Renal manifestations of Henoch-Schönlein purpura (IgA vasculitis)
- Patrick Niaudet, MD
Patrick Niaudet, MD
- Section Editor — Pediatric Nephrology
- Professor of Pediatrics
- Hôpital Necker-Enfants Malades, Paris, France
- Gerald B Appel, MD
Gerald B Appel, MD
- Section Editor — Glomerular Diseases
- Professor of Medicine
- Columbia University College of Physicians and Surgeons
- Gene G Hunder, MD
Gene G Hunder, MD
- Section Editor — Vasculitis
- Emeritus Consultant
- Professor Emeritus
- Mayo Clinic College of Medicine
- Section Editors
- Richard J Glassock, MD, MACP
Richard J Glassock, MD, MACP
- Editor-in-Chief — Nephrology
- Section Editor — Glomerular Diseases
- Emeritus Professor
- The David Geffen School of Medicine at UCLA
- Fernando C Fervenza, MD, PhD
Fernando C Fervenza, MD, PhD
- Section Editor — Glomerular Diseases
- Professor of Medicine
- Mayo Clinic College of Medicine
Henoch-Schönlein purpura (HSP), also termed IgA vasculitis (IgAV), is a systemic vasculitis with a prominent cutaneous component. Cutaneous manifestations similar to those of HSP (IgAV) are also seen in several other disorders, including mixed (IgG/IgM) cryoglobulinemia, anti-neutrophil cytoplasmic autoantibody (ANCA)-associated small vessel vasculitis, and hypersensitivity vasculitis (which includes what had been called serum sickness) [1,2]. Skin vasculitis with palpable petechiae or purpura is typically a major finding in these disorders (picture 1). A general overview of the different vasculitic disorders is discussed elsewhere. (See "Overview of cutaneous small vessel vasculitis" and "Overview of and approach to the vasculitides in adults".)
HSP (IgAV) is characterized by the tissue deposition of IgA-containing immune complexes . The histologic findings in the kidney are identical to those in IgA nephropathy, suggesting that the two disorders may have a similar pathogenesis. Likewise, the finding of high circulating levels of galactose-deficient IgA1 in patients with both IgA nephropathy and HSP (IgAV) suggest a common underlying pathogenetic mechanism . The observation of the simultaneous occurrence of HSP (IgAV) and IgA nephropathy in twins after an adenovirus infection is further evidence in support of a common pathogenesis . (See "Pathogenesis of IgA nephropathy".)
IgA deposition is prominent in both HSP (IgAV) and IgA nephropathy (picture 2), but the renal injury may be mediated at least in part by IgG autoantibodies directed against mesangial cell antigens [4,5]. The course of the renal disease and circulating antibody titers are roughly parallel, and these autoantibodies do not appear to be present in patients with HSP (IgAV) who do not have renal involvement .
HSP (IgAV) occurs more often in children than in adults, but renal involvement is more likely to occur and to be more severe, in older children and in adults, prompting more aggressive therapy [6-10]. The renal manifestations of HSP (IgAV), including prognosis and treatment, will be reviewed here. The clinical features, pathogenesis, diagnosis and management of HSP (IgAV) in general are discussed separately. (See "Henoch-Schönlein purpura (immunoglobulin A vasculitis): Clinical manifestations and diagnosis" and "Henoch-Schönlein purpura (immunoglobulin A vasculitis): Management".)
The classic tetrad of Henoch-Schönlein purpura (HSP), also termed IgA vasculitis (IgAV), is rash, arthralgias, abdominal pain, and renal disease, which can develop in any order and at any time over a period of several days to several weeks [7,8,11,12]. Renal involvement is typically noted within a few days to one month after the onset of systemic symptoms, but is not predictably related to the severity of extrarenal involvement. In a prospective study of 223 children under 16 years of age, the presence of abdominal pain but not melena or joint symptoms conferred a significant increase in the risk for nephritis (odds ratio 2.1, 95% CI 1.1-3.7) . (See "Henoch-Schönlein purpura (immunoglobulin A vasculitis): Clinical manifestations and diagnosis".)
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