Skip to main content
SpringerLink
Log in

IgA vasculitis nephritis in children and adults: one or different entities?

  • Review
  • Published:
Pediatric Nephrology Aims and scope Submit manuscript

Abstract

The clinical features of the kidney involvement in immunoglobulin A (IgA) vasculitis (IgAVN) differ in children and adults for both clinical presentation and progression. IgAVN in children has mostly a self-limiting course and favorable resolution, while in adults the kidney involvement is frequently severe with unfavorable outcome. However, a subset of children is at risk of progression within the pediatric age or decades later in adulthood, particularly when the diagnosis and a prompt intervention are delayed. Factors predicting progression and outcome in the whole spectrum of age have been investigated in recent research, as well as the relationship between IgAVN and primary IgAN, which share the same pathology features, in the light of peculiar clinical differences and progression tendencies, and hence need for selective treatments. The search for a personalized treatment in children with IgAV and in different ages of life should rely on the identification of different risks for progression. This review will focus on recent studies which contribute to improve our knowledge in this still largely unclear area.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Davin JC, Coppo R (2014) Henoch-Schönlein purpura nephritis in children. Nat Rev Nephrol 10:563–573. https://doi.org/10.1038/nrneph.2014.126

    Article  CAS  PubMed  Google Scholar 

  2. Ozen S, Ruperto N, Dillon MJ, Bagga A, Barron K, Davin JC, Kawasaki T, Lindsley C, Petty RE, Prieur AM, Ravelli A, Woo P (2006) EULAR/PReS endorsed consensus criteria* for the classification of childhood vasculitides. Ann Rheum Dis 65:936–941. https://doi.org/10.1136/ard.2005.046300

    Article  CAS  PubMed  Google Scholar 

  3. Ozen S, Pistorio A, Iusan SM, Bakkaloglu A, Herlin T, Brik R, Buoncompagni A, Lazar C, Bilge I, Uziel Y, Rigante D, Cantarini L, Hilario MO, Silva CA, Alegria M, Norambuena X, Belot A, Berkun Y, Estrella AI, Olivieri AN, Alpigiani MG, Rumba I, Sztajnbok F, Tambic-Bukovac L, Breda L, Al-Mayouf S, Mihaylova D, Chasnyk V, Sengler C, Klein-Gitelman M, Djeddi D, Nuno L, Pruunsild C, Brunner J, Kondi A, Pagava K, Pederzoli S, Martini A, Ruperto N, Paediatric Rheumatology International Trials Organisation (PRINTO) (2010) EULAR/PRINTO/PRES criteria for Henoch-Schonlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: final classification criteria. Ann Rheum Dis 69:798–806. https://doi.org/10.1136/ard.2009.116657

    Article  PubMed  Google Scholar 

  4. Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, Flores-Suarez LF, Gross WL, Guillevin L, Hagen EC, Hoffman GS, Jayne DR, Kallenberg CG, Lamprecht P, Langford CA, Luqmani RA, Mahr AD, Matteson EL, Merkel PA, Ozen S, Pusey CD, Rasmussen N, Rees AJ, Scott DG, Specks U, Stone JH, Takahashi K, Watts RA (2013) 2012 Revised International Chapel Hill consensus conference nomenclature of vasculitides. Arthritis Rheum. 65:1-11. https://doi.org/10.1002/art.37715

  5. Davin JC (2011) Henoch-Schönlein purpura nephritis: pathophysiology, treatment, and future strategy. Clin J Am Soc Nephrol 6:679–689. https://doi.org/10.2215/CJN.06710810

    Article  PubMed  Google Scholar 

  6. Audemard-Verger A, Pillebout E, Guillevin L, Thervet E, Terrier B (2015) IgA vasculitis (Henoch-Shönlein purpura) in adults: diagnostic and therapeutic aspects. Autoimmun Rev 14:579–585. https://doi.org/10.1016/j.autrev.2015.02.003

    Article  CAS  PubMed  Google Scholar 

  7. Selewski DT, Ambruzs JM, Appel GB, Bomback AS, Matar RB, Cai Y, Cattran DC, Chishti AS, D'Agati VD, D'Alessandri-Silva CJ, Gbadegesin RA, Hogan JJ, Iragorri S, Jennette JC, Julian BA, Khalid M, Lafayette RA, Liapis H, Lugani F, Mansfield SA, Mason S, Nachman PH, Nast CC, Nester CM, Noone DG, Novak J, O'Shaughnessy MM, Reich HN, Rheault MN, Rizk DV, Saha MK, Sanghani NS, Sperati CJ, Sreedharan R, Srivastava T, Swiatecka-Urban A, Twombley K, Vasylyeva TL, Weaver DJ, Yin H, Zee J, Falk RJ, Gharavi AG, Gillespie BW, Gipson DS, Greenbaum LA, Holzman LB, Kretzler M, Robinson BM, Smoyer WE, Flessner M, Guay-Woodford LM, Kiryluk K, CureGN Consortium (2018) Clinical characteristics and treatment patterns of children and adults with IgA nephropathy or IgA vasculitis: findings from the CureGN study. Kidney Int Rep 3:1373–1384. https://doi.org/10.1016/j.ekir.2018.07.021

