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Henoch-Schönlein

Purpura
National Kidney and Urologic Diseases Information Clearinghouse

What is Henoch-Schönlein in 30 to 50 percent of cases, people have an


upper respiratory tract infection, such as a
purpura (HSP)? cold, before getting HSP.2 HSP has also been
Henoch-Schönlein purpura is a disease that associated with
U.S. Department causes small blood vessels in the body to
of Health and become inflamed and leak. The primary • infectious agents such as chickenpox,
Human Services
symptom is a rash that looks like many small measles, hepatitis, and HIV viruses
raised bruises. HSP can also affect the • medications
NATIONAL
INSTITUTES kidneys, digestive tract, and joints. HSP can
OF HEALTH occur any time in life, but it is most common • foods
in children between 2 and 6 years of age.1 • insect bites
Most people recover from HSP completely,
• exposure to cold weather
though kidney damage is the most likely
long-term complication. In adults, HSP can • trauma
lead to chronic kidney disease (CKD) and
Genetics may increase the risk of HSP, as
kidney failure, described as end-stage renal
it has occurred in different members of the
disease when treated with blood-filtering
same family, including in twins.
treatments called dialysis or a kidney
transplant.
What are the symptoms of
What are the causes of HSP? HSP?
Henoch-Schönlein purpura is caused by The symptoms of HSP include the following:
an abnormal immune system response in • Rash. Leaking blood vessels in the
which the body’s immune system attacks skin cause a rash that looks like bruises
the body’s own cells and organs. Usually, or small red dots on the legs, arms,
the immune system makes antibodies, or and buttocks. The rash may first look
proteins, to protect the body from foreign like hives and then change to look like
substances such as bacteria or viruses. In bruises, and it may spread to the chest,
HSP, these antibodies attack the blood back, and face. The rash does not
vessels. The factors that cause this immune disappear or turn pale when pressed.
system response are not known. However,

2Appel GB, Radhakrishnan J, D’Agati VD. Secondary


1McCarthy JH, Tizard EJ. Clinical practice: diagnosis glomerular disease. In: Brenner BM, ed. Brenner
and management of Henoch-Schönlein purpura. & Rector’s The Kidney. Vol. 1. 9th ed. Philadelphia:
European Journal of Pediatrics. 2010;169:643–650. Saunders Elsevier; 2012: 1192–1277.
• Digestive tract problems. HSP can What are the complications
cause vomiting and abdominal pain,
which can range from mild to severe.
of HSP?
Blood may also appear in the stool, In children, the risk of kidney damage
though severe bleeding is rare. leading to long-term problems may be as
high as 15 percent, but kidney failure affects
• Arthritis. Pain and swelling can occur only about 1 percent of children with HSP.1
in the joints, usually in the knees and Up to 40 percent of adults with HSP will
ankles and less frequently in the elbows have CKD or kidney failure within 15 years
and wrists. after diagnosis.3
• Kidney involvement. Hematuria—
A rare complication of HSP is
blood in the urine—is a common sign
intussusception of the bowel, which includes
that HSP has affected the kidneys.
the small and large intestines. With this
Proteinuria—large amounts of protein
condition, a section of the bowel folds into
in the urine—or development of high
itself like a telescope, causing the bowel to
blood pressure suggests more severe
become blocked.
kidney problems.
• Other symptoms. In some cases, Women with a history of HSP who become
boys with HSP develop swelling of pregnant are at higher risk for high blood
the testicles. Symptoms affecting the pressure and proteinuria during pregnancy.
central nervous system, such as seizures,
and lungs, such as pneumonia, have How is HSP diagnosed?
been seen in rare cases. A diagnosis of HSP is suspected when a
Though the rash affects all people with HSP, person has the characteristic rash and one of
pain in the joints or abdomen precedes the the following:
rash in about one-third of cases by as many • abdominal pain
as 14 days.1
• joint pain
• antibody deposits on the skin
• hematuria or proteinuria

3FeehallyJ, Floege J. IgA nephropathy and Henoch-


Schönlein nephritis. In: Floege, J, Johnson RJ, Feehally
J, ed. Comprehensive Clinical Nephrology. 4th ed. St.
Louis, MO: Elsevier Saunders; 2010: 270–281.

