Henoch-Schonlein Purpura


 HSPchild_0.pngCassio Lynm, MA in JAMA, February 15, 2012 – Vol 307, No. 7.


Henoch-Schonlein purpura is an IgA vasculitis characterized by a tetrad of:

  • Palpable purpura
  • Arthritis/arthralgia
  • Abdominal pain
  • Renal disease


  • HSP is primarily a disease of childhood with male predominance.
  • It often occurs after an upper respiratory infection, which is perhaps why it is often seen in fall, winter, and spring but less commonly in the summer months.


  • HSP is characterized by deposition of IgA in small vessels.
  • Immunofluorescence shows deposition of IgA, C3, and fibrin within walls of affected vessels. 

Clinical manifestations


  • Palpable purpura is commonly a presenting sign.
    • Usually begins as erythematous, macular, or urticarial wheals.
    • Wheals coalesce into petechiae and palpable purpura
    • Generally found in dependent areas like legs and buttocks. 

Typical (severe) palpable purpura of HSP in 13 year old girl with renal involvement palpableHSP.pnghttp://www.vasculitis.org.uk/living-with-vasculitis/vasculitis-in-children



  • Occurs in a majority of patients
  • Joint pain and swelling is often transient, migratory, and involves fewer than 4 joints.
    • Generally the large joints of the lower or upper extremities.  


  • Nausea, vomiting, abdominal pain, and transient paralytic ileus are the more common manifestations.
  • The major serious complication of HSP is intussusception
    • Incidence of about 3%
  • Other GI manifestations such as GI hemorrhage, bowel ischemia and necrosis, and bowel perforation are less commonly reported outcomes.


  • Hematuria with minimal proteinuria is the most common renal manifestation
  • Nephrotic range proteinuria, elevated serum creatinine, and hypertension are less common.
  • Hypertension may also be seen.

Other organs

  • Involvement of the scrotum, central or peripheral nervous system, and lung involvement are much less common but have been seen.


Lab findings and studies

  • Elevated serum IgA levels
  • Normochromic anemia
    • May be seen with GI bleeding
  • Markers of inflammation (leukocytosis, ESR)
    • Usually elevated when a bacterial URI preceded the condition
  • Biopsy of organs involved would show IgA deposition.


hsphisto_0.png Clinical Journal of the American Society of Nephrology http://cjasn.asnjournals.org/content/2/5/1054.long



  • The diagnosis of HSP is clinical based on symptoms and time course.
  • The differential diagnosis includes:
    • Microscopic polyarteritis (MPA)
    • Granulomatosis with polyangiitis (GPA/Wegener's granulomatosis) 
    • Systemic lupus erythematosus
    • Presence of c-ANCA (GPA) and p-ANCA (MPA) can help distinguish between HSP and the other vasculitities.
    • Abdominal symptoms without characteristic rash can make diagnosis difficult.



  • HSP is self-limiting, so treatment is generally symptomatic.
  • Steroids have not been shown to change the course of the disease
    • Can shorten the duration of joint and abdominal pain.
  • Historically, the evidence on the use of steroids in preventing renal disease has been mixed
    • A recent Cochrane Review suggests that steroids are not helpful in preventing future renal disease.
  • Indications for hospitalization include:
    • Inability to take PO water (hydration)
    • Severe intractable abdominal pain
    • Overt GI bleeding
    • Joint involvement that inhibits ability to walk
    • Renal insufficiency.
  • Other tests that may be required include an abdominal ultrasound to monitor for intussusception.



  • The symptoms generally last 3-12 weeks
    • Symptoms usually have a waxing and waning course with each episode less severe than the previous.
  • Follow-up for 3-6 months after the onset of disease is recommended in order to monitor for renal complications.
    • Repeat urinalyses and serum creatinine. 
    • 97% of those who develop renal complications do so within the first 6 months.
  • The long term morbidity and mortality of HSP is generally low, especially in children.
  • Most of the morbidity is related to renal complications, particularly renal failure. 



  1. Hahn D, Hodson EM, Willis NS, Craig JC. Interventions for preventing and treating kidney disease in Henoch-Schonlein Purpura (HSP). status and date: New search for studies and content updated (conclusions changed), published in. 2015;(8). http://onlinelibrary.wiley.com/doi/10.1002/14651858.CD005128.pub3/epdf/s.... Accessed December 20, 2015.
  2. Henoch-Schönlein purpura (immunoglobulin A vasculitis): Clinical manifestations and diagnosis. http://www.uptodate.com.proxy.uchicago.edu/contents/henoch-schonlein-pur.... Accessed December 20, 2015.
  3. Henoch-Schönlein purpura (immunoglobulin A vasculitis): Management. http://www.uptodate.com.proxy.uchicago.edu/contents/henoch-schonlein-pur.... Accessed December 20, 2015.
  4. Idiopathic IgA Nephropathy: Pathogenesis, Histopathology, and Therapeutic Options. http://cjasn.asnjournals.org/content/2/5/1054.long. Accessed December 20, 2015.
  5. Punnoose AR, Lynm C, Golub RM. Henoch-Schönlein Purpura. JAMA. 2012;307(7):742-742.
  6. Tizard EJ. Henoch-Schönlein purpura. Archives of disease in childhood. 1999;80(4):380-383.

Back to Table of Contents