Exploring the Pathophysiology of Henoch-Schönlein Purpura (HSP): A Comprehensive Review

Year : 2024 | Volume : | | Page :
    By

    Himanshu Jain,

  • Kuljeet Kaur,

  1. Lecturer, Department of Pharmaceutical Sciences, University Institute of pharmaceutical sciences and research, Baba Farid University of Health sciences, Punjab, India
  2. Research Scholar, Department of Pharmaceutical Sciences, University Institute of pharmaceutical sciences and research, Baba Farid University of Health sciences, Punjab, India

Abstract

Blood from tiny veins can leak into nearby tissues, causing purpura, a disorder marked by red spots or patches on the skin that may have an impact on the kidneys. Henoch-Schönlein purpura (HSP) is associated with vasculitis and inflammation of blood vessels. The range of renal conditions associated with purpura includes severe glomerulonephritis and mild proteinuria. For instance, immunological complex accumulation in the renal glomeruli of HSP patients might result in inflammation and renal injury. The pathophysiology of HSP is largely dependent on immunological pathways, including complement activation and immune complex deposition. Effective management of these renal symptoms depends on rapid identification and action. In addition to skin discoloration and renal involvement, purpura may present with other symptoms, including fatigue, joint pain, and mucosal bleeding. Though the precise etiology of HSP is unknown, it is thought to be related to an unbalanced immune system reaction that may be brought on by an infection or other circumstances. Treatment strategies depend on the underlying cause and may involve immunosuppressive therapy, plasmapheresis, or supportive care. For the most effective treatment for patients with HSP, a multidisciplinary strategy comprising gastroenterologists, nephrologists, and rheumatologists is recommended. Supportive treatment for gastrointestinal and renal disorders may include fluid replacement, pain relief, and, in certain situations, immunosuppressive medication. A long-term follow-up is necessary to evaluate renal function and keep an eye out for disease recurrence. This study aims to give a thorough overview of HSP, including information on its pathophysiology, diagnosis, possibilities for treatment, and clinical signs and symptoms.

Keywords: Henoch-Schönlein purpura (HSP), renal disorder, IgA vasculitis, galactose-deficient IgA1, anti-endothelial cell antibodies, Nephritic

How to cite this article:
Himanshu Jain, Kuljeet Kaur. Exploring the Pathophysiology of Henoch-Schönlein Purpura (HSP): A Comprehensive Review. International Journal of Cell Biology and Cellular Functions. 2024; ():-.
How to cite this URL:
Himanshu Jain, Kuljeet Kaur. Exploring the Pathophysiology of Henoch-Schönlein Purpura (HSP): A Comprehensive Review. International Journal of Cell Biology and Cellular Functions. 2024; ():-. Available from: https://journals.stmjournals.com/ijcbcf/article=2024/view=147909


