Immunoglobulin A vasculitis (Henoch-Schönlein purpura) in children - A Literature Review
DOI:
https://doi.org/10.25753/BirthGrowthMJ.v32.i3.27759Keywords:
child, diagnosis, epidemiology, IgA vasculitis, physiopathology, therapyAbstract
Immunoglobulin A vasculitis (IgAV) is a small-vessel vasculitis that primarily affects children. Major manifestations include purpuric rash, proteinuria/hematuria, arthralgia, and abdominal pain. In this article, the authors review data on the epidemiology, diagnosis, and treatment of IgAV in children.
IgAV affects 10 to 56 children per 100,000 per year. The mean age at presentation is six years. Both genetic and environmental factors contribute to its pathogenesis, but the deposition of immune complexes containing abnormal glycosylated immunoglobulin (Ig) A1 plays a predominant role.
The course of the disease is usually benign and supportive care is sufficient. Short-term complications are mostly related to gastrointestinal involvement, including the risk of perforation and bleeding. Long-term morbidity is due to chronic kidney disease and hypertension. Corticosteroids are not recommended for prevention of renal involvement, but may be useful as a treatment strategy, as well as more aggressive immunosuppressive drugs.
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References
Wang K, Sun X, Cao Y, Dai L, Sun F, Yu P, et al. Risk factors for renal involvement and severe kidney disease in 2731 Chinese children with Henoch-Schönlein purpura: A retrospective study. Medicine (Baltimore). 2018;97(38):e12520. doi: https://doi.org/10.1097/MD.0000000000012520.
González LM, Janniger CK, Schwartz RA. Pediatric Henoch-Schönlein purpura. Int J Dermatol. 2009;48(11):1157-65. doi: https://doi.org/10.1111/j.1365-4632.2009.04162.x.
Schönlein JL. Allgemeine und Specielle Pathologie und Therapie, Vol. 2, 3rd edn. Wurzburg: Herisau, 1837: 48.
Henoch EH. Über eine eigenthumliche Form von Purpura. Berl KlinWochenschr 1874; 11: 641–3.
Gairdner D. The Schönlein–Henoch syndrome (anaphylactoid purpura). Q JMed 1948; 17: 95–122.
Ozen S, Pistorio A, Iusan SM, Bakkaloglu A, Herlin T, Brik R, et al. EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria. Ann Rheum Dis. 2010 May;69(5):798-806. doi: https://doi.org/10.1136/ard.2009.116657.
Yang YH, Yu HH, Chiang BL. The diagnosis and classification of Henoch-Schönlein purpura: an updated review. Autoimmun Rev. 2014;13(4-5):355-8. doi: https://doi.org/10.1016/j.autrev.2014.01.031. Epub 2014 Jan 12.
Pillebout E, Sunderkötter C. IgA vasculitis. Semin Immunopathol. 2021;43(5):729-38. doi: https://doi.org/10.1007/s00281-021-00874-9. Epub 2021 Jun 25.
Hetland LE, Susrud KS, Lindahl KH, Bygum A. Henoch-Schönlein Purpura: A Literature Review. Acta Derm Venereol. 2017;97(10):1160-6. doi: https://doi.org/10.2340/00015555-2733.
Chan H, Tang YL, Lv XH, Zhang GF, Wang M, Yang HP, et al. Risk Factors Associated with Renal Involvement in Childhood Henoch-Schönlein Purpura: A Meta-Analysis. PLoS One. 2016;11(11):e0167346. doi: https://doi.org/10.1371/journal.pone.0167346.
Xi L, Xu S, Jiang Y, Su H, Sun Y, Wen Y, et al. Predictive Value of Laboratory Indexes on Renal Involvement in Children with Henoch-Schönlein Purpura. Int J Gen Med. 2021;14:7951-9. doi: https://doi.org/10.2147/IJGM.S337437.
Jelusic M, Sestan M, Giani T, Cimaz R. New Insights and Challenges Associated With IgA Vasculitis and IgA Vasculitis With Nephritis-Is It Time to Change the Paradigm of the Most Common Systemic Vasculitis in Childhood? Front Pediatr. 2022;10:853724. doi: https://doi.org/10.3389/fped.2022.853724.
