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Henoch-Schnlein

PurpuraNational Kidney and Urologic Diseases Information Clearinghouse

U.S. Departmentof Health andHuman Services

NATIONALINSTITUTES

OF HEALTH

What is Henoch-Schnleinpurpura (HSP)?Henoch-Schnlein purpura is a disease that

causes small blood vessels in the body tobecome inflamed and leak. The primarysymptom is a rash that looks like many smallraised bruises. HSP can also affect thekidneys, digestive tract, and joints. HSP canoccur any time in life, but it is most commonin children between 2 and 6 years of age.1

Most people recover from HSP completely,though kidney damage is the most likelylong-term complication. In adults, HSP canlead to chronic kidney disease (CKD) andkidney failure, described as end-stage renal

disease when treated with blood-filteringtreatments called dialysis or a kidneytransplant.

1McCarthy JH, Tizard EJ. Clinical practice: diagnosisand management of Henoch-Schnlein purpura.European Journal of Pediatrics. 2010;169:643650.

What are the causes of HSP?Henoch-Schnlein purpura is caused byan abnormal immune system response in

which the bodys immune system attacksthe bodys own cells and organs. Usually,the immune system makes antibodies, orproteins, to protect the body from foreign

substances such as bacteria or viruses. InHSP, these antibodies attack the blood

vessels. The factors that cause this immunesystem response are not known. However,

in 30 to 50 percent of cases, people have anupper respiratory tract infection, such as acold, before getting HSP.2 HSP has also beenassociated with

2Appel GB, Radhakrishnan J, DAgati VD. Secondaryglomerular disease. In: Brenner BM, ed. Brenner& Rectors The Kidney. Vol. 1. 9th ed. Philadelphia:Saunders Elsevier; 2012: 11921277.

infectious agents such as chickenpox,measles, hepatitis, and HIV viruses

medications

foods

insect bites

exposure to cold weather

trauma

Genetics may increase the risk of HSP, asit has occurred in different members of thesame family, including in twins.

What are the symptoms ofHSP?The symptoms of HSP include the following:

Rash. Leaking blood vessels in theskin cause a rash that looks like bruisesor small red dots on the legs, arms,and buttocks. The rash may first looklike hives and then change to look like

bruises, and it may spread to the chest,back, and face. The rash does notdisappear or turn pale when pressed.

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Digestive tract problems. HSP cancause vomiting and abdominal pain,which can range from mild to severe.Blood may also appear in the stool,though severe bleeding is rare.

Arthritis. Pain and swelling can occurin the joints, usually in the knees andankles and less frequently in the elbowsand wrists.

Kidney involvement. Hematuriablood in the urineis a common sign

that HSP has affected the kidneys.Proteinurialarge amounts of proteinin the urineor development of highblood pressure suggests more severekidney problems.

Other symptoms. In some cases,boys with HSP develop swelling ofthe testicles. Symptoms affecting thecentral nervous system, such as seizures,and lungs, such as pneumonia, havebeen seen in rare cases.

Though the rash affects all people with HSP,pain in the joints or abdomen precedes therash in about one-third of cases by as manyas 14 days.1

What are the complicationsof HSP?In children, the risk of kidney damageleading to long-term problems may be ashigh as 15 percent, but kidney failure affectsonly about 1 percent of children with HSP.1

Up to 40 percent of adults with HSP willhave CKD or kidney failure within 15 yearsafter diagnosis.3

A rare complication of HSP isintussusception of the bowel, which includesthe small and large intestines. With thiscondition, a section of the bowel folds intoitself like a telescope, causing the bowel tobecome blocked.

Women with a history of HSP who becomepregnant are at higher risk for high bloodpressure and proteinuria during pregnancy.

3Feehally J, Floege J. IgA nephropathy and Henoch-Schnlein nephritis. In: Floege, J, Johnson RJ, FeehaJ, ed. Comprehensive Clinical Nephrology. 4th ed. St.Louis, MO: Elsevier Saunders; 2010: 270281.