References

1. Jaszczura M, Dyga K, Bryłka A, Góra A, Machura E. IgA vasculitis, formerly known as Henoch–Schönlein purpura–the most common vasculitis in children. Pediatr Pol. 2018;93:336–42. DOI: 10.5114/polp.2018.78000.
2. Trnka PH. Henoch-Schönlein purpura in children. J Paediatr Child Health. 2013;49:995–1003. DOI: 10.1111/jpc.12403.
3. Heineke MH, Ballering AV, Jamin A, Ben Mkaddem SB, Monteiro RC, Van Egmond M. New insights in the pathogenesis of immunoglobulin A vasculitis (Henoch-Schönlein purpura). Autoimmun Rev. 2017;16:1246–53. DOI: 10.1016/j.autrev.2017.10.009.
4. Trapani S, Micheli A, Grisolia F, Resti M, Chiappini E, Falcini F, De Martino M. Henoch Schonlein purpura in childhood: Epidemiological and clinical analysis of 150 cases over a 5-year period and review of literature. Rheumatism. 2005;35:143–53. DOI: 10.1016/j.semarthrit.2005.08.007.
5. Gardner-Medwin JM, Dolezalova P, Cummins C, Southwood TR. Incidence of Henoch-Schonlein purpura, Kawasaki disease, and rare vasculitides in children of different ethnic origins. Lancet. 2002;360:1197–202. DOI: 10.1016/S0140-6736(02)11279-7.
6. Calviño MC, Llorca J, García-Porrúa C, Fernández-Iglesias JL, Rodriguez-Ledo P, González-Gay MA. Henoch-Schönlein purpura in children from northwestern Spain: A 20-year epidemiologic and clinical study. Medicine. 2001;80:279–90. DOI: 10.1097/00005792-200109000-00001.
7. Watson L, Richardson ARW, Holt RCL, Jones CA, Beresford MW. Henoch Schonlein Purpura – A 5-Year Review and Proposed Pathway. PLOS ONE. 2012;7. DOI: 10.1371/journal.pone.002
9512.
8. Tizard EJ. Henoch-Schönlein purpura. Arch Dis Child. 1999;80:380–3. DOI: 10.1136/adc.80.4.
380.
9. Prais D, Amir J, Nussinovitch M. Recurrent Henoch-Schönlein purpura in children. J Clin Rheumatol. 2007;13:25–8. DOI: 10.1097/01.rhu.0000255692.46165.19.
10. Calvo-Río V, Hernández JL, Ortiz-Sanjuán F, Loricera J, Palmou-Fontana N, González-Vela MC, et al. Relapses in patients with Henoch–Schönlein purpura: Analysis of 417 patients from a single center. Medicine. 2016;95. DOI: 10.1097/MD.0000000000004217.
11. Lei WT, Tsai PL, Chu SH, Kao YH, Lin CY, Fang LC, et al. Incidence and risk factors for recurrent Henoch-Schönlein purpura in children from a 16-year nationwide database. Pediatr Rheumatol Online J. 2018;16:25. DOI: 10.1186/s12969-018-0247-8.
12. Piram M, Mahr A. Epidemiology of immunoglobulin A vasculitis (Henoch–Schönlein): Current state of knowledge. Curr Opin Rheumatol. 2013;25:171–8. DOI: 10.1097/BOR.0b013e32835d
8e2a.
13. Saulsbury FT. Henoch–Schönlein purpura. Curr Opin Rheumatol. 2010;22:598–602. DOI: 10.1097/BOR.0b013e32833af608.
14. Butt G, Anees M, Rana M. Frequency of renal manifestations in patients of Henoch-Schonlein purpura. J Pak Assoc Dermatol. 2021;31:146–52.
15. Hetland LE, Susrud KS, Lindahl KH, Bygum A. Henoch-Schönlein purpura: A literature review. Acta Derm Venereol. 2017;97:1160–6. DOI: 10.2340/00015555-2733.
16. Sag E, Arici ZS, Ozen S. IgA vasculitis (Henoch–Schönlein purpura) in children. Expert Opin Orphan Drugs. 2017;5:405–10. DOI: 10.1080/21678707.2017.1311783.
17. Abbas S, Geetha S, Deepthi RV, Kamar J, Uthup S. Clinical profile and outcome of Henoch Schonlein purpura in a tertiary care hospital in South India. Int J Contemp Pediatr. 2017;4:822–6. DOI: 10.18203/2349-3291.ijcp20171493.