    Article  PubMed  PubMed Central  Google Scholar 

  8. Coppo R (2018) Towards a personalized treatment for IgA nephropathy considering pathology and pathogenesis. Nephrol Dial Transplant 34:1832–1838. https://doi.org/10.1093/ndt/gfy338

    Article  CAS  Google Scholar 

  9. Hu S, Birg A, Hovaida M, Gavin MW, McCarthy D (2017) Avoiding “‘rash’” conclusions: challenge of IgA vasculitis in adults. Dig Dis Sci 62:874–878. https://doi.org/10.1007/s10620-017-4505-2

    Article  CAS  PubMed  Google Scholar 

  10. Gardner-Medwin JMM, Dolezalova P, Cummins C, Southwood TR (2002) Incidence of Henoch-Schönlein purpura, Kawasaki disease, and rare vasculitides in children of different ethnic origins. Lancet 360:1197–1202. https://doi.org/10.1016/S0140-6736(02)11279-7

    Article  PubMed  Google Scholar 

  11. Pillebout É, Verine J (2014) Purpura rhumatoïde de l’adulte. Rev Med Interne 35:372–381

    Article  CAS  PubMed  Google Scholar 

  12. Watson L, Richardson ARW, Holt RCL, Jones CA, Beresford MW (2012) Henoch-Shonlein purpura - a 5-year review and proposed pathway. PLoS One 7:e29512. https://doi.org/10.1371/journal.pone.0029512

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  13. Oni L, Sampath S (2019) Childhood IgA vasculitis (Henoch-Schonlein purpura)-advances and knowledge gaps. Front Pediatr 7:257. https://doi.org/10.3389/fped.2019.00257

    Article  PubMed  PubMed Central  Google Scholar 

  14. Nicoara O, Twombley K (2019) Immunoglobulin A nephropathy and Immunoglobulin A vasculitis. Pediatr Clin N Am 66:101–110. https://doi.org/10.1016/j.pcl.2018.08.008

    Article  Google Scholar 

  15. Tracy A, Subramanian A, Adderley NJ, Cockwell P, Ferro C, Ball S, Harper L, Nirantharakumar K (2019) Cardiovascular, thromboembolic and renal outcomes in IgA vasculitis (Henoch-Schönlein purpura): a retrospective cohort study using routinely collected primary care data. Ann Rheum Dis 78:261–269. https://doi.org/10.1136/annrheumdis-2018-214142

    Article  CAS  PubMed  Google Scholar 

  16. Ronkainen J, Nuutinen M, Koskimies O (2002) The adult kidney 24 years after childhood Henoch-Schönlein purpura: a retrospective cohort study. Lancet 360:666–670. https://doi.org/10.1016/S0140-6736(02)09835-5

    Article  PubMed  Google Scholar 

  17. Goldstein AR, White RHR, Akuse R, Chantler C (1992) Long-term follow-up of childhood Henoch-Schönlein nephritis. Lancet 339:280–282. https://doi.org/10.1016/0140-6736(92)91341-5

    Article  CAS  PubMed  Google Scholar 

  18. Davin JC, Weening JJ (2003) Diagnosis of Henoch-Schönlein purpura: renal or skin biopsy? Pediatr Nephrol 18:1201–1203

    Article  PubMed  Google Scholar 

  19. Hočevar A, Tomšič M, Rotar Ž (2019) IgA vasculitis in adults: few certainties and many uncertainties. Ann Rheum Dis 79:e47. https://doi.org/10.1136/annrheumdis-2018-214907

    Article  PubMed  Google Scholar 

  20. Gupta V, Aggarwal A, Gupta R, Chandra Chowdhury A, Agarwal V, Lawrence A, Misra R (2018) Differences between adult and pediatric onset Henoch-Schonlein purpura from North India. Int J Rheum Dis 21:292–298. https://doi.org/10.1111/1756-185X.13221

    Article  PubMed  Google Scholar 

  21. Amoroso A, Berrino M, Canale L, Coppo R, Cornaglia M, Guarrera S, Mazzola G, Scolari F (1997) Immunogenetics of Henoch Schonlein disease. Eur J Immunogenet 24:323–333. https://doi.org/10.1046/j.1365-2370.1997.d01-112.x

    Article  CAS  PubMed  Google Scholar 

  22. Lõpez-Mejías R, Genre F, Pérez BS, Castañeda S, Ortego-Centeno N, Llorca J, Ubilla B, Remuzgo-Martínez S, Mijares V, Pina T, Calvo-Río V, Márquez A, Sala-Icardo L, Miranda-Filloy JA, Conde-Jaldón M, Ortiz-Fernández L, Rubio E, León Luque M, Blanco-Madrigal JM, Galíndez-Aguirregoikoa E, González-Vela MC, Ocejo-Vinyals JG, González Escribano F, Martín J, Blanco R, González-Gay MA (2015) HLA-DRB1 association with Henoch-Schonlein purpura. Arthritis Rheum 67:823–827. https://doi.org/10.1002/art.38979

    Article  Google Scholar 

  23. López-Mejías R, Castañeda S, Genre F, Remuzgo-Martínez S, Carmona FD, Llorca J, Blanco R, Martín J, González-Gay MA (2018) Genetics of immunoglobulin-a vasculitis (Henoch-Schönlein purpura): an updated review. Autoimmun Rev 17:301–315. https://doi.org/10.1016/j.autrev.2017.11.024

