Updates on Henoch-Schonlein Purpura

Henoch-Schönlein purpura (HSP), currently also known as immunoglobulin A (IgA) vasculitis, is the most common vasculitis in children. It is a systemic autoimmune disease mediated by complexes containing abnormal IgA. The cause of HSP is not well known, but the disease is often triggered by an upper respiratory infection in individuals with genetic susceptibility. The diagnosis relies on internationally agreed criteria, including palpable cutaneous purpura of orthostatic location associated with at least one of the following findings: arthralgia/arthritis, gastrointestinal manifestations, leukocytoclastic vasculitis with IgA deposits and/or renal involvement. The skin lesions are essential for the diagnosis. The digestive symptoms, mostly severe abdominal pain, intestinal bleeding, and more rarely, intussusception, maybe the initial and most worrisome clinical component of HSP during the acute presentation of the disease. Nephropathy determines the long-term prognosis. The clinical course of HSP is, in general, favorable. Bed rest results in remission of the purpura that often recurs as the child restarts standing and walking. Corticosteroids are effective, although not usually required, to treat abdominal pain and other severe manifestations. No medical treatment can avoid the possibility of renal involvement that may occur for several months after resolution of the skin lesions. Corticosteroids are used to treat severe forms of HSP nephropathy, which anatomopathologically corresponds to IgA glomerulonephritis. Active research studies are needed to clarify the pathogenesis, the prognostic factors, and the measures to be taken for the prevention and treatment of renal disease.

Keywords: Anaphylactoid purpura, Asialoglycoprotein receptor, ß1, 3- galactosyltransferase, Childhood systemic vasculitis, Children, Galactose deficient IgA1, Henoch-Schönlein purpura, IgA1 isoform, IgA vasculitis, IgA glomerulonephritis, IgAV nephropathy, IgG-Gd-IgA, Immune complexes, Leukocytoclastic vasculitis, Lower limb purpura, Non-granulomatous vasculitis, Pediatric purpura, Pediatric vasculitis, Rheumatoid purpura, Small vessel vasculitis.