Predominately small vessel

Henoch-Schönlein purpura (HSP)

  • What is it?
    • Henoch-Schönlein purpura (HSP), also known as IgA vasculitis, is the most common form of systemic vasculitis in children. This disease involves vasculitis of the small blood vessels (swelling of these vessel walls) around the body, and leads to effects across a variety of organs. The most commonly affected organs include the skin, joints, intestines/bowels and kidneys.
    • In the term HSP, “Henoch-Schönlein” describes the last names of the two individuals that discovered and described this disease. The second part of the term, “purpura”, describes one of the key features of this disease. Purpura describes purplish-red spots which form on the body caused by small blood vessels rupturing. This rupturing causes the blood to leak and pool underneath the skin. In general, this does not cause any significant harm to the body. However, it does often reveal underlying serious medical conditions. In HSP, the purpura is often also described as “palpable purpura”, which means that these spots can actually be felt, though this is not a necessary factor.
    • The most serious cases of patients with HSP often involve inflammation of the blood vessels in the kidney. This can lead to HSP (IgAV) Nephritis, which is often milder in children when compared to adolescents and adults.
  • How does it happen?
    • IgA vasculitis (HSP) is believed to involve the abnormal action of the immune system, which involves specific immune system proteins called IgA (immunoglobulin A) that function as antibodies. In this disease, IgA complexes collect on blood vessel walls and recruit new immune cells to come and accumulate in this area. This can occur in the skin, joints, intestines, kidneys and maybe even the testes and central nervous system (brain and spinal cord). This can lead to blood vessels leaking or even bursting, leading to the main feature of the disease, palpable purpura.
  • Though the true causes of HSP are currently unknown, certain factors have been discussed in studies with HSP patients. For example, infections caused by viruses of bacteria have been discussed as a possible cause because of HSP appearing after upper respiratory tract infections (of the nose, mouth, throat, voice box etc.). Also, the role of immune system proteins/antibodies like IgA suggests that HSP could be related to abnormal functioning of the child’s immune system.
  • HSP is the most common form of Systemic Vasculitis in children, though still a rare disease in general. HSP is not contagious, cannot be inherited and cannot be prevented. It has been found that anywhere from 8-20 new cases per 100,000 children are discovered each year. In some studies, it has been found that HSP affects boys almost twice as often as it affects girls. Around 90% of all cases are found in children under the age of 10. This explains why HSP is around 100 times more likely in children than in adults. It mainly affects children between the ages of 3-8 years old, with an average age of 6.  No preferences for race or ethnicity have been found, though a study has noted that African-Americans could be affected less. This disease is also most commonly seen in the winter months in Europe and the Northern Hemisphere, though it can be seen in the other seasons as well.
  • Children can be diagnosed with HSP using mainly clinical features such as palpable purpura. Other diagnostic tests can also be used to confirm this diagnosis.
  • Click here for more detailed information on classifying and diagnosing HSP.
  • Patients with Henoch-Schonlein purpura recover quite well on average, and do so with a treatment plan consisting adequate hydration, rest and drugs such as Aspirin and Tylenol.
    • Click here for more detailed information on suggested treatment protocols.

Sources:

Bluman, J., & Goldman, R. D. (2014). Henoch-Schönlein purpura in children Limited benefit of corticosteroids. Canadian Family Physician, 60(11), 1007-1010.

Davin, J. C., & Coppo, R. (2014). Henoch-Schonlein purpura nephritis in children. Nature Reviews Nephrology, 10(10), 563-573.

Dedeoglu, F. & Kim S., Henoch-Schönlein purpura (immunoglobulin A vasculitis): Clinical manifestations and diagnosis. In: UpToDate,TePas E. (Ed), UpToDate. Retrieved from https://www.uptodate.com/contents/henoch-schonlein-purpura-immunoglobulin-a-vasculitis-clinical-manifestations-and-diagnosis  (Accessed on September 22, 2016.)

González, L. M., Janniger, C. K., & Schwartz, R. A. (2009). Pediatric Henoch–Schönlein purpura. International journal of dermatology, 48(11), 1157-1165.

Hung, S. P., Yang, Y. H., Lin, Y. T., Wang, L. C., Lee, J. H., & Chiang, B. L. (2009). Clinical manifestations and outcomes of Henoch-Schönlein purpura: comparison between adults and children. Pediatrics & Neonatology, 50(4), 162-168.

Johnson, E. F., Lehman, J. S., Wetter, D. A., Lohse, C. M., & Tollefson, M. M. (2015). Henoch–Schönlein purpura and systemic disease in children: retrospective study of clinical findings, histopathology and direct immunofluorescence in 34 paediatric patients. British Journal of Dermatology, 172(5), 1358-1363.