    Article  CAS  PubMed  Google Scholar 

  24. González-Gay MA, López-Mejías R, Pina T, Blanco R, Castañeda S (2018) IgA vasculitis: genetics and clinical and therapeutic management. Curr Rheumatol Rep 20:24. https://doi.org/10.1007/s11926-018-0735-3

    Article  CAS  PubMed  Google Scholar 

  25. He X, Zhao P, Kang S, Ding Y, Luan J, Liu Z, Wu Y, Yin W (2012) C1GALT1 polymorphisms are associated with Henoch-Schönlein purpura nephritis. Pediatr Nephrol 27:1505–1509. https://doi.org/10.1007/s00467-012-2178-9

    Article  PubMed  Google Scholar 

  26. Gale DP, Molyneux K, Wimbury D, Higgins P, Levine AP, Caplin B, Ferlin A, Yin P, Nelson CP, Stanescu H, Samani NJ, Kleta R, Yu X, Barratt J (2017) Galactosylation of IgA1 is associated with common variation in C1GALT1. J Am Soc Nephrol 28:2158–2166. https://doi.org/10.1681/ASN.2016091043

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  27. He X, Yu C, Zhao P, Ding Y, Liang X, Zhao Y, Yue X, Wu Y, Yin W (2013) The genetics of Henoch–Schönlein purpura: a systematic review and meta-analysis. Rheumatol Int 33:1387–1395. https://doi.org/10.1007/S00296-012-2661-4

    Article  PubMed  Google Scholar 

  28. Amoroso A, Danek G, Vatta S, Crovella S, Berrino M, Guarrera S, Fasano ME, Mazzola G, Amore A, Gianoglio B, Peruzzi L, Coppo R (1998) Polymorphisms in angiotensin-converting enzyme gene and severity of renal disease in Henoch-Schonlein patients. Nephrol Dial Transplant 13:3184–3188. https://doi.org/10.1093/ndt/13.12.3184

    Article  CAS  PubMed  Google Scholar 

  29. Jin Y, Xie Q, Li N, Mo X, Liu S, Tao Y, Wang J (2019) Exploration of susceptible genes associated with Henoch-Schönlein purpura by whole exome sequencing. Adv Clin Exp Med 28:1199–1207. https://doi.org/10.17219/acem/103800

    Article  PubMed  Google Scholar 

  30. Liu D, Lu F, Zhai S, Wei L, Ma S, Chen X, Dong L, Guo Y, Wu J, Wang Z (2010) Renin-angiotensin system gene polymorphisms in children with Henoch-Schönlein purpura in West China. J Renin-Angiotensin-Aldosterone Syst 11:248–255. https://doi.org/10.1177/1470320310374214

    Article  CAS  Google Scholar 

  31. Martin J, Paco L, Ruiz MP, Lopez-Nevot MA, Garcia-Porrua C, Amoli MM, Calviño MC, Ollier WE, Gonzalez-Gay MA (2005) Inducible nitric oxide synthase polymorphism is associated with susceptibility to Henoch–Schönlein purpura in northwestern Spain. J Rheumatol 32:1081–1085

    CAS  PubMed  Google Scholar 

  32. Berthelot L, Jamin A, Viglietti D, Chemouny JM, Ayari H, Pierre M, Housset P, Sauvaget V, Hurtado-Nedelec M, Vrtovsnik F, Daugas E, HSPrognosis Group, Monteiro RC, Pillebout E, members of the HSPrognosis Group (2017) Value of biomarkers for predicting immunoglobulin a vasculitis nephritis outcome in an adult prospective cohort. Nephrol Dial Transplant 33:1579–1590. https://doi.org/10.1093/ndt/gfx300

    Article  CAS  Google Scholar 

  33. Suzuki H, Kiryluk K, Novak J, Moldoveanu Z, Herr AB, Renfrow MB, Wyatt RJ, Scolari F, Mestecky J, Gharavi AG, Julian BA (2011) The pathophysiology of IgA nephropathy. J Am Soc Nephrol 22:1795–1803. https://doi.org/10.1681/ASN.2011050464

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  34. Monteiro RC (2018) Recent advances in the physiopathology of IgA nephropathy. Nephrol Ther 14:S1–S8. https://doi.org/10.1016/j.nephro.2018.02.004

    Article  PubMed  Google Scholar 

  35. Suzuki H, Yasutake J, Makita Y, Tanbo Y, Yamasaki K, Sofue T, Kano T, Suzuki Y (2018) IgA nephropathy and IgA vasculitis with nephritis have a shared feature involving galactose-deficient IgA1-oriented pathogenesis. Kidney Int 93:753–760. https://doi.org/10.1016/j.kint.2017.10.019

    Article  CAS  Google Scholar 

  36. Takahata A, Arai S, Hiramoto E, Kitada K, Kato R, Makita Y, Suzuki H, Nakata J, Araki K, Miyazaki T, Suzuki Y (2020) Crucial role of AIM/CD5L in the development of glomerular inflammation in IgA nephropathy. J Am Soc Nephrol 31:2013–2024. https://doi.org/10.1681/ASN.2019100987

