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IgA vasculitis

Last updated: April 28, 2021

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IgA vasculitis (IgAV), formerly known as Henoch-Schonlein purpura (HSP), is an acute immune complex-mediated small vessel vasculitis that most commonly occurs in children. It is often preceded by an upper respiratory tract infection and typically presents with a tetrad of symptoms: palpable purpura, arthritis/arthralgia, abdominal pain, and renal disease. However, in any individual patient, only some of the classic tetrad of symptoms may be present. IgAV is a clinical diagnosis, but in particularly unclear or atypical cases a biopsy may be used to confirm the diagnosis. Because the disease course is usually self-limiting, treatment is generally supportive. Severe cases may require glucocorticoids, antihypertensive drugs, and possibly dialysis. IgAV has an excellent prognosis, usually resolving within one month when not complicated by significant renal disease.

  • Sex: >
  • Age: more common in children
    • 90% of cases < 10 years [1]
    • Peak incidence: 6 years [2]

Epidemiological data refers to the US, unless otherwise specified.

The exact pathogenesis is unknown and assumed to be multifactorial. Factors that likely play a role include:

History

  • Symptom onset often 1–3 weeks after an infection, typically affecting the upper respiratory tract [3]

Manifestations [1][5]

IgAV is characterized by a tetrad of clinical features: palpable purpura, arthritis/arthralgia, GI symptoms, and renal disease. IgAV is one of the important differential diagnoses to consider in cases of pediatric limp.

IgAV is characterized by PAPAH: purpura, abdominal pain, arthritis/arthralgia, and hematuria.

IgAV is one of the important differential diagnoses to consider in pediatric limp.

IgAV is a clinical diagnosis; , laboratory tests are not essential to IgAV diagnosis. However, they may be useful for excluding differential diagnoses in patients exhibiting only one or two of the IgAV tetrad of symptoms, as is often the case in the first few days. Laboratory tests are also useful for monitoring the extent of renal involvement, which helps determine the prognosis. Definitive diagnosis in uncertain cases is made via biopsy. [1][6]

Laboratory tests

The platelet count in IgAV is normal or elevated, as opposed to other causes of purpura.

Imaging

  • Indication: performed in patients with marked abdominal symptoms or suspected complications
  • Modalities: abdominal ultrasound/CT

Biopsy

Clinical feature of IgAV Differential diagnosis Distinguishing feature
Purpura [5]
Arthritis/arthralgia
  • Arthritis typically in a single joint
  • No rash, abdominal pain, or renal symptoms
Renal disease
  • No rash, joint, or GI symptoms
  • Primarily affects (young) adults

IgAV is a unique cause of purpura without thrombocytopenia.

The differential diagnoses listed here are not exhaustive.

Most cases of IgAV are self-limiting and only require supportive care (e.g., pain management) with regular outpatient follow-up; . Severe IgAV requires hospitalization and intensive medical therapy.

Mild disease

  • Outpatient treatment
  • Usually no treatment necessary
  • NSAIDs for pain management, rest, and adequate hydration
  • Discontinuation of suspected precipitating drug, if applicable

Severe disease [7]

We list the most important complications. The selection is not exhaustive.

  • All patients
    • Blood pressure monitoring and urinalysis
    • Timing
      • Symptomatic patients: weekly or biweekly testing
      • After symptoms subside: monthly testing for the first 6 months, then every other month for an additional 6 months
      • Symptom-free for > 12 months: perform tests at subsequent well-child visits
  • In patients with abnormal blood pressure or urinalysis
  1. Reamy BV, Williams PM, Lindsay TJ. Henoch-Schönlein purpura. Am Fam Physician. 2009; 80 (7): p.697-704.
  2. Gardner-Medwin JM, Dolezalova P, Cummins C, Southwood TR. Incidence of Henoch-Schönlein purpura, Kawasaki disease, and rare vasculitides in children of different ethnic origins.. The Lancet. 2002 .
  3. Lenis M. González MD Camila Krysicka Janniger MD Robert A. Schwartz MD, MPH. Pediatric Henoch–Schönlein purpura. International Journal of Dermatology. 2009 .
  4. Saulsbury FT. Epidemiology of Henoch-Schönlein purpura.. Cleveland Clinic journal of medicine. 2002; 69 Suppl 2 : p.SII87-9. doi: 10.3949/ccjm.69.suppl_2.sii87 . | Open in Read by QxMD
  5. Dedeoglu F, Kim S. IgA Vasculitis (Henoch-Schönlein Purpura): Clinical Manifestations and Diagnosis. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/henoch-schonlein-purpura-immunoglobulin-a-vasculitis-clinical-manifestations-and-diagnosis.Last updated: December 15, 2015. Accessed: April 17, 2017.
  6. Harber M. Practical Nephrology. Springer ; 2014
  7. Dedeoglu F, Kim S. IgA Vasculitis (Henoch-Schönlein Purpura): Management. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/henoch-schonlein-purpura-immunoglobulin-a-vasculitis-management.Last updated: November 3, 2016. Accessed: April 17, 2017.
  8. Niaudet P, Appel GB, Hunder GG. IgA vasculitis (Henoch-Schönlein purpura): Renal manifestations. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/iga-vasculitis-henoch-schonlein-purpura-renal-manifestations.Last updated: May 14, 2018. Accessed: March 22, 2019.
  9. Sharma A. Textbook of Systemic Vasculitis. Jaypee Brothers Medical Publishers ; 2015