How is HSP diagnosed?A diagnosis of HSP is suspected when a

person has the characteristic rash and one ofthe following:

abdominal pain

joint pain

antibody deposits on the skin

hematuria or proteinuria

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Antibody deposits on the skin can confirmthe diagnosis of HSP. These deposits canbe detected using a skin biopsy, a procedurethat involves taking a piece of skin tissuefor examination with a microscope. A skinbiopsy is performed by a health care providerin a hospital with little or no sedation andlocal anesthetic. The skin tissue is examinedin a lab by a pathologista doctor whospecializes in diagnosing diseases.

A kidney biopsy may also be needed. Akidney biopsy is performed by a health careprovider in a hospital with light sedation andlocal anesthetic. The health care provideruses imaging techniques such as ultrasoundor a computerized tomography scan to guidethe biopsy needle into the organ. The kidneytissue is examined in a lab by a pathologist.The test can confirm diagnosis and beused to determine the extent of kidneyinvolvement, which will help guide treatmentdecisions.

Hematuria and proteinuria are detectedusing urinalysis, which is testing of a urinesample. The urine sample is collected in aspecial container in a health care providersoffice or commercial facility and can betested in the same location or sent to alab for analysis. For the test, a nurse ortechnician places a strip of chemically treatedpaper, called a dipstick, into the urinesample. Patches on the dipstick change color

when blood or protein are present in urine.

How is HSP treated?No specific treatment for HSP exists.The main goal of treatment is to relievesymptoms such as joint pain, abdominalpain, and swelling. People with kidneyinvolvement may receive treatment aimed atpreventing long-term kidney disease.

Treatment is rarely required for the rash.Joint pain is often treated with nonsteroidalanti-inflammatory medications, such asaspirin or ibuprofen. Recent research

has shown corticosteroidsmedicationsthat decrease swelling and reduce theactivity of the immune systemto beeven more effective in treating joint pain.Corticosteroids are also used to treatabdominal pain.

Though rare, surgery may be needed to treatintussusception or to determine the cause ofswollen testicles.

HSP that affects the kidneys may be treatedwith corticosteroid and immunosuppressivemedications. Immunosuppressivemedications prevent the body from makingantibodies. Adults with severe, acutekidney failure are treated with high-dosecorticosteroids and the immunosuppressivecyclophosphamide (Cytoxan).

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People with HSP that is causing high bloodpressure may need to take medicationsthatwhen taken as prescribed by theirhealth care providerlower their bloodpressure and can also significantly slow theprogression of kidney disease. Two typesof blood pressure lowering medications,angiotensin-converting enzyme (ACE)inhibitors and angiotensin receptor blockers(ARBs), have proven effective in slowingthe progression of kidney disease. Manypeople require two or more medications to

control their blood pressure. In addition toan ACE inhibitor or an ARB, a diureticamedication that helps the kidneys removefluid from the bloodmay be prescribed.Beta blockers, calcium channel blockers, andother blood pressure medications may alsobe needed.

Blood and urine tests are used to check thekidney function of people with HSP for atleast 6 months after the main symptomsdisappear.

Eating, Diet, and NutritionEating, diet, and nutrition have not beenshown to play a role in causing or preventingHSP.

Points to Remember Henoch-Schnlein purpura (HSP) is a

disease that causes small blood vesselsin the body to become inflamed andleak.

HSP is caused by an abnormal immunesystem response in which the bodysimmune system attacks the bodys owncells and organs. The factors that causethis immune system response are notknown.

The symptoms of HSP include thefollowing:

rash

digestive tract problems

arthritis

kidney involvement

In children, the risk of kidney damageleading to long-term problems may beas high as 15 percent, but kidney failureaffects only about 1 percent of children

with HSP. Up to 40 percent of adultswith HSP will have CKD or kidneyfailure within 15 years after diagnosis.

A diagnosis of HSP is suspected whena person has the characteristic rash andone of the following:

abdominal pain

joint pain

antibody deposits on the skin

hematuria or proteinuria

Antibody deposits on the skin canconfirm the diagnosis of HSP.

No specific treatment for HSP exists.The main goal of treatment is to relievesymptoms such as joint pain, abdominalpain, and swelling. People with kidneyinvolvement may receive treatmentaimed at preventing long-term kidneydisease.