Zhao YL, Liu ZJ, Bai XM, Wang YC, Li GH, Yan XY. Obesity increases the risk of renal involvement in children with Henoch-Schönlein purpura. Eur J Pediatr. 2015;174(10):1357-63. doi: https://doi.org/10.1007/s00431-015-2547-z. Epub 2015 Apr 22.
Bérubé MD, Blais N, Lanthier S. Neurologic manifestations of Henoch-Schönlein purpura. Handb Clin Neurol. 2014;120:1101-11. doi: https://doi.org/10.1016/B978-0-7020-4087-0.00074-7.
Karakayali B, Yilmaz S, Çakir D, Günes PG, Güven S, Islek I. Henoch-Schonlein purpura associated with primary active Epstein-Barr virus infection: a case report. Pan Afr Med J. 2017;27:29. doi: https://doi.org/10.11604/pamj.2017.27.29.10481.
Hwang HH, Lim IS, Choi BS, Yi DY. Analysis of seasonal tendencies in pediatric Henoch-Schönlein purpura and comparison with outbreak of infectious diseases. Medicine (Baltimore). 2018;97(36):e12217. doi: https://doi.org/10.1097/MD.0000000000012217.
Borocco C, Lafay C, Plantard I, Gottlieb J, Koné-Paut I, Galeotti C. SARS-CoV-2-associated Henoch-Schönlein purpura in a 13-year-old girl. Arch Pediatr. 2021;28(7):573-5. doi: https://doi.org/10.1016/j.arcped.2021.06.004. Epub 2021 Jul 9.
AlGhoozi DA, AlKhayyat HM. A child with Henoch-Schonlein purpura secondary to a COVID-19 infection. BMJ Case Rep. 2021;14(1):e239910. doi: https://doi.org/10.1136/bcr-2020-239910. PMID: 33408113; PMCID: PMC7789096.
Sirufo MM, Raggiunti M, Magnanimi LM, Ginaldi L, De Martinis M. Henoch-Schönlein Purpura Following the First Dose of COVID-19 Viral Vector Vaccine: A Case Report. Vaccines (Basel). 2021;9(10):1078. doi: https://doi.org/10.3390/vaccines9101078.
Gálvez-Olortegui J, Álvarez-Vargas M, Durand-Vergara J, Díaz-Lozano M, Gálvez-Olortegui T, Armas-Ramírez I, et al. Henoch Schonlein purpura associated with bee sting: case report. Medwave. 2015;15(9):e6297. English, Spanish. doi: https://doi.org/10.5867/medwave.2015.09.6297.
Lau KK, Suzuki H, Novak J, Wyatt RJ. Pathogenesis of Henoch-Schönlein purpura nephritis. Pediatr Nephrol. 2010;25(1):19-26. doi: 10.1007/s00467-009-1230-x. Epub 2009 Jun 13. Erratum in: Pediatr Nephrol. 2010;25(1):179.
Heineke MH, Ballering AV, Jamin A, Ben Mkaddem S, Monteiro RC, Van Egmond M. New insights in the pathogenesis of immunoglobulin A vasculitis (Henoch-Schönlein purpura). Autoimmun Rev. 2017;16(12):1246-53. doi: https://doi.org/10.1016/j.autrev.2017.10.009. Epub 2017 Oct 14.
Zhang F, Chen L, Shang S, Jiang K. Atypical purpura location in a pediatric patient with Henoch-Schönlein purpura: A case report. Medicine (Baltimore). 2018;97(48):e13294. doi: https://doi.org/10.1097/MD.0000000000013294.
Chen JY, Mao JH. Henoch-Schönlein purpura nephritis in children: incidence, pathogenesis and management. World J Pediatr. 2015;11(1):29-34. doi: https://doi.org/10.1007/s12519-014-0534-5. Epub 2014 Dec 29. PMID: 25557596.
Garzoni L, Vanoni F, Rizzi M, Simonetti GD, Simonetti BG, Ramelli GP, et al. Nervous system dysfunction in Henoch-Schonlein syndrome: systematic review of the literature. Rheumatology (Oxford). 2009;48(12):1524-9. doi: https://doi.org/10.1093/rheumatology/kep282. Epub 2009 Sep 21.
Belman AL, Leicher CR, Moshé SL, Mezey AP. Neurologic manifestations of Schoenlein-Henoch purpura: report of three cases and review of the literature. Pediatrics. 1985;75(4):687-92.