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  37. Kiryluk K, Moldoveanu Z, Sanders JT, Eison TM, Suzuki H, Julian BA, Novak J, Gharavi AG, Wyatt RJ (2011) Aberrant glycosylation of IgA1 is inherited in both pediatric IgA nephropathy and Henoch-Schönlein purpura nephritis. Kidney Int 80:79–87. https://doi.org/10.1038/ki.2011.16

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  38. Suzuki H, Moldoveanu Z, Julian BA, Wyatt RJ, Novak J (2019) Autoantibodies specific for galactose-deficient IgA1 in IgA vasculitis with nephritis. Kidney Int Rep 4:1717–1724. https://doi.org/10.1016/j.ekir.2019.08.015

    Article  PubMed  PubMed Central  Google Scholar 

  39. Mizerska-Wasiak M, Gajewski Ł, Cichoń-Kawa K, Małdyk J, Dziedzic-Jankowska K, Leszczyńska B, Rybi-Szumińska A, Wasilewska A, Pukajło-Marczyk A, Zwolińska D, Bieniaś B, Sikora P, Szczepańska M, Stelmaszczyk-Emmel A, Górska E, Pańczyk-Tomaszewska M (2018) Serum GDIgA1 levels in children with IgA nephropathy and Henoch-Schönlein nephritis. Cent Eur J Immunol 43:162–167. https://doi.org/10.5114/ceji.2018.77386

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  40. Zhai YL, Zhu L, Shi SF, Liu LJ, Lv JC, Zhang H (2016) Increased APRIL expression induces IgA1 aberrant glycosylation in IgA nephropathy. Medicine (Baltimore) 95:e3099. https://doi.org/10.1097/MD.0000000000003099

    Article  CAS  Google Scholar 

  41. Coppo R (2018) The gut-renal connection in IgA nephropathy. Semin Nephrol 38:504–512

    Article  CAS  PubMed  Google Scholar 

  42. Davin JC, Forget P, Mahieu PR (1988) Increased intestinal permeability to (51 Cr) EDTA is correlated with IgA immune complex-plasma levels in children with IgA-associated nephropathies. Acta Paediatr Scand 77:118–124

    Article  CAS  PubMed  Google Scholar 

  43. Kovács T, Kun L, Schmelczer M, Wagner L, Davin JC, Nagy J (1996) Do intestinal hyperpermeability and the related food antigens play a role in the progression of IgA nephropathy? I. Study of intestinal permeability. Am J Nephrol 16:500–505

    Article  PubMed  Google Scholar 

  44. Wu H, Wang X, Yang Z, Zhao Q, Wen Y, Li X, Zhang W, Gao R (2020) Serum soluble CD89-IgA complexes are elevated in IgA nephropathy without immunosuppressant history. Dis Markers 2020:8393075. https://doi.org/10.1155/2020/8393075

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  45. Pillebout E, Jamin A, Ayari H, Housset P, Pierre M, Sauvaget V, Viglietti D, Deschenes G, Monteiro RC, Berthelot L, HSPrognosis group (2017) Biomarkers of IgA vasculitis nephritis in children. PLoS One 12:e0188718. https://doi.org/10.1371/journal.pone.0188718

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  46. Suzuki H, Allegri L, Suzuki Y, Hall S, Moldoveanu Z, Wyatt RJ, Novak J, Julian BA (2016) Galactose-deficient IgA1 as a candidate urinary polypeptide marker of IgA nephropathy? Dis Markers 2016:7806438. https://doi.org/10.1155/2016/7806438

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  47. Suzuki H (2019) Biomarkers for IgA nephropathy on the basis of multi-hit pathogenesis. Clin Exp Nephrol 23:26–31

    Article  CAS  PubMed  Google Scholar 

  48. Jennette JC, Xiao H, Hu P (2013) Complement in ANCA-associated vasculitis. Semin Nephrol 33:557–564. https://doi.org/10.1016/j.semnephrol.2013.08.006

    Article  CAS  PubMed Central  Google Scholar 

  49. Hastings MC, Moldoveanu Z, Suzuki H, Berthoux F, Julian BA, Sanders JT, Renfrow MB, Novak J, Wyatt RJ (2013) Biomarkers in IgA nephropathy: relationship to pathogenetic hits. Expert Opin Med Diagn 7:615–627. https://doi.org/10.1517/17530059.2013.856878

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  50. Coppo R (2017) Biomarkers and targeted new therapies for IgA nephropathy. Pediatr Nephrol 32:725–731. https://doi.org/10.1007/s00467-016-3390-9

    Article  PubMed  Google Scholar 

  51. Trimarchi H, Coppo R (2019) Glomerular endothelial activation, C4d deposits and microangiopathy in immunoglobulin A nephropathy. Nephrol Dial Transplant. https://doi.org/10.1093/ndt/gfz241

  52. Espinosa M, Ortega R, Sánchez M, Segarra A, Salcedo MT, González F, Camacho R, Valdivia MA, Cabrera R, López K, Pinedo F, Gutierrez E, Valera A, Leon M, Cobo MA, Rodriguez R, Ballarín J, Arce Y, García B, Muñoz MD, Praga M, Spanish Group for Study of Glomerular Diseases (GLOSEN) (2014) Association of C4d deposition with clinical outcomes in IgA nephropathy. Clin J Am Soc Nephrol 9:897–904. https://doi.org/10.2215/CJN.09710913

