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".)
Subscribers log in hereLiterature review current through: Jul 2017. | This topic last updated: Jan 13, 2016.References
- Kauffmann RH, Herrmann WA, Meÿer CJ, et al. Circulating IgA-immune complexes in Henoch-Schönlein purpura. A longitudinal study of their relationship to disease activity and vascular deposition of IgA. Am J Med 1980; 69:859.
- Meadow SR, Scott DG. Berger disease: Henoch-Schönlein syndrome without the rash. J Pediatr 1985; 106:27.
- Kiryluk K, Moldoveanu Z, Sanders JT, et al. Aberrant glycosylation of IgA1 is inherited in both pediatric IgA nephropathy and Henoch-Schönlein purpura nephritis. Kidney Int 2011; 80:79.
- O'Donoghue DJ, Darvill A, Ballardie FW. Mesangial cell autoantigens in immunoglobulin A nephropathy and Henoch-Schönlein purpura. J Clin Invest 1991; 88:1522.
- O'Donoghue DJ, Jewkes F, Postlethwaite RJ, Ballardie FW. Autoimmunity to glomerular antigens in Henoch-Schoenlein nephritis. Clin Sci (Lond) 1992; 83:281.
- Gardner-Medwin JM, Dolezalova P, Cummins C, Southwood TR. Incidence of Henoch-Schönlein purpura, Kawasaki disease, and rare vasculitides in children of different ethnic origins. Lancet 2002; 360:1197.
- Meadow SR. Schönlein-Henoch syndrome. In: Pediatric Nephrology, 2nd ed, Edelmann CM (Ed), Little, Brown and Co., Boston 1992. p.1525.
- Blanco R, Martínez-Taboada VM, Rodríguez-Valverde V, et al. Henoch-Schönlein purpura in adulthood and childhood: two different expressions of the same syndrome. Arthritis Rheum 1997; 40:859.
- Kellerman PS. Henoch-Schönlein purpura in adults. Am J Kidney Dis 2006; 48:1009.
- Jauhola O, Ronkainen J, Koskimies O, et al. Renal manifestations of Henoch-Schonlein purpura in a 6-month prospective study of 223 children. Arch Dis Child 2010; 95:877.
- Cameron JS. Henoch-Schönlein purpura: clinical presentation. Contrib Nephrol 1984; 40:246.
- Saulsbury FT. Henoch-Schönlein purpura in children. Report of 100 patients and review of the literature. Medicine (Baltimore) 1999; 78:395.
- Austin HA 3rd, Balow JE. Henoch-Schönlein nephritis: prognostic features and the challenge of therapy. Am J Kidney Dis 1983; 2:512.
- Counahan R, Winterborn MH, White RH, et al. Prognosis of Henoch-Schönlein nephritis in children. Br Med J 1977; 2:11.
- Habib R, Niaudet P, Levy M. Schönlein-Henoch purpura nephritis and IgA nephropathy. In: Renal Pathology with Clinical and Functional Correlations, Tisher CC, Brenner BM (Eds), Lippincott, Philadelphia 1993. p.472.
- Goldstein AR, White RH, Akuse R, Chantler C. Long-term follow-up of childhood Henoch-Schönlein nephritis. Lancet 1992; 339:280.
- Niaudet P, Habib R. Methylprednisolone pulse therapy in the treatment of severe forms of Schönlein-Henoch purpura nephritis. Pediatr Nephrol 1998; 12:238.
- Yoshikawa N, White RH, Cameron AH. Prognostic significance of the glomerular changes in Henoch-Schoenlein nephritis. Clin Nephrol 1981; 16:223.
- Halling SF, Söderberg MP, Berg UB. Henoch Schönlein nephritis: clinical findings related to renal function and morphology. Pediatr Nephrol 2005; 20:46.
- Trapani S, Micheli A, Grisolia F, et al. Henoch Schonlein purpura in childhood: epidemiological and clinical analysis of 150 cases over a 5-year period and review of literature. Semin Arthritis Rheum 2005; 35:143.
- Chang WL, Yang YH, Wang LC, et al. Renal manifestations in Henoch-Schönlein purpura: a 10-year clinical study. Pediatr Nephrol 2005; 20:1269.