18. 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. 2019;58:1607–16. DOI: 10.1093/rheumatology/kez041.
19. Audemard-Verger A, Pillebout E, Guillevin L, Thervet E, Terrier B. IgA vasculitis (Henoch–Shönlein purpura) in adults: Diagnostic and therapeutic aspects. Autoimmun Rev. 2015;14:579–85. DOI: 10.1016/j.autrev.2015.02.003.
20. Calvo-Río V, Loricera J, Mata C, Martín L, Ortiz-Sanjuán F, Alvarez L, et al. Henoch-Schönlein purpura in northern Spain: Clinical spectrum of the disease in 417 patients from a single center. Medicine. 2014;93:106–13. DOI: 10.1097/MD.0000000000000019.
21. Alharthi AA. Henoch-Schonlein purpura in Saudi Arabia: A retrospective study of 27 children in Taif region. Curr Pediatr Res. 2016;20:126–31.
22. Watson L, Richardson ARW, Holt RCL, Jones CA, Beresford MW. Henoch Schonlein Purpura – A 5-year review and proposed pathway. PLOS ONE. 2012;7. DOI: 10.1371/journal.pone.0029512.
23. 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;174:1357–63. DOI: 10.1007/s00431-015-2547-z.
24. Narchi H. Risk of long term renal impairment and duration of follow up recommended for Henoch-Schönlein purpura with normal or minimal urinary findings: A systematic review. Arch Dis Child. 2005;90:916–20. DOI: 10.1136/adc.2005.074641.
25. Modi S, Mohan M, Jennings A. Acute scrotal swelling in Henoch-Schonlein purpura: Case report and review of the literature. Urol Case Rep. 2016;6:9–11. DOI: 10.1016/j.eucr.2016.01.004.
26. Dos Santos D, Langer FW, Dos Santos T, Rafael Tronco Alves G, Feiten M, Teixeira de Paula Neto W. Posterior reversible encephalopathy syndrome as a complication of Henoch-Schönlein purpura in a seven-year-old girl. Scott Med J. 2017;62:34–7. DOI: 10.1177/0036933017690467. PubMed: 28152662.
27. Hwang JJ, Ahn J, Kim KP, Choi HI, Choi JY, Song R, et al. Henoch-Schönlein purpura with muscle involvement, presenting as myositis. J Clin Rheumatol. 2017;23:60–2. DOI: 10.1097/RHU.
0000000000000476. PubMed: 28002161.
28. Michas G, Grigoriou K, Syrigos D, Alexopoulos N, Evdoridis C, Trikas A. A rare cause of myocarditis resulting in acute heart failure in the setting of Henoch-Schönlein purpura. Hellenic J Cardiol. 2017;58:439–42. DOI: 10.1016/j.hjc.2017.05.002. PubMed: 28528257.
29. Woof JM, Kerr MA. The function of immunoglobulin A in immunity. J Pathol. 2006;208:270–82. DOI: 10.1002/path.1855. PubMed: 16961578.
30. Crago SS, Kutteh WH, Moro I, Allansmith MR, Radl J, Haaijman JJ, Mestecky J. Distribution of IgA1-, IgA2-, and J chain-containing cells in human tissues. J Immunol. 1984;132:16–8. DOI: 10.4049/jimmunol.132.1.16. PubMed: 6418796.
31. Roos A, Bouwman LH, van Gijlswijk-Janssen DJ, Faber-Krol MC, Stahl GL, Daha MR. Human IgA activates the complement system via the mannan-binding lectin pathway. J Immunol. 2001;167:2861–8. DOI: 10.4049/jimmunol.167.5.2861. PubMed: 11509633.
32. Hiemstra PS, Gorter A, Stuurman ME, Van Es LA, Daha MR. Activation of the alternative pathway of complement by human serum IgA. Eur J Immunol. 1987;17:321–6. DOI: 10.1002/eji.1830170
304. PubMed: 3569402.