    Article  PubMed  PubMed Central  Google Scholar 

  53. Fabiano RCG, de Almeida AS, Bambirra EA, Oliveira EA, Simões E, Silva AC, Pinheiro SVB (2017) Mesangial C4d deposition may predict progression of kidney disease in pediatric patients with IgA nephropathy. Pediatr Nephrol 32:1211–1220. https://doi.org/10.1007/s00467-017-3610-y

    Article  PubMed  Google Scholar 

  54. Chua JS, Zandbergen M, Wolterbeek R, Baelde HJ, van Es LA, de Fijter JW, Bruijn JA, Bajema IM (2019) Complement-mediated microangiopathy in IgA nephropathy and IgA vasculitis with nephritis. Mod Pathol 32:1147–1157. https://doi.org/10.1038/s41379-019-0259-z

    Article  CAS  PubMed  Google Scholar 

  55. Heineke MH, Ballering AV, Jamin A, Ben Mkaddem S, Monteiro RC, Van Egmond M (2017) New insights in the pathogenesis of immunoglobulin A vasculitis (Henoch-Schönlein purpura). Autoimmun Rev 16:1246–1253. https://doi.org/10.1016/j.autrev.2017.10.009

    Article  CAS  PubMed  Google Scholar 

  56. Legendre P, Régent A, Thiebault M, Mouthon L (2016) Anti-endothelial cell antibodies in vasculitis: a systematic review. Autoimmun Rev 16:146–153. https://doi.org/10.1016/j.autrev.2016.12.012

    Article  CAS  PubMed  Google Scholar 

  57. Yang YH, Lai HJ, Kao CK, Lin YT, Chiang BL (2004) The association between transforming growth factor-β gene promoter C-509T polymorphism and Chinese children with Henoch-Schönlein purpura. Pediatr Nephrol 19:972–975. https://doi.org/10.1007/s00467-004-1547-4

    Article  PubMed  Google Scholar 

  58. Yang YH, Wang SJ, Chuang YH, Lin YT, Chiang BL (2002) The level of IgA antibodies to human umbilical vein endothelial cells can be enhanced by TNF-α treatment in children with Henoch-Schönlein purpura. Clin Exp Immunol 130:352–357. https://doi.org/10.1046/j.1365-2249.2002.01964.x

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  59. Working Group of the International IgA Nephropathy Network and the Renal Pathology Society, Cattran DC, Coppo R, Cook HT, Feehally J, Roberts IS, Troyanov S, Alpers CE, Amore A, Barratt J, Berthoux F, Bonsib S, Bruijn JA, D’Agati V, D’Amico G, Emancipator S, Emma F, Ferrario F, Fervenza FC, Florquin S, Fogo A, Geddes CC, Groene HJ, Haas M, Herzenberg AM, Hill PA, Hogg RJ, Hsu SI, Jennette JC, Joh K, Julian BA, Kawamura T, Lai FM, Leung CB, Li LS, Li PK, Liu ZH, Mackinnon B, Mezzano S, Schena FP, Tomino Y, Walker PD, Wang H, Weening JJ, Yoshikawa N, Zhang H (2009) The Oxford classification of IgA nephropathy: rationale, clinicopathological correlations, and classification. Kidney Int 76:534–545. https://doi.org/10.1038/ki.2009.243

    Article  Google Scholar 

  60. Kim CH, Lim BJ, Bae YS, Kwon YE, Kim YL, Nam KH, Park KS, An SY, Koo HM, Doh FM, Lee MJ, Oh HJ, Yoo TH, Kang SW, Choi KH, Jeong HJ, Han SH (2014) Using the Oxford classification of IgA nephropathy to predict long-term outcomes of Henoch-Schönlein purpura nephritis in adults. Mod Pathol 27:972–982. https://doi.org/10.1038/modpathol.2013.222

    Article  CAS  PubMed  Google Scholar 

  61. Xu K, Zhang L, Ding J, Wang S, Su B, Xiao H, Wang F, Zhong X, Li Y (2018) Value of the Oxford classification of IgA nephropathy in children with Henoch–Schönlein purpura nephritis. J Nephrol 31:279–286. https://doi.org/10.1007/s40620-017-0457-z

    Article  PubMed  Google Scholar 

  62. Hotta O, Oda T (2019) The epipharynx-kidney axis triggers glomerular vasculitis in immunoglobulin a nephropathy. Immunol Res 67:304–309. https://doi.org/10.1007/s12026-019-09099-3

    Article  PubMed  Google Scholar 

  63. Cornes JS (1965) Peyer’s patches in the human gut. Proc R Soc Med 58:716

    CAS  PubMed  PubMed Central  Google Scholar 

  64. Darrigues J, van Meerwijk JPM, Romagnoli P (2018) Age-dependent changes in regulatory T lymphocyte development and function: a mini-review. Gerontology 64:28–35

    Article  CAS  PubMed  Google Scholar 

  65. Donadio ME, Loiacono E, Peruzzi L, Amore A, Camilla R, Chiale F, Vergano L, Boido A, Conrieri M, Bianciotto M, Bosetti FM, Coppo R (2014) Toll-like receptors, immunoproteasome and regulatory T cells in children with Henoch-Schönlein purpura and primary IgA nephropathy. Pediatr Nephrol 29:1545–1551. https://doi.org/10.1007/s00467-014-2807-6

