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Henoch-Schonlein Purpura (Henoch-Schönlein Purpura, HSP)

An autoimmune disease affecting mostly children between ages 2 and 11 that causes the blood vessels in the skin to swell. Rash and bruising are the main symptoms.

PubMed Health Glossary
(Source: NIH - National Institute of Diabetes and Digestive and Kidney Diseases)

About Henoch-Schonlein Purpura (HSP)

Henoch-Schönlein purpura is a disease that causes small blood vessels in the body to become inflamed and leak. The primary symptom is a rash that looks like many small raised bruises. HSP can also affect the kidneys, digestive tract, and joints. HSP can occur any time in life, but it is most common in children between 2 and 6 years of age. Most people recover from HSP completely, though kidney damage is the most likely long-term complication. In adults, HSP can lead to chronic kidney disease (CKD) and kidney failure, described as end-stage renal disease when treated with blood-filtering treatments called dialysis or a kidney transplant.

What are the causes of HSP?

Henoch-Schönlein purpura is caused by an abnormal immune system response in which the body's immune system attacks the body's own cells and organs. Usually, the immune system makes antibodies, or proteins, to protect the body from foreign substances such as bacteria or viruses. In HSP, these antibodies attack the blood vessels....Read more about Henoch-Schonlein Purpura NIH - National Institute of Diabetes and Digestive and Kidney Diseases

What works? Research summarized

Evidence reviews

Interventions for preventing and treating kidney disease in Henoch‐Schönlein Purpura

Henoch‐Schönlein Purpura (HSP) causes inflammation of small blood vessels in children and affects approximately 20/100,000 children annually. Symptoms and signs include a purpuric skin rash (which comprises small spots and larger bruises), abdominal pain, gastrointestinal bleeding, joint pain and swelling, facial swelling and evidence of kidney disease with blood and protein in the urine. Kidney disease occurs in about one third of children with HSP. In the majority this is mild (small amounts of blood in the urine only) and resolves completely but a few children have persistent kidney disease that can progress to kidney failure.

Efficacy and safety of mycophenolate mofetil for Henoch-Schonlein purpura nephritis: a systematic review

Bibliographic details: Xiong JC, Tian ML, He ZX, Wu HM, Hu ZX.  Efficacy and safety of mycophenolate mofetil for Henoch-Schonlein purpura nephritis: a systematic review. Chinese Journal of Evidence-Based Medicine 2014; 14(2): 184-190 Available from: http://www.cjebm.org.cn/en/oa/DArticle.aspx?type=view&id=20140216

Meta-analysis of the association between angiotensin-converting enzyme I/D polymorphism and susceptibility to children with Henoch-Schonlein purpura or Henoch-Scholein purpura nephritis

OBJECTIVE: To explore the relationship between angiotension-converting enzyme (ACE) I/D polymorphism and susceptibility to Henoch-Schönlein purpura or Henoch-Schönlein purpura nephritis (HSP/HSPN) especially among Chinese children.

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Summaries for consumers

Interventions for preventing and treating kidney disease in Henoch‐Schönlein Purpura

Henoch‐Schönlein Purpura (HSP) causes inflammation of small blood vessels in children and affects approximately 20/100,000 children annually. Symptoms and signs include a purpuric skin rash (which comprises small spots and larger bruises), abdominal pain, gastrointestinal bleeding, joint pain and swelling, facial swelling and evidence of kidney disease with blood and protein in the urine. Kidney disease occurs in about one third of children with HSP. In the majority this is mild (small amounts of blood in the urine only) and resolves completely but a few children have persistent kidney disease that can progress to kidney failure.

TPO receptor agonists for treating chronic idiopathic thrombocytopenic purpura

Chronic idiopathic thrombocytopenic purpura (ITP) is an acquired autoimmune disorder characterized by low platelet counts. To date, the therapies that primarily aim to reduce platelet destruction, such as corticosteroids, intravenous immunoglobulins and splenectomy, have been the mainstay of treatment in ITP. However, TPO receptor agonists such as romiplostim and eltrombopag, which aim to enhance platelet production, are novel drugs that have been suggested to be more effective. This review included six trials with 808 patients and compared TPO receptor agonists with placebo or standard of care (SOC).

Drug therapy for treating idiopathic thrombocytopenic purpura during pregnancy

Idiopathic thrombocytopenic purpura (ITP) is an immune‐mediated hematologic disorder caused by a low blood platelet count (thrombocytopenia). Antiplatelet antibodies act against the platelets resulting in platelet destruction by the spleen. In adults, the clinical features of ITP often have an insidious onset and are highly variable, ranging from no symptoms, mild bruising, to mucosal bleeding, and skin discolorations. Management of ITP during pregnancy is complex because of large differences between maternal and fetal platelet counts. The circulating antibodies can cross the placenta and cause a neonatal passive immune thrombocytopenia that may increase the risk of cerebral haemorrhage in the newborn infant. For this reason, it seems reasonable that cesarean section delivery is safer for the infant than vaginal delivery yet the mode of delivery may not affect the rate of haemorrhage. Many different pharmacological interventions are used for treating this medical disorder and treatment for ITP in pregnant women is not standardised. Some of these drugs have potential side effects for pregnant women and some can cause fetal malformation.

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Terms to know

Blood Vessels
Tubes that carry blood to and from all parts of the body. The three main types of blood vessels are arteries, capillaries, and veins.
Dialysis
The process of filtering the blood when the kidneys are not able to cleanse it.
Kidney
One of a pair of organs in the abdomen. The kidneys remove waste and extra water from the blood (as urine) and help keep chemicals (such as sodium, potassium, and calcium) balanced in the body. The kidneys also make hormones that help control blood pressure and stimulate bone marrow to make red blood cells.
Purpura
Purpura is the appearance of red or purple discolorations on the skin that do not blanch on applying pressure. They are caused by bleeding underneath the skin.
Rash
Any change in the skin which affects its appearance or texture. A rash may be localized to one part of the body, or affect all the skin. Rashes may cause the skin to change color, itch, become warm, bumpy, dry, cracked or blistered, swell and may be painful.

More about Henoch-Schonlein Purpura

Photo of a child

Also called: Acute vascular purpura, Anaphylactoid purpura, Autoimmune purpura, Henoch's purpura, Henoch-Schoenlein vasculitis, Schoenlein-Henoch purpura

Other terms to know: See all 5
Blood Vessels, Dialysis, Kidney

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