Henoch-Schonlein Purpura (HSP)

  • Overview

    Henoch-Schonlein purpura (HSP) is a form of vasculitis, a condition that involves inflammation of the blood vessels.

    • It’s one of the most common forms of vasculitis in childhood.
    • HSP is seen most frequently in children between the ages of 5 and 15 years.
    • It occurs more frequently in boys than girls.
    • A family connection has been noted with HSP, where the disease has happened to two or more siblings of the same family, either at the same time, or one after another.

    Individualized care for your child

    In the rare instance that your child has an aggressive form of HSP, she may end up with serious kidney disease. The End-Stage Renal Program at Boston Children's Hospital consists of an expert team of physicians, surgeons, nurses, nutritionists and social workers who assist children with chronic renal failure.

    We work with families to plan individualized therapy for each child.

    Our goal is to preserve each patient's growth and development by integrating schooling and providing guidance on behavioral and financial issues. We also assist in arranging transport and home help support, as well as visiting nursing services.

    Boston Children's Hospital
    300 Longwood Avenue
    Boston MA 02115

  • In-Depth

    What causes Henoch-Schonlein purpura? 

    As with the other forms of vasculitis, the cause of HSP is not known. HSP may be associated with an upper respiratory tract infection or possibly an allergic reaction.

    Most children with HSP recover completely.

    What are the symptoms of Henoch-Schonlein purpura?

    • purpura - bleeding into the skin, mucous membranes, internal organs and other tissues
    • arthralgia - pain in the joints
    • inflammation of the joints
    • abdominal pain
    • gastrointestinal bleeding - bleeding in the gastrointestinal tract, which includes the mouth, esophagus, stomach and intestines
    • nephritis - inflammation of the kidneys
    • subcutaneous edema - Swelling just below the skin
    • encephalopathy - dysfunction of the brain
    • orchitis - inflammation of the testicle(s)

    Renal (kidney) involvement is common, occurring in 30 to 70 percent of children with HSP.

    • Kidney disease is usually noted after the onset of other symptoms.
    • Worsening of the kidney symptoms and biopsy-confirmed worsening of the kidney lesions may be observed in patients with repeated attacks of rash or hematuria (blood in the urine).
  • Tests

    How is Henoch-Schonlein purpura diagnosed?

    Henoch-Schonlein purpura is diagnosed based on criteria established by the American College of Rheumatology.

    These criteria include:

    • Palpable purpura - Bleeding into the skin or mucous membranes and other tissues or the presence of certain cells when a tissue sample from the purpura is examined under the microscope.
    • Younger than 20 at the onset of the disease.
    • Bowel angina - pain in the abdomen, which is worse after meals.
    • Bowel ischemia - decreased blood flow to an area due to narrowing in an artery, which may result in bloody diarrhea.

    In addition, ultrasound may be used to examine your child’s gastrointestinal tract for presence of the disease.

    If severe kidney lesions are suspected, your child’s doctor may perform a biopsy of your child’s kidney to see how serious the situation is.

  • How is HSP treated?

    Treatments for HSP may include:

    • adequate hydration, or fluid intake
    • careful attention to nutrition
    • pain control with medications such as acetaminophen
    • glucocorticoids (steroids that help control inflammation)

    Most children with HSP recover completely.

    In a very few number of children, the disease is much more aggressive, leading to serious kidney disease. This appears to be more likely in patients with aggressive initial disease who progressed to end-stage kidney disease in less than three years after the onset of HSP.

    If your child is in this situation, a kidney transplant may need to be performed.

  • Research & Innovation

    Anti-rejection research

    Researchers in the Children’s Kidney Transplant Program lead the global development of and participation in pediatric kidney transplant clinical research.

    We are committed to developing innovative and improved anti-rejection medication protocols with the goal of more successful transplants and reduced side effects and complications.

    As a result of trials managed through our center, certain steroids have been eliminated from post-transplant treatment plans for some children.

    National Institutes of Health research programs

    We currently participate in more NIH-sponsored pediatric kidney transplant research studies than any other program in the nation.

    Additional information about our research is available on our research Web site, including a list of transplant studies currently enrolling eligible patients.

    TransplantResearch Center (TRC)

    In 2003, in an effort to speed the knowledge and results gained from research into innovative and improved approaches to the care of transplant patients, Children’s Hospital Boston and Brigham and Woman's Hospital founded the Transplantation Research Center (TRC).

    The TRC provides a rich environment for collaboration of scientists and physicians from both institutions in the areas of transplantation and immunology.

    The TRC, in conjunction with Children’s Pediatric Transplant Center (PTC)—one of the most advanced and comprehensive transplant centers in the country—reaffirm Children's collaborative "bench-to-bedside" philosophy of care.

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