The clinical spectrum of Henoch–Schönlein purpura in children: a single-center study

  • Şerife Gül Karadağ
  • Ayşe Tanatar
  • Hafize Emine Sönmez
  • Figen Çakmak
  • Aysel Kıyak
  • Sevgi Yavuz
  • Mustafa Çakan
  • Nuray Aktay AyazEmail author
Original Article



Henoch–Schönlein purpura (HSP) is the most common vasculitis of children. The aim of this study is to evaluate the demographic and clinic findings of patients with HSP and also to determine predictive factors for assessing the development of gastrointestinal system (GIS) and renal involvement.


This study was performed prospectively among children with HSP who are under 18 years of age and being followed-up in the Pediatric Rheumatology Unit of Health Sciences University Kanuni Sultan Süleyman Training and Research Hospital between January 2016 and January 2018.


A total of 265 patients, 137 boys (51.7%) and 128 girls (48.3%), were involved to the study. The mean ± standard deviation of age at the diagnosis was 7.5 ± 3.2. The most common disease onset season was spring (31.7%). The rate of arthritis, GIS involvement, and renal involvement were 54%, 51.3%, and 29.1%, respectively. GIS bleeding was more frequent in males than females (p = 0.007). Boys over 7 years of age had significantly more common GIS bleeding (p = 0.04). Intussusception, relapse, and serious GIS involvement requiring hospitalization and steroid treatment were highly associated with severe renal involvement.


We demonstrated that patients suffering intussusception, relapse, and serious GIS involvement or requiring hospitalization and steroid treatment had tendency to present with severe renal involvement. Therefore, these patients should be followed up carefully for not overlooking renal involvement of HSP.


Children Gastrointestinal system involvement Henoch–Schönlein purpura Intussusception Relapse Renal involvement 


Compliance with ethical standards

The study protocol was approved by the local Ethical Committee. All patients were anonymous. The parents and patients gave a general consent approving anonymous data use for academic purpose. The study protocol was approved by the local Ethical Committee