- Stewart M, Savage JM, Bell B, McCord B. Long term renal prognosis of Henoch-Schönlein purpura in an unselected childhood population. Eur J Pediatr 1988; 147:113.
- Narchi H. Risk of long term renal impairment and duration of follow up recommended for Henoch-Schonlein purpura with normal or minimal urinary findings: a systematic review. Arch Dis Child 2005; 90:916.
- Rieu P, Noël LH. Henoch-Schönlein nephritis in children and adults. Morphological features and clinicopathological correlations. Ann Med Interne (Paris) 1999; 150:151.
- Uppal SS, Hussain MA, Al-Raqum HA, et al. Henoch-Schönlein's purpura in adults versus children/adolescents: A comparative study. Clin Exp Rheumatol 2006; 24:S26.
- Pillebout E, Thervet E, Hill G, et al. Henoch-Schönlein Purpura in adults: outcome and prognostic factors. J Am Soc Nephrol 2002; 13:1271.
- Coppo R, Mazzucco G, Cagnoli L, et al. Long-term prognosis of Henoch-Schönlein nephritis in adults and children. Italian Group of Renal Immunopathology Collaborative Study on Henoch-Schönlein purpura. Nephrol Dial Transplant 1997; 12:2277.
- Coppo R, Andrulli S, Amore A, et al. Predictors of outcome in Henoch-Schönlein nephritis in children and adults. Am J Kidney Dis 2006; 47:993.
- Shrestha S, Sumingan N, Tan J, et al. Henoch Schönlein purpura with nephritis in adults: adverse prognostic indicators in a UK population. QJM 2006; 99:253.
- Fogazzi GB, Pasquali S, Moriggi M, et al. Long-term outcome of Schönlein-Henoch nephritis in the adult. Clin Nephrol 1989; 31:60.
- Rauta V, Törnroth T, Grönhagen-Riska C. Henoch-Schoenlein nephritis in adults-clinical features and outcomes in Finnish patients. Clin Nephrol 2002; 58:1.
- Monastiri K, Selmi H, Tabarki B, et al. Primary antiphospholipid syndrome presenting as complicated Henoch-Schönlein purpura. Arch Dis Child 2002; 86:132.
- Michel BA, Hunder GG, Bloch DA, Calabrese LH. Hypersensitivity vasculitis and Henoch-Schönlein purpura: a comparison between the 2 disorders. J Rheumatol 1992; 19:721.
- Levy M, Broyer M, Arsan A, et al. Anaphylactoid purpura nephritis in childhood: natural history and immunopathology. Adv Nephrol Necker Hosp 1976; 6:183.
- Calviño MC, Llorca J, García-Porrúa C, et al. Henoch-Schönlein purpura in children from northwestern Spain: a 20-year epidemiologic and clinical study. Medicine (Baltimore) 2001; 80:279.
- Ronkainen J, Ala-Houhala M, Huttunen NP, et al. Outcome of Henoch-Schoenlein nephritis with nephrotic-range proteinuria. Clin Nephrol 2003; 60:80.
- Ronkainen J, Nuutinen M, Koskimies O. The adult kidney 24 years after childhood Henoch-Schönlein purpura: a retrospective cohort study. Lancet 2002; 360:666.
- Shin JI, Park JM, Kim JH, et al. Factors affecting histological regression of crescentic Henoch-Schönlein nephritis in children. Pediatr Nephrol 2006; 21:54.
- Davin JC, Coppo R. Pitfalls in recommending evidence-based guidelines for a protean disease like Henoch-Schönlein purpura nephritis. Pediatr Nephrol 2013; 28:1897.
- Kidney Disease: Improving Global Outcomes. Chapter 11: Henoch-Schonlein purpura nephritis. Kidney Int Suppl (2011) 2012; 2:218.
- Davin JC. Henoch-Schonlein purpura nephritis: pathophysiology, treatment, and future strategy. Clin J Am Soc Nephrol 2011; 6:679.
- Tarshish P, Bernstein J, Edelmann CM Jr. Henoch-Schönlein purpura nephritis: course of disease and efficacy of cyclophosphamide. Pediatr Nephrol 2004; 19:51.
- Pillebout E, Alberti C, Guillevin L, et al. Addition of cyclophosphamide to steroids provides no benefit compared with steroids alone in treating adult patients with severe Henoch Schönlein Purpura. Kidney Int 2010; 78:495.