    Article  PubMed  Google Scholar 

  66. Counahan R, Winterborn MH, White RH, Heaton JM, Meadow SR, Bluett NH, Swetschin H, Cameron JS, Chantler C (1977) Prognosis of Henoch-Schönlein nephritis in children. Br Med J 2:11–14. https://doi.org/10.1136/bmj.2.6078.11

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  67. Coppo R, Andrulli S, Amore A, Gianoglio B, Conti G, Peruzzi L, Locatelli F, Cagnoli L (2006) Predictors of outcome in Henoch-Schönlein nephritis in children and adults. Am J Kidney Dis 47:993–1003. https://doi.org/10.1053/j.ajkd.2006.02.178

    Article  PubMed  Google Scholar 

  68. Working Group of the International IgA Nephropathy Network and the Renal Pathology Society, Roberts IS, Cook HT, Troyanov S, Alpers CE, Amore A, Barratt J, Berthoux F, Bonsib S, Bruijn JA, Cattran DC, Coppo R, D’Agati V, D’Amico G, Emancipator S, Emma F, Feehally J, Ferrario F, Fervenza FC, Florquin S, Fogo A, Geddes CC, Groene HJ, Haas M, Herzenberg AM, Hill PA, Hogg RJ, Hsu SI, Jennette JC, Joh K, Julian BA, Kawamura T, Lai FM, Li LS, Li PK, Liu ZH, Mackinnon B, Mezzano S, Schena FP, Tomino Y, Walker PD, Wang H, Weening JJ, Yoshikawa N, Zhang H (2009) The Oxford classification of IgA nephropathy: pathology definitions, correlations, and reproducibility. Kidney Int 76:546–556. https://doi.org/10.1038/ki.2009.168

    Article  Google Scholar 

  69. Haas M, Verhave JC, Liu ZH, Alpers CE, Barratt J, Becker JU, Cattran D, Cook HT, Coppo R, Feehally J, Pani A, Perkowska-Ptasinska A, Roberts IS, Soares MF, Trimarchi H, Wang S, Yuzawa Y, Zhang H, Troyanov S, Katafuchi R (2017) A multicenter study of the predictive value of crescents in IgA nephropathy. J Am Soc Nephrol 28:691–701. https://doi.org/10.1681/ASN.2016040433

    Article  CAS  PubMed  Google Scholar 

  70. Trimarchi H, Barratt J, Cattran DC, Cook HT, Coppo R, Haas M, Liu ZH, Roberts IS, Yuzawa Y, Zhang H, Feehally J, IgAN Classification Working Group of the International IgA Nephropathy Network and the Renal Pathology Society; Conference Participants (2017) Oxford classification of IgA nephropathy 2016: an update from the IgA nephropathy classification working group. Kidney Int 91:1014–1021. https://doi.org/10.1016/j.kint.2017.02.003

    Article  PubMed  Google Scholar 

  71. Yun D, Kim DK, Oh KH, Joo KW, Moon KC, Kim YS, Lee K, Han SS (2020) MEST-C pathological score and long-term outcomes of child and adult patients with Henoch-Schönlein purpura nephritis. BMC Nephrol 21:33. https://doi.org/10.1186/s12882-020-1691-5

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  72. Jimenez A, Chen A, Lin J-JJ, South AM (2019) Does MEST-C score predict outcomes in pediatric Henoch-Schönlein purpura nephritis? Pediatr Nephrol 34:2583–2589. https://doi.org/10.1007/s00467-019-04327-2

    Article  PubMed  PubMed Central  Google Scholar 

  73. Shi D, Chan H, Yang X, Zhang G, Yang H, Wang M, Li Q (2019) Risk factors associated with IgA vasculitis with nephritis (Henoch–Schönlein purpura nephritis) progressing to unfavorable outcomes: a meta-analysis. PLoS One 14:e0223218. https://doi.org/10.1371/journal.pone.0223218

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  74. Huang X, Wu J, Wu XM, Hao YX, Zeng CH, Liu ZH, Tang Z (2018) Significance of histological crescent formation in patients with IgA vasculitis (Henoch-Schönlein purpura)-related nephritis: a cohort in the adult Chinese population. BMC Nephrol 19:334. https://doi.org/10.1186/s12882-018-1117-9

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  75. Coppo R, D'Arrigo G, Tripepi G, Russo ML, Roberts ISD, Bellur S, Cattran D, Cook TH, Feehally J, Tesar V, Maixnerova D, Peruzzi L, Amore A, Lundberg S, Di Palma AM, Gesualdo L, Emma F, Rollino C, Praga M, Biancone L, Pani A, Feriozzi S, Polci R, Barratt J, Del Vecchio L, Locatelli F, Pierucci A, Caliskan Y, Perkowska-Ptasinska A, Durlik M, Moggia E, Ballarin JC, Wetzels JFM, Goumenos D, Papasotiriou M, Galesic K, Toric L, Papagianni A, Stangou M, Benozzi L, Cusinato S, Berg U, Topaloglu R, Maggio M, Ots-Rosenberg M, D'Amico M, Geddes C, Balafa O, Quaglia M, Cravero R, Lino Cirami C, Fellstrom B, Floege J, Egido J, Mallamaci F, Zoccali C, ERA-EDTA Immunonephrology Working Group (2020) Is there long-term value of pathology scoring in immunoglobulin A nephropathy? A validation study of the Oxford classification for IgA nephropathy (VALIGA) update. Nephrol Dial Transplant 35:1002–1009. https://doi.org/10.1093/ndt/gfy302

