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Review
. 2021 May 25;10(11):2310.
doi: 10.3390/jcm10112310.

IgA Vasculitis and IgA Nephropathy: Same Disease?

Evangeline Pillebout  1   2
Affiliations
Review

IgA Vasculitis and IgA Nephropathy: Same Disease?

Evangeline Pillebout. J Clin Med. .

Abstract

Many authors suggested that IgA Vasculitis (IgAV) and IgA Nephropathy (IgAN) would be two clinical manifestations of the same disease; in particular, that IgAV would be the systemic form of the IgAN. A limited number of studies have included sufficient children or adults with IgAN or IgAV (with or without nephropathy) and followed long enough to conclude on differences or similarities in terms of clinical, biological or histological presentation, physiopathology, genetics or prognosis. All therapeutic trials available on IgAN excluded patients with vasculitis. IgAV and IgAN could represent different extremities of a continuous spectrum of the same disease. Due to skin rash, patients with IgAV are diagnosed precociously. Conversely, because of the absence of any clinical signs, a renal biopsy is practiced for patients with an IgAN to confirm nephropathy at any time of the evolution of the disease, which could explain the frequent chronic lesions at diagnosis. Nevertheless, the question that remains unsolved is why do patients with IgAN not have skin lesions and some patients with IgAV not have nephropathy? Larger clinical studies are needed, including both diseases, with a common histological classification, and stratified on age and genetic background to assess renal prognosis and therapeutic strategies.

Keywords: IgA Nephropathy; IgA Vasculitis; adults; children; genetics; physiopathology; presentation; prognosis; treatment.

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Conflict of interest statement

No conflicts of interest to declare.

Figures

Figure 1
Figure 1
Mesangial and capillary wall IgA deposits (immunofluorescence staining for IgA, original magnification ×400).
Figure 2
Figure 2
IgAN and IgAV clinical presentation over time from childhood to older age [35]. Reprinted with permission from ref. [35]. Copyright 2000 Rights and Permissions of Elsevier.
Figure 3
Figure 3
Physiopathology mechanism.
See this image and copyright information in PMC

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