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What is Henoch-Schönlein Purpura (HSP) / IgA Vasculitis?
Henoch-Schönlein Purpura (HSP), also known as IgA Vasculitis, is a type of vasculitis. It refers to the inflammation of blood vessels. Affected blood vessels can be located in the skin, gut and kidneys. Although majority of cases occur in children, HSP can also occur in adults.
HSP is not inherited or contagious. The cause of HSP is largely unknown, and may be the result of the immune system responding inappropriately to certain triggers such as a cold or acute respiratory infection. The symptoms usually get better in days or weeks without treatment.
Symptoms and Signs of HSP
1. Rashes
The most common type is 'palpable purpura' (tiny raised bruises). They usually occur on the legs or buttocks. In adults, blisters or ulcers may occur.
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Typical HSP rash in the buttocks (left) and lower limbs (right).
Photos retrieved from The Royal Children’s Hospital, Melbourne.
2. Pain or swelling in the joints
Presence of pain, redness or warmth around affected joint. Usually occur in the ankles, knees and elbows. Smaller joints in the hands and feet may also be affected.
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Wrist (left) and ankle (right) arthritis.
Photos retrieved from Johns Hopkins.
3. Gut involvement
Inflammation of the blood vessels in the gut may lead to symptoms such as abdominal pain, nausea or vomiting. Bloody diarrhoea may also be seen in severe cases.
4. Kidney disease
Signs of kidney disease include:
Blood in the urine (microscopic haematuria)
Protein in the urine (proteinuria)
These signs are detected via urine microscopy tests.
Patients often do not have symptoms. However, some may experience leg swelling or high blood pressure.
Most kidney abnormalities resolve after a few weeks. For some patients, the abnormalities persist and may result in severe kidney inflammation ("glomerulonephritis").
How is HSP Diagnosed?
Diagnosis is based on clinical symptoms and signs. Laboratory tests are often performed to determine severity of disease and to exclude other diseases.
These routine tests performed are:
- Full blood count
- Kidney function
- Urine
In unusual or severe cases, you may need a biopsy (e.g. from the skin or kidney) to look for vasculitis (blood vessel inflammation) or kidney involvement.
You may also be required to undergo radiological procedures such as CT scans of the abdomen to check if your gut is affected.
How is HSP Treated?
HSP usually go on for a limited period of time with no long-term complication. Rest and adequate hydration are essential in ensuring full recovery.
The components of HSP treatment are:
- Patient education
- Self-care – hydration and adequate rest
- Medication
- - Non-steroidal anti-inflammatory drugs (NSAIDs) will help to reduce joint inflammation.
- - If your kidney and/or gut is affected, you will need a course of corticosteroids and/or immunosuppressant (e.g. azathioprine).
What are the Chances of Recovery from HSP?
Most children with HSP fully recover but a minority of patients may develop long-term complications such as kidney disease.
In adults, risk of significant kidney disease is higher. Hence, your rheumatologist will continue to follow up with you for regular blood checks and urine tests even after the rash subsides.
Be sure to follow up regularly until your doctor discharges you.
If you have any problems with your treatment please contact your own doctor.
