IgA vasculitis

Last updated: August 4, 2023

Summarytoggle arrow icon

IgA vasculitis (IgAV), previously referred to as Henoch-Schonlein purpura (HSP), is an acute immune complex-mediated small vessel vasculitis that most commonly occurs in children. Onset is often preceded by an upper respiratory tract or gastrointestinal infection; IgAV in adults may be idiopathic. Affected individuals typically develop palpable purpura, arthritis and/or arthralgia, and abdominal pain. Renal involvement (i.e., IgAV nephritis) is more common and usually more severe in adults than in children, typically manifesting with hematuria. While IgAV is a clinical diagnosis, laboratory studies are used to identify organ involvement and exclude differential diagnoses, and skin biopsy can confirm the diagnosis in patients with atypical presentations. IgAV is usually self-limited; treatment is generally supportive. Systemic glucocorticoids may be required depending on severity of manifestations (e.g., in IgAV nephritis, orchitis). IgAV has an excellent prognosis in children, usually resolving within one month when not complicated by IgAV nephritis. Adults often manifest with more severe features and have lower remission rates. All patients require follow-up assessments to rule out the development of chronic renal disease.

Epidemiologytoggle arrow icon

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

Epidemiological data refers to the US, unless otherwise specified.

Etiologytoggle arrow icon

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

Pathophysiologytoggle arrow icon

Clinical featurestoggle arrow icon

An upper respiratory tract infection often precedes symptom onset by 1–3 weeks. [3]

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

IgAV is an important differential diagnosis to consider in children with a limp.

IgAV is much less common but typically more severe (e.g., involving hemorrhagic or necrotic skin lesions, IgAV nephritis) in adults than in children. [8]

Diagnosticstoggle arrow icon

Approach [5][7][9][10]

Suspect IgA vasculitis in any patient with palpable purpura, arthralgia, and abdominal pain. [7]

Consider the differential diagnosis for palpable purpura (e.g., small-vessel vasculitides, coagulopathies).

Laboratory studies [5][7][9]

Baseline laboratory studies

Obtain the following studies in all patients to assess for renal and GI involvement:

Consider an alternative diagnosis if coagulopathy and thrombocytopenia are present. [8]

Additional laboratory studies

The following studies may support the diagnosis of IgAV but are not required in the diagnostic workup.

Imaging [5][9]

Imaging studies should be obtained according to the patient's symptoms and/or suspected complications.

Intussusception is the most common complication of IgAV requiring surgery in children. [9]

Biopsy [8][9]

Histology from the skin and/or kidney confirms the diagnosis of IgAV. [8]

EULAR/PRINTO/PReS classification criteria [15][16]

Classification criteria can be used to distinguish IgAV from other types of vasculitides, but should not be used as diagnostic criteria for IgAV. [9][15]

Differential diagnosestoggle arrow icon

Differential diagnosis of IgAV based on clinical features
Clinical feature of IgAV Differential diagnosis Distinguishing features of the differential diagnosis
Purpura [17]
  • 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.

Treatmenttoggle arrow icon

Approach [9][10][11]

Decisions about pharmacotherapy are based on symptom severity and should be guided by a specialist.

Most cases of IgAV are self-limited and only require supportive care.

Avoid NSAIDs in patients with IgAV nephritis or GI bleeding. [9]

Systemic glucocorticoids [5][9]

Rule out intussusception before starting systemic glucocorticoids in patients with severe abdominal pain. [5]

Specialist consultation [5][9]

Consider early consultation as follows:

Disposition [19]

Complicationstoggle arrow icon

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

Follow-uptoggle arrow icon

Patients with IgAV require follow-up due to an increased risk of chronic kidney disease. [5][9][21]

  • Monitoring
  • Frequency
    • Patients with proteinuria or hypertension: every 2 weeks for 1 month, then every 1–2 months for 6–12 months, then every 3–6 months
    • Patients without proteinuria or hypertension: every 2 weeks for 1 month, then every 2–3 months for 6–12 months, then annually
    • Repeat if the patient develops AKI or a nonrenal flare (e.g., cutaneous lesions, GI symptoms)
  • Refer to nephrology if there is:

IgAV nephritis typically develops within 6 months of IgAV symptom onset. [21]

Prognosistoggle arrow icon

  • IgAV usually resolves with full recovery. However, relapse is likely in patients with previous renal involvement.
  • The prognosis is worse in patients with nephrotic range proteinuria. In rare cases (∼ 1%), ESRD may occur.

