What are the commonalities between IgA nephropathy in Henoch-Schönlein purpura (IgA vasculitis)?

Updated: Jan 08, 2021
  • Author: Rajendra Bhimma, MBChB, MD, PhD, DCH (SA), FCP(Paeds)(SA), MMed(Natal); Chief Editor: Craig B Langman, MD  more...
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Answer

IgAV and IgA nephropathy appear to be related disorders. However, the precise relation between them requires further definition. The question has been raised as to whether IgAV and IgA nephropathy are two aspects of a single disease entity or two distinct entities. The following commonalities and differences have been noted:

  • IgA nephropathy almost exclusively involves young adults and typically affects only the kidneys, whereas IgAV affects mostly children and involves the skin and connective tissues, GI tract, joints, and scrotum, as well as the kidneys. [32, 33]

  • IgAV-associated nephritis is more benign in children than in adults, with the latter tending to show a chronic and relentless course similar to that of primary IgA nephropathy. [34, 35, 33]

  • ​In IgAV-associated nephritis, repeated and prolonged episodes of acute glomerular inflammation lead to fibrous scars and hyperfiltration in the remaining areas, resulting in chronic kidney disease that may progress to chronic kidney failure. Thus the number and severity of acute episodes of IgAV-associated nephritis have a crucial role in the subsequent progression and loss of kidney function. In IgA nephropathy, initiation and progression to chronic kidney failure occurs slowly and often is asymptomatic, with development of glomerulosclerosis and tubulointerstitial fibrosis. [36]
  • The occurrence of extrarenal manifestations in IgA nephropathy is similar to that in IgAV.

  • IgA nephropathy has developed in patients with a history of IgAV, and IgAV and IgA nephropathy have occurred in the same families; in a survey of 40 families in which 2 or more members had IgA nephropathy, 5 presented with IgAV. [37]

  • Patients with IgAV who undergo kidney transplantation develop IgA deposits in the graft.

  • The prevalence of both conditions is high in certain geographic areas.

  • Similar changes in the IgA system (ie, high IgA, IgA-1C, IgA1-IC, IgA-fibronectin aggregates, aberrantly glycosylated IgA in the circulation) occur in both diseases. [38, 39, 40]

  • Cystic changes in the ovaries of a prepubertal girl with IgAV have been recorded. [41]


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