33. Aleyd E, Heineke MH, van Egmond M. The era of the immunoglobulin A Fc receptor FcαRI: its function and potential as target in disease. Immunol Rev. 2015;268:123–38. DOI: 10.1111/imr.
12337.
34. Blank U, Launay P, Benhamou M, Monteiro RC. Inhibitory ITAMs as novel regulators of immunity. Immunol Rev. 2009;232:59–71. DOI: 10.1111/j.1600-065X.2009.00832.x. PubMed: 19909356.
35. Pasquier B, Launay P, Kanamaru Y, Moura IC, Pfirsch S, Ruffié C, Hénin D, Benhamou M, Pretolani M, Blank U, Monteiro RC. Identification of FcαRI as an inhibitory receptor that controls inflammation: dual role of FcRγ ITAM. Immunity. 2005;22:31–42. DOI: 10.1016/j.immuni.2004.
11.017. PubMed: 15664157.
36. Ben Mkaddem SB, Rossato E, Heming N, Monteiro RC. Anti-inflammatory role of the IgA Fc receptor (CD89): From autoimmunity to therapeutic perspectives. Autoimmun Rev. 2013;12:666–9. DOI: 10.1016/j.autrev.2012.10.011. PubMed: 23201915.
37. Heineke MH, van Egmond M. Immunoglobulin A: Magic bullet or Trojan horse? Eur J Clin Invest. 2017;47:184–92. DOI: 10.1111/eci.12716.
38. Aleyd E, van Hout MW, Ganzevles SH, Hoeben KA, Everts V, Bakema JE, van Egmond M. IgA enhances NETosis and release of neutrophil extracellular traps by polymorphonuclear cells via Fcα receptor I. J Immunol. 2014;192:2374–83. DOI: 10.4049/jimmunol.1300261. PubMed: 24493821.
39. van der Steen LP, Bakema JE, Sesarman A, Florea F, Tuk CW, Kirtschig G, Hage JJ, Sitaru C, van Egmond M. Blocking Fcα Receptor I on Granulocytes Prevents Tissue Damage Induced by IgA Autoantibodies. J Immunol. 2012;189:1594–601. DOI: 10.4049/jimmunol.1101763.
40. Jennewein MF, Alter G. The immunoregulatory roles of antibody glycosylation. Trends Immunol. 2017;38:358–72. DOI: 10.1016/j.it.2017.02.004. PubMed: 28385520.
41. Launay P, Grossetête B, Arcos-Fajardo M, Gaudin E, Torres SP, Beaudoin L, Patey-Mariaud de Serre N, Lehuen A, Monteiro RC. Fcα receptor (CD89) mediates the development of immunoglobulin A (IgA) nephropathy (Berger’s disease): evidence for pathogenic soluble receptor–IgA complexes in patients and CD89 transgenic mice. J Exp Med. 2000;191:1999–2009. DOI: 10.1084/jem.191.11.1999. PubMed: 10839814.
42. Tissandié E, Morelle W, Berthelot L, Vrtovsnik F, Daugas E, Walker F, Lebrec D, Trawalé JM, Francoz C, Durand F, Moura IC, Paradis V, Moreau R, Monteiro RC. Both IgA nephropathy and alcoholic cirrhosis feature abnormally glycosylated IgA1 and soluble CD89-IgA and IgG–IgA complexes: common mechanisms for distinct diseases. Kidney Int. 2011;80:1352–63. DOI: 10.1038/ki.2011.276. PubMed: 21866091.
43. Vuong MT, Hahn-Zoric M, Lundberg S, Gunnarsson I, Van Kooten C, Wramner L, Seddighzadeh M, Fernström A, Hanson LA, Do LT, Jacobson SH, Padyukov L. Association of soluble CD89 levels with disease progression but not susceptibility in IgA nephropathy. Kidney Int. 2010;78:1281–7. DOI: 10.1038/ki.2010.314. PubMed: 20811333.
44. Wilkinson A. Early recognition and treatment of Henoch-Schönlein purpura in children. Nurs Child Young People. 2019;31:36–40. DOI: 10.7748/ncyp.2019.e1118. PubMed: 31486599.
45. Watanabe T. Henoch-Schönlein purpura following influenza vaccinations during the pandemic of influenza A (H1N1). Pediatr Nephrol. 2011;26:795–8. DOI: 10.1007/s00467-010-1722-8. PubMed: 21120537.