Sohagia, A. B., Gunturu, S. G., Tong, T. R., & Hertan, H. I. (2010). Henoch-Schonlein purpura—a case report and review of the literature.Gastroenterology research and practice, 2010.

    • According to the EULAR/PRINTO/PRES criteria for classification of HSP, a patient must present with purpura in the lower limbs mostly, without thrombocytopenia (low levels of specific immune cells) and without coagulopathy (weaker ability to form clots). The patient must also have at least 1 of the following 4 features:
    1. Abdominal pain
    2. Arthritis or arthralgia
    3. Renal involvement (proteinuria, hematuria)
    4. Leukocytoclastic vasculitis or proliferative glomerulonephritis, with predominant immunoglobulin A (IgA) deposition

     

    Diagnosis:

    In order to reach a diagnosis, it is important to consider a combination of clinical features, laboratory tests, along with biopsy results. For more details view the diagnostic algorithm for HSP.

  • The most common symptoms displayed by HSP patients are discussed below. The main system involved is the skin, followed by the involvement of the joints, intestinal tract and the kidneys. Other organ systems can also be affected during HSP, however those cases are rare. These symptoms usually appear over the course of days to weeks, and can appear in any order (though purpura and joint pain appear first in most cases).
    • Skin
      • The classic rash of HSP (i.e. purpura) is initially presented in about three-quarters of children with HSP. A lack of this sign often makes it more difficult to diagnose a patient with HSP.
      • Rash
        • The rash is symmetrically distributed and often in areas such as the legs and the buttocks (mainly in toddlers). In children with decreased mobility, it can also be seen on the face, arms, and the trunk of the body.
        • Note that skin lesions (damage to the skin) can also begin to form in these areas.
        • It often begins with the presentation of wheals (round, pale red raised bubbles on the skin)
          • These wheals can be erythematous (redness of the skin), macular (appear in spots/blotches), or utricarial (hives-like).
        • These wheals then come together and turn into palpable purpura, ecchymoses and petechiae.
          • Palpable purpura
            • Main symptom
            • Involves purple-red spots which can be distinctly felt, and do not disappear when pressure is applied (non-blanching). This is unique because these spots are raised and can be touched.
          • Ecchymoses
            • The changing of the color of the skin, often because of bleeding underneath (commonly seen because of bruising)
          • Petechiae
            • Bleeding in the skin that leads to small red or purple spots (not large like palpable purpura so cannot be felt)
          • Edema (Swelling)
            • Swelling is common feature that is seen in younger children (less than 3 years old).
            • It is seen in dependent areas (like the ankles) and periorbital areas (on and around the eyelids)
          • Joints
            • Arthritis (inflammation/swelling in a joint) and/or arthralgia (pain in a joint) occur in up to 84% of patients with HSP.
            • Often seen in the knees, ankles and hips.
              • Less often, it is seen in the wrists, hands and elbows.
            • In a large majority of patients, this is not the presenting symptom (presenting symptom in 15% of patients)
            • This often leads to children not wanting to move around and be mobile
          • Gastrointestinal tract
            • Symptoms related to this system are seen in around 50% of patients with HSP, and can range from mild to severe findings
            • On average, these symptoms appear around 8 days after the rash. The diagnosis of HSP is more difficult when gastrointestinal symptoms occur before the rash.
            • Guaiac-positive stools (show the presence of blood) are seen in around 56% of patients
            • Endoscopies can reveals lesions in the small and large intestine
            • Intussusception is the most common and serious intestinal complication that can occurs in children with HSP, however it is quite rare (seen in around 2.5-3.5% of patients)
              • This involves a part of the intestine that slides into the next portion like a telescope. This leads to blockage of blood flow and causes parts of the intestine tissue to die.
              • This usually requires surgery to correct
            • In a review conducted in Chicago, 42% of children with HSP reported feeling severe abdominal pain
          • Kidneys (Renal involvement)
            • Across many studies, 20-54% of children with HSP showed some sort of kidney involvement, with greater risk in older children (greater than 8 years old)
            • A large study involving 1133 children with HSP provided data regarding kidney involvement
              • Renal symptoms were found to appear within the first 4 weeks in 84% of patients, and within 6 months in 97% of all patients.
              • Hematuria (blood in urine) and/or proteinuria (protein in urine) was seen in 34% of patients
              • In patients with kidney disease, 79% had hematuria (blood in urine) and low levels of proteinuria (protein in urine).
                • 21% also showed high levels of proteinuria, high levels of urea in blood or serum creatinine, and/or hypertension.
              • Other organs
                • Scrotum
                  • Seen in around 2-38% of male patients with HSP. It is not usually the initial symptom of the disease, and can present itself as pain, tenderness and swelling of the testicle(s) and/or scrotum.
                • Respiratory tract
                  • In one study of French patients with HSP, 97% of patients were found to have impaired lung diffusion capacity. Also, 69% of these patients were found to have changes in the area around the lung sacs in the chest cavity. Severe lung problems are rare in HSP cases and are mainly seen in older patients (adults and adolescents).
                • Central Nervous System (brain and spinal cord) and Peripheral Nervous System (rest of body)
                  • Rare reports show certain conditions in children with HSP that include headaches, seizures, loss of control of bodily movements, escaped blood in the brain etc. Most of these features occur for a certain amount of a time and disappear later (not permanent). However, strokes can lead to more permanent effects.
                • Eyes
                  • Inflammation of a certain layer of the eye (uveitis), or inflammation of the cornea (keratitis), are rare features seen in HSP patients and most likely suggest some other diseases.
  • Diagnostic Purposes
    • No particular laboratory tests exist to help diagnose a patient with HSP
    • However, some tests can help in cases where HSP needs to be separated from other diseases
      • Some diseases also display purpura, but mainly due to thrombocytosis (higher levels of immune cells) or coagulopathy (reduced ability to form blood clots).
      • To point towards HSP, patients should have:
        • Normal platelet (immune cell) count
        • Normal coagulation results (normal prothrombin time)
      • Immunoglobulin A (IgA)
        • IgA proteins are a part of the immune system and function as antibodies
        • Serum immunoglobulin A levels have been found to be increased in children with HSP in around 50-70% of cases
        • These increases are related to the involvement of the kidneys
      • Additional Results
        • Blood test results in children with HSP show general effects that are not actually directly caused by HSP

