IgA Vasculitis: Genetics and Clinical and Therapeutic Management

  • Miguel A. González-Gay
  • Raquel López-Mejías
  • Trinitario Pina
  • Ricardo Blanco
  • Santos Castañeda
Vasculitis (L Espinoza, Section Editor)
Part of the following topical collections:
  1. Topical Collection on Vasculitis


Purpose of Review

The purpose of the study is to perform an update on the current knowledge on genetics, clinical manifestations, and therapy in immunoglobulin A vasculitis (IgAV) (Henoch-Schönlein purpura).

Recent Findings

A strong genetic predisposition in individuals with IgAV was confirmed. It was due to the association with the HLA class II region that in people of European background is mainly related to HLA-DRB1*01 allele. Recent reports support the claim that kidney disease is more common in adults than in children with IgAV. The clinical spectrum and outcome of adults with IgAV depends on the age of onset. Relapses are not uncommon in IgAV. The presence of renal impairment or proteinuria excretion exceeding 1 g/24 h at the time of disease diagnosis and the degree of renal damage on the kidney biopsy are the best predictors of end-stage renal failure in adults with IgAV. The levels of urinary IgA at the onset of the disease may predict a poor renal outcome. The use of prednisone does not seem to prevent persistent kidney disease in children with IgAV. No additional benefit of adding cyclophosphamide to glucocorticoids in adults with IgAV was found. Rituximab seems to be a promising therapy in the management of adults with IgAV.


In this overview, we focus on the genetics, clinical manifestations, and therapy of IgA vasculitis, emphasizing the main differences in the clinical expression of the disease between children and adults.


IgA vasculitis Henoch-Schönlein purpura Genetics Clinical manifestations Treatment 


Compliance with Ethical Standards

Conflict of Interest

The authors declare that they have no conflict of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.


Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  • Miguel A. González-Gay
    • 1
    • 2
    • 3
  • Raquel López-Mejías
    • 2
  • Trinitario Pina
    • 2
  • Ricardo Blanco
    • 2
  • Santos Castañeda
    • 4
  1. 1.University of CantabriaSantanderSpain
  2. 2.Epidemiology, Genetics and Atherosclerosis Research Group on Systemic Inflammatory Diseases, Rheumatology Division, IDIVALHospital Universitario Marqués de ValdecillaSantanderSpain
  3. 3.Cardiovascular Pathophysiology and Genomics Research Unit, School of Physiology, Faculty of Health SciencesUniversity of the WitwatersrandJohannesburgSouth Africa
  4. 4.Rheumatology Division, Hospital de La Princesa, IIS-PrincesaUniversidad Autónoma de Madrid (UAM)MadridSpain

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