IgA Vasculitis: Genetics and Clinical and Therapeutic Management

Autores de IDIVAL

• Miguel Ángel González-Gay Mantecón

  Autor

• 

  Raquel López Mejías

  Autor

• Trinitario Pina Murcia

  Autor

• 

  Ricardo Blanco Alonso

  Autor

Autores ajenos al IDIVAL

• Castañeda S

Unidades

• INMUNOPATOLOGÍA

  

  Investigador Corresponsable

  Ricardo Blanco Alonso

Abstract

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 Avasculitis (IgAV) (Henoch-Schonlein 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. Summary In this overview, we focus on the genetics, clinical manifestations, and therapy of IgAvasculitis, emphasizing the main differences in the clinical expression of the disease between children and adults.

Keywords

• IgAvasculitis; Henoch-Schonlein purpura; Genetics; Clinical manifestations; Treatment