    Article  CAS  PubMed  Google Scholar 

  76. Rianthavorn P, Chacranon M (2018) Long-term renal outcome in pediatric glomerulonephritis associated with crescent formation. Clin Exp Nephrol 22:661–667. https://doi.org/10.1007/s10157-017-1498-2

    Article  PubMed  Google Scholar 

  77. Coppo R, Davin JC (2014) The difficulty in considering modifiable pathology risk factors in children with IgA nephropathy: crescents and timing of renal biopsy. Pediatr Nephrol 30:189–192. https://doi.org/10.1007/s00467-014-2954-9

    Article  PubMed  Google Scholar 

  78. Hennies I, Gimpel C, Gellermann J, Möller K, Mayer B, Dittrich K, Büscher AK, Hansen M, Aulbert W, Wühl E, Nissel R, Schalk G, Weber LT, Pohl M, Wygoda S, Beetz R, Klaus G, Fehrenbach H, König S, Staude H, Beringer O, Bald M, Walden U, von Schnakenburg C, Bertram G, Wallot M, Häffner K, Wiech T, Hoyer PF, Pohl M, German Society of Pediatric Nephrology (2018) Presentation of pediatric Henoch–Schönlein purpura nephritis changes with age and renal histology depends on biopsy timing. Pediatr Nephrol 33:277–286. https://doi.org/10.1007/s00467-017-3794-1

    Article  PubMed  Google Scholar 

  79. Jelusic M, Sestan M, Cimaz R, Ozen S (2019) Different histological classifications for Henoch-Schönlein purpura nephritis : which one should be used? Pediatr Rheumatol 17:1–7. https://doi.org/10.1186/s12969-019-0311-z

  80. Koskela M, Ylinen E, Ukonmaanaho EM, Autio-Harmainen H, Heikkilä P, Lohi J, Jauhola O, Ronkainen J, Jahnukainen T, Nuutinen M (2017) The ISKDC classification and a new semiquantitative classification for predicting outcomes of Henoch–Schönlein purpura nephritis. Pediatr Nephrol 32:1201–1209. https://doi.org/10.1007/s00467-017-3608-5

    Article  PubMed  Google Scholar 

  81. Oh HJ, Ahn SV, Yoo DE, Kim SJ, Shin DH, Lee MJ, Kim HR, Park JT, Yoo TH, Kang SW, Choi KH, Han SH (2012) Clinical outcomes, when matched at presentation, do not vary between adult-onset Henöch-Schönlein purpura nephritis and IgA nephropathy. Kidney Int 82:1304–1312. https://doi.org/10.1038/ki.2012.302

    Article  CAS  PubMed  Google Scholar 

  82. Barbour S, Reich H (2018) An update on predicting renal progression in IgA nephropathy. Curr Opin Nephrol Hypertens 27:214–220. https://doi.org/10.1097/MNH.0000000000000405

    Article  CAS  PubMed  Google Scholar 

  83. Kidney Disease: Improving Global Outcomes (KDIGO) Glomerulonephritis Work Group (2012) KDIGO Clinical Practice Guideline for Glomerulonephritis. Kidney Int Suppl 2:1–274. https://doi.org/10.1038/kisup.2012.9

    Article  Google Scholar 

  84. Tarshish P, Bernstein J, Edelmann CM (2004) Henoch-Schönlein purpura nephritis: course of disease and efficacy of cyclophosphamide. Pediatr Nephrol 19:51–56. https://doi.org/10.1007/s00467-003-1315-x

    Article  PubMed  Google Scholar 

  85. Pillebout E, Alberti C, Guillevin L, Ouslimani A, Thervet E, CESAR study group (2010) Addition of cyclophosphamide to steroids provides no benefit compared with steroids alone in treating adult patients with severe Henoch Schönlein purpura. Kidney Int 78:495–502. https://doi.org/10.1038/ki.2010.150

    Article  CAS  PubMed  Google Scholar 

  86. Andersen RF, Rubak S, Jespersen B, Rittig S (2009) Early high-dose immunosuppression in Henoch-Schonlein nephrotic syndrome may improve outcome. Scand J Urol Nephrol 43:409–415. https://doi.org/10.3109/00365590903164480

    Article  CAS  PubMed  Google Scholar 

  87. Ronkainen J, Autio-Harmainen H, Nuutinen M (2003) Cyclosporin A for the treatment of severe Henoch-Schönlein glomerulonephritis. Pediatr Nephrol 18:1138–1142. https://doi.org/10.1007/s00467-003-1245-7

    Article  PubMed  Google Scholar 

  88. Ohara S, Kawasaki Y, Miyazaki K, Ono A, Suzuki Y, Suyama K, Hosoya M (2013) Efficacy of cyclosporine A for steroid-resistant severe Henoch-Schönlein purpura nephritis. Fukushima J Med Sci 59:102–107

    Article  PubMed  Google Scholar 

  89. Niaudet P, Habib R (1998) Methylprednisolone pulse therapy in the treatment of severe forms of Schonlein-Henoch purpura nephritis. Pediatr Nephrol 12:238–243. https://doi.org/10.1007/s004670050446