2 Henoch-Schönlein Purpura
Antibody deposits on the skin can confirm How is HSP treated?
the diagnosis of HSP. These deposits can
No specific treatment for HSP exists.
be detected using a skin biopsy, a procedure
The main goal of treatment is to relieve
that involves taking a piece of skin tissue
symptoms such as joint pain, abdominal
for examination with a microscope. A skin
pain, and swelling. People with kidney
biopsy is performed by a health care provider
involvement may receive treatment aimed at
in a hospital with little or no sedation and
preventing long-term kidney disease.
local anesthetic. The skin tissue is examined
in a lab by a pathologist—a doctor who Treatment is rarely required for the rash.
specializes in diagnosing diseases. Joint pain is often treated with nonsteroidal
anti-inflammatory medications, such as
A kidney biopsy may also be needed. A
aspirin or ibuprofen. Recent research
kidney biopsy is performed by a health care
has shown corticosteroids—medications
provider in a hospital with light sedation and
that decrease swelling and reduce the
local anesthetic. The health care provider
activity of the immune system—to be
uses imaging techniques such as ultrasound
even more effective in treating joint pain.
or a computerized tomography scan to guide
Corticosteroids are also used to treat
the biopsy needle into the organ. The kidney
abdominal pain.
tissue is examined in a lab by a pathologist.
The test can confirm diagnosis and be Though rare, surgery may be needed to treat
used to determine the extent of kidney intussusception or to determine the cause of
involvement, which will help guide treatment swollen testicles.
decisions.
HSP that affects the kidneys may be treated
Hematuria and proteinuria are detected with corticosteroid and immunosuppressive
using urinalysis, which is testing of a urine medications. Immunosuppressive
sample. The urine sample is collected in a medications prevent the body from making
special container in a health care provider’s antibodies. Adults with severe, acute
office or commercial facility and can be kidney failure are treated with high-dose
tested in the same location or sent to a corticosteroids and the immunosuppressive
lab for analysis. For the test, a nurse or cyclophosphamide (Cytoxan).
technician places a strip of chemically treated
paper, called a dipstick, into the urine
sample. Patches on the dipstick change color
when blood or protein are present in urine.

3 Henoch-Schönlein Purpura
People with HSP that is causing high blood • The symptoms of HSP include the
pressure may need to take medications following:
that—when taken as prescribed by their – rash
health care provider—lower their blood
pressure and can also significantly slow the – digestive tract problems
progression of kidney disease. Two types – arthritis
of blood pressure lowering medications,
angiotensin-converting enzyme (ACE) – kidney involvement
inhibitors and angiotensin receptor blockers • In children, the risk of kidney damage
(ARBs), have proven effective in slowing leading to long-term problems may be
the progression of kidney disease. Many as high as 15 percent, but kidney failure
people require two or more medications to affects only about 1 percent of children
control their blood pressure. In addition to with HSP. Up to 40 percent of adults
an ACE inhibitor or an ARB, a diuretic—a with HSP will have CKD or kidney
medication that helps the kidneys remove failure within 15 years after diagnosis.
fluid from the blood—may be prescribed.
• A diagnosis of HSP is suspected when
Beta blockers, calcium channel blockers, and
a person has the characteristic rash and
other blood pressure medications may also
one of the following:
be needed.
– abdominal pain
Blood and urine tests are used to check the
kidney function of people with HSP for at – joint pain
least 6 months after the main symptoms – antibody deposits on the skin
disappear.
– hematuria or proteinuria

Eating, Diet, and Nutrition • Antibody deposits on the skin can


confirm the diagnosis of HSP.
Eating, diet, and nutrition have not been
shown to play a role in causing or preventing • No specific treatment for HSP exists.
HSP. The main goal of treatment is to relieve
symptoms such as joint pain, abdominal
pain, and swelling. People with kidney
Points to Remember involvement may receive treatment
• Henoch-Schönlein purpura (HSP) is a aimed at preventing long-term kidney
disease that causes small blood vessels disease.
in the body to become inflamed and
leak.
• HSP is caused by an abnormal immune
system response in which the body’s
immune system attacks the body’s own
cells and organs. The factors that cause
this immune system response are not
known.

4 Henoch-Schönlein Purpura
Hope through Research For More Information
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Information Clearinghouse
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1 AMS Circle
Bethesda, MD 20892–3675
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5 Henoch-Schönlein Purpura
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U.S. DEPARTMENT OF HEALTH


AND HUMAN SERVICES
National Institutes of Health

NIH Publication No. 12–5860


August 2012

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