 

  • HSP that occurs after bacterial infections often show:
    • Leukocytosis (higher levels of white blood cells)
    • Increased ESR
  • HSP that occurs after viral infections does not show these increases
  • Complement system
    • Hypocomplementemia
      • Lower level of proteins that are a part of the compliment system which helps the immune system
      • Seen in a significant portion of children who had a recent streptococcal (bacterial) infection
      • A study with 338 children with HSP found that 53% of children had lower levels of compliment C3 and/or compliment C4.
  • Imaging studies after often done with children with HSP in order to assess serious abdominal symptoms. Other tests can also be done to assess symptoms related to the scrotum of males.
  • Possible imaging tools used in assessing HSP:
    • Abdominal radiography
      • Helps show the loops of the bowel and whether there is reduced muscle movement in the intestines (decreased motility)
    • Abdominal ultrasonography
      • Can show thickness of the bowel walls, fluid in the abdominal cavity, swelling of blood clots (hematomas) and intussusception (“telescoping” of the intestines).
    • Doppler flow studies / Radionuclide scans
      • Helps to assess pain in the scrotum of males caused by HSP (might instead be caused by testicular torsion)
        • Normal blood flow to the testicles points towards HSP, while decreased blood flow reflects testicular torsion
  • The diagnosis of HSP is often done using mainly clinical features such as the clear sign of palpable purpura in the lower limbs and buttocks. However, when this is not obvious and a diagnosis is more difficult, then a biopsy of the skin or kidney can be done. These cases include where there is no rash, an unusual rash, or in cases of significant kidney disease.
  • Skin
    • Sample blood vessels in skin to make diagnosis of HSP
    • Use light microscopy to show vasculitis with IgA deposits that point towards HSP
  • Kidney
    • As mentioned before, this is done when the diagnosis of HSP is difficult or if severe kidney involvement is present
    • For HSP, the biopsy is done to reveal IgA deposition through microscopy

In this image, you can see the diagnostic algorithm for HSP by following the diagram from the “INDICATIONS FOR TESTING” box (at the top) to the middle-right box for “IgA vasculitis”.

Retrieved from: https://arupconsult.com/content/takayasu-arteritis/?tab=tab_item-1This is the website for ARUP Consult, whose disclaimer and policies outline that the “content at this website may be freely downloaded and used for non-commercial purposes.”

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Davin, J. C., & Coppo, R. (2014). Henoch-Schonlein purpura nephritis in children. Nature Reviews Nephrology,10(10), 563-573.

Davin, J. C., Ten Berge, I. J., & Weening, J. J. (2001). What is the difference between IgA nephropathy and Henoch-Schönlein purpura nephritis?. Kidney international,59(3), 823-834.