46. Jariwala S, Vernon N, Shliozberg J. Henoch-Schönlein purpura after hepatitis A vaccination. Ann Allergy Asthma Immunol. 2011;107:180–1. DOI: 10.1016/j.anai.2011.05.006. PubMed: 21802028.
47. Mitsui H, Shibagaki N, Kawamura T, Matsue H, Shimada S. A clinical study of Henoch-Schönlein purpura associated with malignancy. J Eur Acad Dermatol Venereol. 2009;23:394–401. DOI: 10.1111/j.1468-3083.2008.03065.x. PubMed: 19207675.
48. Blanco R, González-Gay MA, Ibáñez D, Alba C, Pérez de Llano LA. Henoch-Schönlein purpura as a clinical presentation of small cell lung cancer. Clin Exp Rheumatol. 1997;15:545–7.
49. Angelier AS, Petit L, Wynckel A, Vuiblet V, Birembaut P, Toubas O, et al. Schoenlein-Henoch purpura as a presentation of squamous cell bronchial carcinoma. Rev Mal Respir. 2011;28:372–6. DOI: 10.1016/j.rmr.2010.09.033. PubMed: 21482344.
50. Nozato K, Morishima Y, Furuta J, Fujita J, Miyazaki K, Ogawa R, et al. A case of Henoch-Schönlein purpura which was difficult to distinguish from a skin rash associated with gefitinib. Nihon Kokyuki Gakkai Zasshi. 2010;48:529–34.
51. Maritati F, Canzian A, Fenaroli P, Vaglio A. Adult-onset IgA vasculitis (Henoch-Schönlein): update on therapy. Presse Med. 2020;49:104035. DOI: 10.1016/j.lpm.2020.104035. PubMed: 32645417.
52. Kango SC, Palet JE, Bognin OB, Ouaimon D, Houndjahoue GF. Diagnostic difficulties in a case of Henoch-Schönlein purpura at the pediatric University Hospital complex of Bangui. J Med Case Rep Case Series. 2022;3:19.
53. Yang YH, Huang YH, Lin YL, Wang LC, Chuang YH, Yu HH, Lin YT, Chiang BL. Circulating IgA from acute stage of childhood Henoch-Schönlein purpura can enhance endothelial interleukin (IL)-8 production through MEK/ERK signalling pathway. Clin Exp Immunol. 2006;144:247–53. DOI: 10.1111/j.1365-2249.2006.03076.x. PubMed: 16634798.
54. Demir S, Candan C, Turhan P, Ergüven M. Henoch Schönlein Purpura / Ig A Vasculitis in Children and Risk Factors for Renal Involvement. Acta Medica. 2021;52:230–38. DOI: 10.32552/2021.
ActaMedica.585.
55. Szemenyei C, Hahn D. Prevention of nephritis in Henoch-Schönlein purpura. J Paediatr Child Health. 2015;51:236–39. DOI: 10.1111/jpc.12785. PubMed: 25677491.
56. Bogdanović R. Henoch-Schönlein purpura nephritis in children: Risk factors, prevention and treatment. Acta Paediatr. 2009;98:1882–89. DOI: 10.1111/j.1651-2227.2009.01445.x.
57. Atkinson SR, Barker DJ. Seasonal distribution of Henoch-Schönlein purpura. Br J Prev Soc Med. 1976;30:22–25. DOI: 10.1136/jech.30.1.22. PubMed: 949569.
58. Reamy BV, Servey JT, Williams PM. Henoch-Schönlein purpura (IgA Vasculitis): Rapid Evidence Review [IgA vasculitis]. Am Fam Physician. 2020;102:229–33. PubMed: 32803924.
59. Lee DH, Lee ES, Hong J, Park KH, Pai KS. A case of Henoch-Schönlein purpura with fulminant complications and its long-term outcome. Child Kidney Dis. 2019;23:128–33. DOI: 10.3339/jkspn.2019.23.2.128.
60. Chan H, Tang YL, Lv XH, Zhang GF, Wang M, Yang HP, Li Q. Risk factors associated with renal involvement in childhood Henoch-Schönlein purpura: A meta-analysis. PLOS ONE. 2016;11. DOI: 10.1371/journal.pone.0167346.
