title: IgA vasculitis (Henoch-Shönlein purpura) in adults: Diagnostic and therapeutic aspects.
abstract: Immunoglobulin A (IgA) vasculitis, formerly called Henoch-Schönlein purpura, is an immune complex vasculitis affecting small vessels with dominant IgA deposits. Clinical manifestations mainly involve cutaneous purpura, arthralgias and/or arthritis, acute enteritis and glomerulonephritis. IgA vasculitis is more common among children than adults, with more severe disease in adults. Gastrointestinal and renal involvements represent the principal causes of morbidity and mortality in adults. Factors associated with long-term end-stage renal disease (ESRD) include baseline renal function impairment and baseline proteinuria >1 or 1.5 g/day, and on renal biopsy degree of interstitial fibrosis, sclerotic glomeruli and fibrinoid necrosis. Management of IgA vasculitis in adults is rendered difficult for clinicians because of the absence of correlation between initial presentation and long-term renal outcome, and the possible occurrence of spontaneous remission in patients with severe presentation or, in contrast, possible evolution to ESRD in patients with mild symptoms. Treatment is often symptomatic because disease course is usually benign. Treatment of severe involvement, including severe gastrointestinal complications or proliferative glomerulonephritis, remains controversial, with no evidence that corticosteroids or immunosuppressive agents improved long-term outcome. Prospective, randomized, controlled trials are thus needed to analyze the benefit-risk ratio of such treatments.
authors: Department of Internal Medicine, National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris Descartes, Paris, France. Electronic address: alexandra.audemard@gmail.com.
authors: Alexandra
authors: A
authors: Audemard-Verger
authors: Department of Nephrology, Hôpital Saint Louis, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France.
authors: Evangeline
authors: E
authors: Pillebout
authors: Department of Internal Medicine, National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris Descartes, Paris, France.
authors: Loïc
authors: L
authors: Guillevin
authors: Department of Nephrology, Hôpital Européen Georges Pompidou, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris Descartes, Paris, France.
authors: Eric
authors: E
authors: Thervet
authors: Department of Internal Medicine, National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris Descartes, Paris, France.
authors: Benjamin
authors: B
authors: Terrier
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D006801:Humans; D011695:IgA Vasculitis / Q000175:diagnosis* / Q000276:immunology / Q000628:therapy*; D007070:Immunoglobulin A / Q000276:immunology*; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007676:Kidney Failure, Chronic / Q000209:etiology; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones; D007070:Immunoglobulin A; D007166:Immunosuppressive Agents
keywords: Characteristics; Henoch–Shönlein purpura; IgA vasculitis; Prognosis; Treatment