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Hope through ResearchThrough its Division of Kidney, Urologic,and Hematologic Diseases, the NationalInstitute of Diabetes and Digestive andKidney Diseases (NIDDK) supports severalprograms and studies devoted to improvingtreatment for patients with progressivekidney disease and kidney failure. TheNIDDK maintains the Pediatric NephrologyProgram, which supports research into thecauses, treatment, and prevention of kidneydiseases in children.

Participants in clinical trials can play a moreactive role in their own health care, gainaccess to new research treatments beforethey are widely available, and help othersby contributing to medical research. Forinformation about current studies, visit

www.ClinicalTrials.gov.

For More InformationAmerican Academy of Pediatrics

National Headquarters141 Northwest Point BoulevardElk Grove Village, IL 600071098Phone: 18004339016 or 8474344000Fax: 8474348000Internet: www.aap.org

American Academy of Family Physicians

P.O. Box 11210Shawnee Mission, KS 662071210

Phone: 18002742237 or 9139066000Fax: 9139066075Internet: www.aafp.org

American Society of Pediatric Nephrology

3400 Research Forest Drive, Suite B7The Woodlands, TX 77381Phone: 2814190052Fax: 2814190082Email: info@aspneph.comInternet: www.aspneph.com

National Institute of Arthritis and

Musculoskeletal and Skin Diseases

Information Clearinghouse

National Institutes of Health1 AMS CircleBethesda, MD 208923675Phone: 187722NIAMS (18772264267)

or 3014954484TTY: 3015652966Fax: 3017186366Email: niamsinfo@mail.nih.govInternet: www.niams.nih.gov

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AcknowledgmentsPublications produced by the Clearinghouseare carefully reviewed by both NIDDKscientists and outside experts. TheNational Kidney and Urologic DiseasesInformation Clearinghouse would like tothank Joseph Flynn, M.D., of the AmericanSociety of Pediatric Nephrology (ASPN),for coordinating the original review ofthis publication by the ASPNs Clinical

Affairs Committee: Barbara Fivush, M.D.(co-chair); Steve Wassner, M.D. (co-chair);John Brandt, M.D.; Deepa Chand, M.D.; IraDavis, M.D.; Stuart Goldstein, M.D.; AnnGuillot, M.D.; Deborah Kees-Folts, M.D.;Juan Kupferman, M.D.; Gary Lerner, M.D.;Tej Mattoo, M.D.; Alicia Neu, M.D.; CynthiaPan, M.D.; William Primack, M.D.; andMichael Somers, M.D.

You may also find additional information about thistopic by visiting MedlinePlus atwww.medlineplus.gov.

This publication may contain information aboutmedications. When prepared, this publicationincluded the most current information available.For updates or for questions about any medications,contact the U.S. Food and Drug Administration toll-free at 1888INFOFDA (18884636332) or visitwww.fda.gov. Consult your health care provider formore information.

The U.S. Government does not endorse or favor anyspecific commercial product or company. Trade,proprietary, or company names appearing in thisdocument are used only because they are considerednecessary in the context of the information provided.If a product is not mentioned, the omission does notmean or imply that the product is unsatisfactory.

National Kidney andUrologic DiseasesInformation Clearinghouse

3 Information WayBethesda, MD 208923580Phone: 18008915390TTY: 18665691162Fax: 7037384929Email: nkudic@info.niddk.nih.govInternet: www.kidney.niddk.nih.gov

The National Kidney and Urologic DiseasesInformation Clearinghouse (NKUDIC)is a service of the National Institute ofDiabetes and Digestive and Kidney Diseases(NIDDK). The NIDDK is part of theNational Institutes of Health of the U.S.Department of Health and Human Services.Established in 1987, the Clearinghouseprovides information about diseases of thekidneys and urologic system to people withkidney and urologic disorders and to theirfamilies, health care professionals, and the

public. The NKUDIC answers inquiries,develops and distributes publications, and

works closely with professional and patientorganizations and Government agenciesto coordinate resources about kidney andurologic diseases.

This publication is not copyrighted. The Clearinghouseencourages users of this publication to duplicate anddistribute as many copies as desired.

This publication is available atwww.kidney.niddk.nih.gov.

U.S. DEPARTMENT OF HEALTH

AND HUMAN SERVICES

National Institutes of Health

NIH Publication No. 125860August 2012

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