Dedeoglu, F. & Kim S., Henoch-Schönlein purpura (immunoglobulin A vasculitis): Clinical manifestations and diagnosis. In: UpToDate,TePas E. (Ed), UpToDate. Retrieved from https://www.uptodate.com/contents/henoch-schonlein-purpura-immunoglobulin-a-vasculitis-clinical-manifestations-and-diagnosis  (Accessed on September 22, 2016.)

Dedeoglu, F. & Kim S., Henoch-Schönlein purpura (immunoglobulin A vasculitis): Management. In: UpToDate,TePas E. (Ed), UpToDate. Retrieved from https://www.uptodate.com/contents/henoch-schonlein-purpura-immunoglobulin-a-vasculitis-management (Accessed on September 22, 2016.)

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General Initial Therapy

  • A vast majority of patients with HSP end up recovering on their own. The generally accepted treatment involves providing the patients with adequate hydration (water), rest, and certain treatments to relieve pain from particular symptoms. Swelling (edema) of the lower limbs, buttocks, and genital area is often reduced by raising the area with the rash and with lots of rest.
  • Certain treatments used to relieve pain are discussed below:
    • Acetaminophen (i.e. Tylenol) OR non-steroidal anti-inflammatory drugs (NSAIDs, i.e. Aspirin)
      • Used in the treatment of joint and abdominal pain
      • These drugs are used instead of glucocorticoids
        • Glucocorticoids (ex. Prednosione) have been suggested to relieve the rash, arthritis, scrotal pain and gastrointestinal pain in patients with HSP
        • However, glucocorticoids are not usually used in treatment of HSP because the data is very limited and there are significant side effects
      • It should be noted that patients that are experiencing intestinal problems may show less improvements with this treatment because of decreased absorption of these medications
      • Mild-to-moderate pain
        • The most common NSAIDs are Aspirin, ibuprofen and naproxen.
        • In the treatment of HSP, naproxen is often used.
          • Ibuprofen and other NSAIDs are found to be just as effective as naproxen
        • Glucocorticoids (ex. prednisone)
          • These drugs, such as prednisone, are only used in cases of severe pain
            • These cases include patients who have symptoms that significantly affect their oral intake, affect their ability to move and complete regular activities, or cause them to be hospitalized.
            • If oral medications cannot be taken by the patient, methylprednisolone can be used instead.
          • Note that these drugs help relieve the inflammation in the body, but do not actually affect the course of the disease.
          • Patients taking these drugs should be watched very closely, and the drugs should be lowered slowly over time (over around 4-8 weeks).
  • Specific treatment for kidney involvement in children should only be considered if higher levels of protein are seen in urine (proteinuria) and if kidney function is significantly affected during the initial episode of the disease.
  • There is no strong evidence as to which treatment for kidney involvement is most effective in HSP patients
  • One study found that high-dose methylprednisolone for 3 days, followed by 3 months of oral prednisone, might be beneficial for patients with severe kidney disease.
    • The main goal of this treatment is to reduce inflammation
  • A couple studies have also suggested that cyclosporine might be helpful in children with HSP and severe proteinuria (high amounts of protein in urine).
  • Some treatments have involved glucocorticoids and azathioprine, while others have used some multi-drug plans.
  • Lastly, in small groups of patients, intravenous immune globulin (IVIG) has been used successfully to treat patients with HSP (IgAV) nephritis (inflammation in the kidneys)
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  • Urinalysis and blood pressure monitoring
    • Patients diagnosed with HSP/IgA vasculitis should be monitored weekly or bi-weekly for their blood pressure and urinalyses should be done.
    • This is done for at least 2 months, and helps assess the degree of kidney involvement in patients with HSP
    • In general, the outcomes of children with HSP are great. On average, if a patient does not have significant kidney involvement, then the episode of HSP can be resolved in 1 month.
    • Two thirds of patients do not show any recurring episodes of this disease
    • The remaining one-third of patients show at least one additional episode within 4 months of the first episode
    • Patients who have a more severe form of HSP, have a greater risk of recurrent episodes of HSP
      • Also, long-term morbidity of patients with HSP is related to the degree of kidney involvement

Below you can find downloadable PDF files of suggested treatment protocols for patients with Henoch-Schönlein purpura (HSP)/IgA vasculitis. These suggested treatment protocols vary depending on the phase of the treatment (induction, maintenance, flare) and on which conditions are presented in patients.

Suggested Treatment Protocols:

  1. (These numbers depend on the types of treatment protocols provided for Henoch-Schönlein purpura (HSP)/ IgA vasculitis)
  2. (These numbers depend on the types of treatment protocols provided for Henoch-Schönlein purpura (HSP)/ IgA vasculitis)
  3. (These numbers depend on the types of treatment protocols provided for Henoch-Schönlein purpura (HSP)/ IgA vasculitis)

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