Henoch-Schonlein Purpura

Introduction

 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

Epidemiology

  • 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.

Pathogenesis

  • 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

Skin

  • 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

 

Arthritis/arthralgia

  • 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.  

Gastrointestinal

  • 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.

Renal

  • 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

 

Diagnosis

  • 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.

 

Treatment

  • 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.

 

Prognosis

  • 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. 

 

References

  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