Referencestoggle arrow icon

  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. Kelly BG, Stratton DB, Mansour I, Tanriover B, Culpepper KS, Curiel-Lewandrowski C. Navigating the initial diagnosis and management of adult IgA vasculitis: A review. JAAD Int. 2022; 8: p.71-78.doi: 10.1016/j.jdin.2022.05.004 . | Open in Read by QxMD
  6. Park J, Berard RA, Grimmer J, Kirpalani A. IgA Vasculitis: a Review and Update on the Management of Renal and Extrarenal Disease, Highlighting What’s New for Biomarkers and Treatment. Curr. Pediatr. Rep. 2021; 9 (4): p.118-126.doi: 10.1007/s40124-021-00247-8 . | Open in Read by QxMD
  7. Reamy BV, Servey JT, Williams PM. Henoch-Schönlein Purpura (IgA Vasculitis): Rapid Evidence Review. Am Fam Physician. 2020; 102 (4): p.229-233.
  8. Pillebout E, Sunderkötter C. IgA vasculitis. Semin Immunopathol. 2021; 43 (5): p.729-738.doi: 10.1007/s00281-021-00874-9 . | Open in Read by QxMD
  9. Ozen S, Marks SD, Brogan P, et al. European consensus-based recommendations for diagnosis and treatment of immunoglobulin A vasculitis—the SHARE initiative. Rheumatology. 2019; 58 (9): p.1607-1616.doi: 10.1093/rheumatology/kez041 . | Open in Read by QxMD
  10. Yaseen K, Herlitz LC, Villa-Forte A. IgA Vasculitis in Adults: a Rare yet Challenging Disease. Curr Rheumatol Rep. 2021; 23 (7).doi: 10.1007/s11926-021-01013-x . | Open in Read by QxMD
  11. Walls R, Hockberger R, Gausche-Hill M, Erickson TB, Wilcox SR. Rosen's Emergency Medicine 10th edition- Concepts and Clinical Practice E-Book. Elsevier Health Sciences ; 2022
  12. Podjasek J, Wetter D, Pittelkow M, Wada D. Henoch-Schönlein Purpura Associated With Solid-organ Malignancies: Three Case Reports and a Literature Review. Acta Derm Venereol. 2012; 92 (4): p.388-392.doi: 10.2340/00015555-1288 . | Open in Read by QxMD
  13. Lin Q, Min Y, Li Y, et al. Henoch–Schönlein purpura with hypocomplementemia. Pediatr Nephrol. 2012; 27 (5): p.801-806.doi: 10.1007/s00467-011-2070-z . | Open in Read by QxMD
  14. Rovin BH, Adler SG, Barratt J, et al. KDIGO 2021 Clinical Practice Guideline for the Management of Glomerular Diseases. Kidney Int. 2021; 100 (4): p.S1-S276.doi: 10.1016/j.kint.2021.05.021 . | Open in Read by QxMD
  15. Ozen S, Pistorio A, Iusan SM, et al. EULAR/PRINTO/PRES criteria for Henoch-Schonlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria. Ann Rheum Dis. 2010; 69 (5): p.798-806.doi: 10.1136/ard.2009.116657 . | Open in Read by QxMD
  16. Hočevar A, Rotar Z, Jurčić V, et al. IgA vasculitis in adults: the performance of the EULAR/PRINTO/PRES classification criteria in adults. Arthritis Res Ther. 2016; 18 (1).doi: 10.1186/s13075-016-0959-4 . | Open in Read by QxMD
  17. Dedeoglu F, Kim S. IgA Vasculitis (Henoch-Schönlein Purpura): Clinical Manifestations and Diagnosis. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. Last updated: December 15, 2015. Accessed: April 17, 2017.
  18. Rasmussen C, Tisseyre M, Garon-Czmil J, et al. Drug-induced IgA vasculitis in children and adults: Revisiting drug causality using a dual pharmacovigilance-based approach. Autoimmun Rev. 2021; 20 (1): p.102707.doi: 10.1016/j.autrev.2020.102707 . | Open in Read by QxMD
  19. Masarweh K, Horovitz Y, Avital A, Spiegel R. Establishing hospital admission criteria of pediatric Henoch–Schonlein purpura. Rheumatol Int. 2014; 34 (11): p.1497-1503.doi: 10.1007/s00296-014-2971-9 . | Open in Read by QxMD
  20. Watson L, Richardson ARW, Holt RCL, Jones CA, Beresford MW. Henoch Schonlein Purpura – A 5-Year Review and Proposed Pathway. PLoS ONE. 2012; 7 (1): p.e29512.doi: 10.1371/journal.pone.0029512 . | Open in Read by QxMD
  21. Sharma A. Textbook of Systemic Vasculitis. Jaypee Brothers Medical Publishers ; 2015

Icon of a lock3 free articles remaining

You have 3 free member-only articles left this month. Sign up and get unlimited access.
 Evidence-based content, created and peer-reviewed by physicians. Read the disclaimer