50% of older children but is only serious in approximately 10% of patients. Less than 1% of patients with HSP progress to end-stage kidney disease. The renal prognosis is worse in older children and adults.
- Monitoring for renal involvement:
- For those with no proteinuria, recommendations are for blood pressure checking and urinalysis at days 7 and 14 and at 1, 3, 6 and 12 months.
- For those with proteinuria, follow-up is recommended at days 7 and 14, monthly from 1-6 months and then at 12 months.
- Other rare complications include myocardial infarction, pulmonary haemorrhage, pleural effusion, intussusception (in 2-3% of patients), gastrointestinal bleeding, bowel infarction, seizures and mononeuropathies.
- Recurrence of symptoms may occur. Recurrence of renal impairment may also occur but is rare.
- HSP is an acute self-limited illness and usually resolves without treatment, but may rarely lead to complications. Initial attacks of HSP can last for several months. One in every four patients will have one or more recurrences.
- Children younger than 3 years have a shorter, milder course and fewer recurrences.
- Chronic kidney disease may progress, sometimes more than ten years after the initial flare.
- The long-term prognosis of HSP is directly dependent on the severity of renal involvement.
- One study reported that adults with HSP had a higher frequency of renal insufficiency and worse renal outcomes than children.
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- Henoch-Schönlein purpura; DermNet NZ
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