- Balow JE. Renal vasculitis. Kidney Int 1985; 27:954.
- Shin JI, Park JM, Shin YH, et al. Cyclosporin A therapy for severe Henoch-Schönlein nephritis with nephrotic syndrome. Pediatr Nephrol 2005; 20:1093.
- Park JM, Won SC, Shin JI, et al. Cyclosporin A therapy for Henoch-Schönlein nephritis with nephrotic-range proteinuria. Pediatr Nephrol 2011; 26:411.
- Jauhola O, Ronkainen J, Autio-Harmainen H, et al. Cyclosporine A vs. methylprednisolone for Henoch-Schönlein nephritis: a randomized trial. Pediatr Nephrol 2011; 26:2159.
- Bergstein J, Leiser J, Andreoli SP. Response of crescentic Henoch-Schoenlein purpura nephritis to corticosteroid and azathioprine therapy. Clin Nephrol 1998; 49:9.
- Oner A, Tinaztepe K, Erdogan O. The effect of triple therapy on rapidly progressive type of Henoch-Schönlein nephritis. Pediatr Nephrol 1995; 9:6.
- Iijima K, Ito-Kariya S, Nakamura H, Yoshikawa N. Multiple combined therapy for severe Henoch-Schönlein nephritis in children. Pediatr Nephrol 1998; 12:244.
- Kawasaki Y, Suzuki J, Suzuki H. Efficacy of methylprednisolone and urokinase pulse therapy combined with or without cyclophosphamide in severe Henoch-Schoenlein nephritis: a clinical and histopathological study. Nephrol Dial Transplant 2004; 19:858.
- Kauffmann RH, Houwert DA. Plasmapheresis in rapidly progressive Henoch-Schoenlein glomerulonephritis and the effect on circulating IgA immune complexes. Clin Nephrol 1981; 16:155.
- Hattori M, Ito K, Konomoto T, et al. Plasmapheresis as the sole therapy for rapidly progressive Henoch-Schönlein purpura nephritis in children. Am J Kidney Dis 1999; 33:427.
- Rostoker G, Desvaux-Belghiti D, Pilatte Y, et al. High-dose immunoglobulin therapy for severe IgA nephropathy and Henoch-Schönlein purpura. Ann Intern Med 1994; 120:476.
- Meulders Q, Pirson Y, Cosyns JP, et al. Course of Henoch-Schönlein nephritis after renal transplantation. Report on ten patients and review of the literature. Transplantation 1994; 58:1179.
- Nast CC, Ward HJ, Koyle MA, Cohen AH. Recurrent Henoch-Schönlein purpura following renal transplantation. Am J Kidney Dis 1987; 9:39.
- Hasegawa A, Kawamura T, Ito H, et al. Fate of renal grafts with recurrent Henoch-Schönlein purpura nephritis in children. Transplant Proc 1989; 21:2130.
- Kanaan N, Mourad G, Thervet E, et al. Recurrence and graft loss after kidney transplantation for henoch-schonlein purpura nephritis: a multicenter analysis. Clin J Am Soc Nephrol 2011; 6:1768.
- Ponticelli C, Moroni G, Glassock RJ. Recurrence of secondary glomerular disease after renal transplantation. Clin J Am Soc Nephrol 2011; 6:1214.
- Thervet E, Aouizerate J, Noel LH, et al. Histologic recurrence of Henoch-Schonlein Purpura nephropathy after renal transplantation on routine allograft biopsy. Transplantation 2011; 92:907.
- Samuel JP, Bell CS, Molony DA, Braun MC. Long-term outcome of renal transplantation patients with Henoch-Schonlein purpura. Clin J Am Soc Nephrol 2011; 6:2034.
- Sanfilippo F, Croker BP, Bollinger RR. Fate of four cadaveric donor renal allografts with mesangial IgA deposits. Transplantation 1982; 33:370.
- Cuevas X, Lloveras J, Mir M, et al. Disappearance of mesangial IgA deposits from the kidneys of two donors after transplantation. Transplant Proc 1987; 19:2208.
- Baliah T, Kim KH, Anthone S, et al. Recurrence of Henoch-Schönlein purpura glomerulonephritis in transplanted kidneys. Transplantation 1974; 18:343.