Di Pietro GM, Castellazzi ML, Mastrangelo A, Montini G, Marchisio P, Tagliablue C. Henoch-Schönlein Purpura in children: not only kidney but also lung. Pediatr Rheumatol Online J. 2019;17(1):75. doi: https://doi.org/10.1186/s12969-019-0381-y.
Gedalia A. Henoch-Schönlein purpura. Curr Rheumatol Rep. 2004;6(3):195-202. doi: https://doi.org/10.1007/s11926-004-0068-2.
Saulsbury FT. Clinical update: Henoch-Schönlein purpura. Lancet. 2007;369(9566):976-8. doi: https://doi.org/10.1016/S0140-6736(07)60474-7.
Du L, Wang P, Liu C, Li S, Yue S, Yang Y. Multisystemic manifestations of IgA vasculitis. Clin Rheumatol. 2021;40(1):43-52. doi: https://doi.org/10.1007/s10067-020-05166-5. Epub 2020 Jun 16.
Ozen S, Marks SD, Brogan P, Groot N, de Graeff N, Avcin T, et al. European consensus-based recommendations for diagnosis and treatment of immunoglobulin A vasculitis-the SHARE initiative. Rheumatology (Oxford). 2019;58(9):1607-16.doi: https://doi.org/10.1093/rheumatology/kez041.
Dyga K, Szczepańska M. IgA vasculitis with nephritis in children. Adv Clin Exp Med. 2020;29(4):513-9. doi: https://doi.org/10.17219/acem/112566.
Lin Q, Min Y, Li Y, Zhu Y, Song X, Xu Q, et al. Henoch-Schönlein purpura with hypocomplementemia. Pediatr Nephrol. 2012;27(5):801-6. doi: https://doi.org/10.1007/s00467-011-2070-z. Epub 2012 Jan 21.
Kidney Disease: Improving Global Outcomes (KDIGO) Glomerular Diseases Work Group. KDIGO 2021 Clinical Practice Guideline for the Management of Glomerular Diseases. Kidney Int. 2021;100(4S):S1-S276. doi: https://doi.org/10.1016/j.kint.2021.05.021.
Fellström BC, Barratt J, Cook H, Coppo R, Feehally J, de Fijter JW, et al. Targeted-release budesonide versus placebo in patients with IgA nephropathy (NEFIGAN): a double-blind, randomised, placebo-controlled phase 2b trial. Lancet. 2017;389(10084):2117-27. doi: https://doi.org/10.1016/S0140-6736(17)30550-0. Epub 2017 Mar 28.
Gohari A, Matsell DG, Mammen C, Goldman RD. Henoch-Schönlein purpura in children: Use of corticosteroids for prevention and treatment of renal disease. Can Fam Physician. 2020;66(12):895-7. doi: https://doi.org/10.46747/cfp.6612895.
Hahn D, Hodson EM, Willis NS, Craig JC. Interventions for preventing and treating kidney disease in Henoch-Schönlein Purpura (HSP). Cochrane Database Syst Rev. 2015;(8):CD005128. doi: https://doi.org/10.1002/14651858.CD005128.pub3.
Oni L, Sampath S. Childhood IgA Vasculitis (Henoch Schonlein Purpura)-Advances and Knowledge Gaps. Front Pediatr. 2019;7:257. doi: https://doi.org/10.3389/fped.2019.00257.
Sugino H, Sawada Y, Nakamura M. IgA Vasculitis: Etiology, Treatment, Biomarkers and Epigenetic Changes. Int J Mol Sci. 2021;22(14):7538. doi: https://doi.org/10.3390/ijms22147538.
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(9):916-20. doi: https://doi.org/10.1136/adc.2005.074641. Epub 2005 May 4.
Liao CH, Tsai M, Yang YH, Chiang BL, Wang LC. Onset age is a risk factor for refractory pediatric IgA vasculitis: a retrospective cohort study. Pediatr Rheumatol Online J. 2020;18(1):86. doi: https://doi.org/10.1186/s12969-020-00480-3.
Trnka P. Henoch-Schönlein purpura in children. J Paediatr Child Health. 2013;49(12):995-1003. doi: https://doi.org/10.1111/jpc.12403. Epub 2013 Oct 18.
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