    Article  CAS  PubMed  Google Scholar 

  90. Koskela M, Jahnukainen T, Endén K, Arikoski P, Kataja J, Nuutinen M, Ylinen E (2019) Methylprednisolone or cyclosporine a in the treatment of Henoch-Schönlein nephritis : a nationwide study. Pediatr Nephrol 34:1447–1456. https://doi.org/10.1007/s00467-019-04238-2

    Article  PubMed  Google Scholar 

  91. Delbet JD, Hogan J, Aoun B, Stoica I, Salomon R, Decramer S, Brocheriou I, Deschênes G, Ulinski T (2017) Clinical outcomes in children with Henoch–Schönlein purpura nephritis without crescents. Pediatr Nephrol 32:1193–1199. https://doi.org/10.1007/s00467-017-3604-9

    Article  PubMed  Google Scholar 

  92. Goldstein AR, White RH, Akuse R, Chantler C (1992) Long-term follow-up of childhood Henoch-Schonlein nephritis. Lancet 339:280–282. https://doi.org/10.1016/0140-6736(92)91341-5

    Article  CAS  PubMed  Google Scholar 

  93. Schärer K, Krmar R, Querfeld U, Ruder H, Waldherr R, Schaefer F (1999) Clinical outcome of Schonlein-Henoch purpura nephritis in children. Pediatr Nephrol 13:816–823. https://doi.org/10.1007/s004670050707

    Article  PubMed  Google Scholar 

  94. Shenoy M, Ognjanovic MV, Coulthard MG (2007) Treating severe Henoch-Schönlein and IgA nephritis with plasmapheresis alone. Pediatr Nephrol 22:1167–1171. https://doi.org/10.1007/s00467-007-0498-y

    Article  PubMed  Google Scholar 

  95. Ring T, Pedersen BB, Salkus G, Goodship THJ (2015) Use of eculizumab in crescentic IgA nephropathy: proof of principle and conundrum? Clin Kidney J 8:489–491. https://doi.org/10.1093/ckj/sfv076

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  96. Rosenblad T, Rebetz J, Johansson M, Békássy Z, Sartz L, Karpman D (2014) Eculizumab treatment for rescue of renal function in IgA nephropathy. Pediatr Nephrol 29:2225–2228. https://doi.org/10.1007/s00467-014-2863-y

    Article  PubMed  Google Scholar 

  97. Hernández-Rodríguez J, Carbonell C, Mirón-Canelo JA, Diez-Ruiz S, Marcos M, Chamorro AJ (2020) Rituximab treatment for IgA vasculitis: a systematic review. Autoimmun Rev 19:102490. https://doi.org/10.1016/j.autrev.2020.102490

    Article  CAS  PubMed  Google Scholar 

  98. Ozen S, Marks SD, Brogan P, Groot N, de Graeff N, Avcin T, Bader-Meunier B, Dolezalova P, Feldman BM, Kone-Paut I, Lahdenne P, McCann L, Pilkington C, Ravelli A, van Royen A, Uziel Y, Vastert B, Wulffraat N, Kamphuis S, Beresford MW (2019) European consensus-based recommendations for diagnosis and treatment of immunoglobulin A vasculitis — the SHARE initiative. Rheumatology (Oxford) 58:1607–1616. https://doi.org/10.1093/rheumatology/kez041

    Article  CAS  Google Scholar 

  99. Barbour SJ, Coppo R, Zhang H, Liu ZH, Suzuki Y, Matsuzaki K, Katafuchi R, Er L, Espino-Hernandez G, Kim SJ, Reich HN, Feehally J, Cattran DC, International IgA Nephropathy Network (2019) Evaluating a new international risk-prediction tool in IgA nephropathy. JAMA Intern Med 179:942–952. https://doi.org/10.1001/jamainternmed.2019.0600

    Article  PubMed  PubMed Central  Google Scholar 

  100. Barbour SJ, Canney M, Coppo R, Zhang H, Liu ZH, Suzuki Y, Matsuzaki K, Katafuchi R, Induruwage D, Er L, Reich HN, Feehally J, Barratt J, Cattran DC, International IgA Nephropathy Network (2020) Improving treatment decisions using personalized risk assessment from the international IgA nephropathy prediction tool. Kidney Int 98:1009–1019. https://doi.org/10.1016/j.kint.2020.04.042

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Fondazione Ricerca Molinette, Regina Margherita Hospital, Turin, Italy

    Licia Peruzzi & Rosanna Coppo

  2. Pediatric Nephrology Unit, Regina Margherita Department, City of Health and Science University Hospital, Turin, Italy

    Licia Peruzzi

Authors
  1. Licia Peruzzi
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Rosanna Coppo
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Rosanna Coppo.

Ethics declarations

The authors do not have any conflict of interest to declare.

The review does not involve human participants and/or animals.

The authors provide the informed consent.

Additional information

Publisher’s note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Peruzzi, L., Coppo, R. IgA vasculitis nephritis in children and adults: one or different entities?. Pediatr Nephrol 36, 2615–2625 (2021). https://doi.org/10.1007/s00467-020-04818-7

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00467-020-04818-7

Keywords

Search

Navigation