Henoch-Schönlein Purpura (HSP)
What is Henoch-Schönlein Purpura (HSP)
It is a medical condition whereby the small blood vessels becomes inflamed and leak resulting in a typical rash. The purpuric purplish rash looks like many small raised bruises appearing typically on buttocks and legs. The rash color will change from red to purple, to brown, before fading over the following 10 days. HSP can also affect the kidneys, digestive tract and joints resulting in kidney damage, abdomen pain and joint pain respectively. It can occur any time in life, but is most common in children between 2 and 6 years old.
Most people recover from HSP completely as the condition improves on its own. But kidney damage is the most likely long-term complication. In adults, it can lead to chronic kidney disease (CKD) and kidney failure resulting in dialysis or a kidney transplant. Death is rare.
Causes of Henoch-Schonlein Purpura (HSP)
An autoimmune response is triggered such that the body produces antibodies that attack the blood vessels resulting in inflammation of the vessels and cause leakage. The exact cause for this immune system response is unknown. In 30-50% cases, patients have an upper respiratory tract infection before getting HSP. HSP has also been associated with
Other risk factors of developing HSP:
It is a medical condition whereby the small blood vessels becomes inflamed and leak resulting in a typical rash. The purpuric purplish rash looks like many small raised bruises appearing typically on buttocks and legs. The rash color will change from red to purple, to brown, before fading over the following 10 days. HSP can also affect the kidneys, digestive tract and joints resulting in kidney damage, abdomen pain and joint pain respectively. It can occur any time in life, but is most common in children between 2 and 6 years old.
Most people recover from HSP completely as the condition improves on its own. But kidney damage is the most likely long-term complication. In adults, it can lead to chronic kidney disease (CKD) and kidney failure resulting in dialysis or a kidney transplant. Death is rare.
Causes of Henoch-Schonlein Purpura (HSP)
An autoimmune response is triggered such that the body produces antibodies that attack the blood vessels resulting in inflammation of the vessels and cause leakage. The exact cause for this immune system response is unknown. In 30-50% cases, patients have an upper respiratory tract infection before getting HSP. HSP has also been associated with
- infectious diseases like chickenpox, measles, hepatitis, and HIV viruses
- medications
- Extreme Allergic reaction to certain foods (Non-specific)
- insect bites
- exposure to cold weather during winter
- trauma
- Genetics may increase the risk of HSP: people within the same family, including in twins.
Other risk factors of developing HSP:
- Age: in children between 2-6 years old
- Sex: More common in boys than girls
- Race: White and Asian children are more likely to develop HSP than are black children.
- Season: countries with 4 seasons when the weather turns cold, the risk is higher.
Symptoms of HSP
Complications of HSP
For most people, symptoms improve within a month without any remnant symptoms. But recurrences are fairly common.
- Rash: Bruises-like rash (red-purplish) or small red dots appear on the legs, arms, and buttocks. It may spread to the chest, back, and face. The rash does not disappear or turn pale when pressed. Rash may be worse in areas of pressure such as the sock line and waistline.
- Gastrointestinal symptoms: Nausea, vomiting and abdominal pain which is worse at night and bloody stools. Severe bleeding is rare. These symptoms may occur before the rash appears.
- Arthritis: Pain and swelling can occur in the joints, usually in the knees and ankles and less frequently in the elbows and wrists. The joint pain appear before the classical rash by 1-2 weeks. Pain and swelling at joints will subside when the disease clears and leave no lasting damage.
- Kidney involvement: Hematuria (blood in the urine) is a common sign that HSP has affected the kidneys. Proteinuria (protein in the urine) or development of high blood pressure suggests more severe kidney damage. A urine test is done for confirmation of blood and protein in urine.
- Central Nervous symptoms: some people may experience headaches and seizures. But this is less common.
- Other symptoms: In some cases, boys with HSP develop swelling of the testicles. Symptoms affecting the lungs such as pneumonia are seen in rare cases.
Complications of HSP
For most people, symptoms improve within a month without any remnant symptoms. But recurrences are fairly common.
- Kidney damage: In children, the risk of kidney damage is about 15% and risk of kidney failure is abut 1%. Up to 40% of adults with HSP will have Chronic Kidney Damage or kidney failure within 15 years after diagnosis. Meaning the risk is higher in adults and some may need dialysis and transplant.
- Bowel Intussusception: A rare complication whereby a section of the bowel folds into itself like a telescope, causing the bowel to become obstructed. This is rare in adults.
- Pregnancy complications: Women with a history of HSP who become pregnant will be at higher risk for development of high blood pressure and proteinuria during pregnancy.
Diagnosis of HSP
A clinical diagnosis of Henoch Schonlein Purpura is suspected when a person has the classical reddish-purple rash and one of the following:
2. Kidney biopsy: Under ultrasound or a computerized tomography (CT) scan guidance, the biopsy needle is inserted into the kidney and the kidney tissue extracted will be examined in a lab. The test can confirm diagnosis, determine the extent of kidney involvement so as to aid treatment options.
3. Urine analysis: Hematuria and proteinuria can be detected using urinalysis. The urine sample is collected in a special container and can be sent to a lab for analysis.
4. Imaging studies like ultrasound: to look for rare complications like bowel intussusception when patient presents with bowel obstruction.
A clinical diagnosis of Henoch Schonlein Purpura is suspected when a person has the classical reddish-purple rash and one of the following:
- abdominal pain
- joint pain
- antibody deposits on the skin
- hematuria or proteinuria on urine analysis test
2. Kidney biopsy: Under ultrasound or a computerized tomography (CT) scan guidance, the biopsy needle is inserted into the kidney and the kidney tissue extracted will be examined in a lab. The test can confirm diagnosis, determine the extent of kidney involvement so as to aid treatment options.
3. Urine analysis: Hematuria and proteinuria can be detected using urinalysis. The urine sample is collected in a special container and can be sent to a lab for analysis.
4. Imaging studies like ultrasound: to look for rare complications like bowel intussusception when patient presents with bowel obstruction.
Treatment for Henoch-Schonlein Purpura (HSP)
There is no specific treatment for HSP, the symptoms will resolve on its own within a month. The main treatment is to relieve symptoms such as joint pain, abdominal pain, and swelling.
There is no specific treatment for HSP, the symptoms will resolve on its own within a month. The main treatment is to relieve symptoms such as joint pain, abdominal pain, and swelling.
- Bed rest, plenty of fluids
- Pain relievers: nonsteroidal anti-inflammatory (NSAIDS) medications like aspirin and neurofen may help to reduce joint pains
- Corticosteroids: Recent research has shown that corticosteroids reduce swelling, reduce inflammation and thus may help in treating joint pain. It is also used to treat abdominal pain.
- Kidney damage: Treatment is to prevent long-term kidney disease. Patients maybe treated with corticosteroid and immunosuppressive medications. In patients with kidney failure, dialysis and transplant are treatment options. Follow up kidney function test and urine analysis will be necessary to monitor the disease progression up till at least 6 months after the main symptoms disappear.
- Surgery may be needed to treat intussusception but it's a rare complication
- Hypertension medications: those with complications like hypertension can be treated with anti-hypertensive medications like angiotensin-converting enzyme (ACE) inhibitors and angiotensin receptor blockers (ARBs) which are effective in slowing the progression of kidney disease.