61. Gaia MJ, Capela M, Borges JP, Marques E, Ferreira G, Vinhas da Silva A. Purpura HS. What to expect. Port J Nephrol Hypert, Lisboa. 2019;33:212–16.
62. Jelusic M, Sestan M, Cimaz R, Ozen S. Different histological classifications for Henoch-Schönlein purpura nephritis: Which one should be used? Pediatr Rheumatol Online J. 2019;17:10. DOI: 10.1186/s12969-019-0311-z. PubMed: 30819179.
63. 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;18:86. DOI: 10.1186/s12969-020-00480-3. PubMed: 33172497.
64. Wang X, Zhu Y, Gao L, Wei S, Zhen Y, Ma Q. Henoch-Schönlein purpura with joint involvement: Analysis of 71 cases. Pediatr Rheumatol. 2016;14:1–8.
65. Dawood SA, Abodiah AM, Alqahtani SM, Shati AA, Alqahtani YA, Alshehri MA, Mahmood SE. Clinico-epidemiological profile and outcome of children with IgA vasculitis in Aseer region, Southwestern Saudi Arabia. Inhealthcare. 2021 Dec 7;9(12):1694.
66. Pillebout E, Alberti C, Guillevin L, Ouslimani A, Thervet E; Cesar Study Group. Addition of cyclophosphamide to steroids provides no benefit compared with steroids alone in treating adult patients with severe Henoch Schönlein purpura. Kidney Int. 2010;78:495–502. DOI: 10.1038/ki.2010.150. PubMed: 20505654.
67. Han DH. Clinical analysis on 106 cases of Henoch–Schönlein purpura. J Intern Korean Med. 2007;28:570–85.
68. Yang YH, Yu HH, Chiang BL. The diagnosis and classification of Henoch–Schönlein purpura: An updated review. Autoimmun Rev. 2014;13:355–58. DOI: 10.1016/j.autrev.2014.01.031. PubMed: 24424188.
69. Van de Perre E, Jones RB, Jayne DRW. IgA vasculitis (Henoch–Schönlein purpura): Refractory and relapsing disease course in the adult population. Clin Kidney J. 2021;14:1953–60. DOI: 10.1093/ckj/sfaa251. PubMed: 34345419.
70. Maritati F, Fenoglio R, Pillebout E, Emmi G, Urban ML, Rocco R, Nicastro M, Incerti M, Goldoni M, Trivioli G, Silvestri E, Mohammad AJ, Jayne D, Eriksson P, Segelmark M, Novikov P, Harris H, Roccatello D, Vaglio A. Brief report: Rituximab for the treatment of adult-onset IgA vasculitis (Henoch-Schönlein). Arthritis Rheumatol. 2018;70:109–14. DOI: 10.1002/art.40339. PubMed: 28973844.
71. Audemard-Verger A, Terrier B, Dechartres A, Chanal J, Amoura Z, Le Gouellec N, Cacoub P, Jourde-Chiche N, Urbanski G, Augusto JF, Moulis G, Raffray L, Deroux A, Hummel A, Lioger B, Catroux M, Faguer S, Goutte J, Martis N, Maurier F, Rivière E, Sanges S, Baldolli A, Costedoat-Chalumeau N, Roriz M, Puéchal X, André M, Lavigne C, Bienvenu B, Mekinian A, Zagdoun E, Girard C, Bérezné A, Guillevin L, Thervet E, Pillebout E; French Vasculitis Study Group. Characteristics and management of IgA vasculitis (Henoch-Schönlein) in adults: Data from 260 patients included in a French multicenter retrospective survey. Arthritis Rheumatol. 2017;69:1862–70. DOI: 10.1002/art.40178. PubMed: 28605168.
72. 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;66:895–97. DOI: 10.46747/cfp.6612895. PubMed: 33334956.
73. Butt G, Anees M, Rana M. Frequency of renal manifestations in patients of Henoch-Schonlein purpura. J Pak Assoc Dermatol. 2021;31:146–52.


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Received 20/03/2024
Accepted 04/05/2024
Published 27/05/2024



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