  1. 1.
    Gardner-Medwin JM, Dolezalova P, Cummins C, Southwood TR (2002) Incidence of Henoch-Schonlein purpura, Kawasaki disease, and rare vasculitides in children of different ethnic origins. Lancet 360(9341):1197–1202. CrossRefGoogle Scholar
  2. 2.
    Yang YH, Hung CF, Hsu CR, Wang LC, Chuang YH, Lin YT, Chiang BL (2005) A nationwide survey on epidemiological characteristics of childhood Henoch-Schonlein purpura in Taiwan. Rheumatology (Oxford) 44(5):618–622. CrossRefGoogle Scholar
  3. 3.
    Ozen S, Bakkaloglu A, Dusunsel R, Soylemezoglu O, Ozaltin F, Poyrazoglu H, Kasapcopur O, Ozkaya O, Yalcinkaya F, Balat A, Kural N, Donmez O, Alpay H, Anarat A, Mir S, Gur-Guven A, Sonmez F, Gok F, Turkish Pediatric Vasculitis Study G (2007) Childhood vasculitides in Turkey: a nationwide survey. Clin Rheumatol 26(2):196–200. Google Scholar
  4. 4.
    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 O (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(5):798–806. CrossRefGoogle Scholar
  5. 5.
    Weiss PF (2012) Pediatric vasculitis. Pediatr Clin N Am 59(2):407–423. CrossRefGoogle Scholar
  6. 6.
    Narchi H (2005) 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 90(9):916–920. CrossRefGoogle Scholar
  7. 7.
    Lei WT, Tsai PL, Chu SH, Kao YH, Lin CY, Fang LC, Shyur SD, Lin YW, Wu SI (2018) Incidence and risk factors for recurrent Henoch-Schonlein purpura in children from a 16-year nationwide database. Pediatr Rheumatol Online J 16(1):25. CrossRefGoogle Scholar
  8. 8.
    Working Group of the International Ig ANN, the Renal Pathology S, 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(5):534–545. CrossRefGoogle Scholar
  9. 9.
    Working Group of the International Ig ANN, the Renal Pathology S, 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(5):546–556. CrossRefGoogle Scholar
  10. 10.
    Yalcinkaya F, Ozen S, Ozcakar ZB, Aktay N, Cakar N, Duzova A, Kasapcopur O, Elhan AH, Doganay B, Ekim M, Kara N, Uncu N, Bakkaloglu A (2009) A new set of criteria for the diagnosis of familial Mediterranean fever in childhood. Rheumatology (Oxford, England) 48(4):395–398. CrossRefGoogle Scholar
  11. 11.
    Paul Brogan, Arvind Bagga, Leukocytoclastic vasculitis. In Ross E. Petty, Ronald M. Laser, Carol B., Lindsley, Lucy R. Wedderburn (2016) Textbook of Pediatric Rheumatology (7th ed) 452–460Google Scholar
  12. 12.
    Trnka P (2013) Henoch-Schonlein purpura in children. J Paediatr Child Health 49(12):995–1003. CrossRefGoogle Scholar
  13. 13.
    Cakir M, Orhan F, Mungan I, Sonmez FM, Aslan Y, Kalyoncu M, Erduran E, Gedik Y, Okten A (2006) Henoch-Schonlein purpura in north-eastern Turkey. Ann Trop Paediatr 26(1):59–65. CrossRefGoogle Scholar
  14. 14.
    Anil M, Aksu N, Kara OD, Bal A, Anil AB, Yavascan O, Un B (2009) Henoch-Schonlein purpura in children from western Turkey: a retrospective analysis of 430 cases. Turk J Pediatr 51(5):429–436Google Scholar
  15. 15.
    Peru H, Soylemezoglu O, Bakkaloglu SA, Elmas S, Bozkaya D, Elmaci AM, Kara F, Buyan N, Hasanoglu E (2008) Henoch Schonlein purpura in childhood: clinical analysis of 254 cases over a 3-year period. Clin Rheumatol 27(9):1087–1092. CrossRefGoogle Scholar
  16. 16.
    Tabel Y, Inanc FC, Dogan DG, Elmas AT (2012) Clinical features of children with Henoch-Schonlein purpura: risk factors associated with renal involvement. Iran J Kidney Dis 6(4):269–274Google Scholar
  17. 17.
    Mao Y, Yin L, Huang H, Zhou Z, Chen T, Zhou W (2014) Henoch-Schonlein purpura in 535 Chinese children: clinical features and risk factors for renal involvement. J Int Med Res 42(4):1043–1049. CrossRefGoogle Scholar
  18. 18.
    Aalberse J, Dolman K, Ramnath G, Pereira RR, Davin JC (2007) Henoch Schonlein purpura in children: an epidemiological study among Dutch paediatricians on incidence and diagnostic criteria. Ann Rheum Dis 66(12):1648–1650. CrossRefGoogle Scholar
  19. 19.
    Wang X, Zhu Y, Gao L, Wei S, Zhen Y, Ma Q (2016) Henoch-Schonlein purpura with joint involvement: analysis of 71 cases. Pediatr Rheumatol Online J 14(1):20. CrossRefGoogle Scholar
  20. 20.
    Chen O, Zhu XB, Ren P, Wang YB, Sun RP, Wei DE (2013) Henoch Schonlein Purpura in children: clinical analysis of 120 cases. Afr Health Sci 13(1):94–99. Google Scholar
  21. 21.
    Calvo-Rio V, Loricera J, Mata C, Martin L, Ortiz-Sanjuan F, Alvarez L, Gonzalez-Vela MC, Gonzalez-Lamuno D, Rueda-Gotor J, Fernandez-Llaca H, Gonzalez-Lopez MA, Armesto S, Peiro E, Arias M, Gonzalez-Gay MA, Blanco R (2014) Henoch-Schonlein purpura in northern Spain: clinical spectrum of the disease in 417 patients from a single center. Medicine (Baltimore) 93(2):106–113. CrossRefGoogle Scholar
  22. 22.
    Hwang HH, Lim IS, Choi BS, Yi DY (2018) Analysis of seasonal tendencies in pediatric Henoch-Schonlein purpura and comparison with outbreak of infectious diseases. Medicine (Baltimore) 97(36):e12217. CrossRefGoogle Scholar
  23. 23.
    al Harbi NN (1996) Henoch-Schoenlein syndrome in children: experience from southern part of Saudi Arabia. East Afr Med J 73(3):191–193Google Scholar
  24. 24.
    Fretzayas A, Sionti I, Moustaki M, Papadimitriou A, Nicolaidou P (2008) Henoch-Schonlein purpura: a long-term prospective study in Greek children. J Clin Rheumatol 14(6):324–331. CrossRefGoogle Scholar
  25. 25.
    Piram M, Maldini C, Biscardi S, De Suremain N, Orzechowski C, Georget E, Regnard D, Kone-Paut I, Mahr A (2017) Incidence of IgA vasculitis in children estimated by four-source capture-recapture analysis: a population-based study. Rheumatology (Oxford) 56(8):1358–1366. CrossRefGoogle Scholar
  26. 26.
    Trapani S, Micheli A, Grisolia F, Resti M, Chiappini E, Falcini F, De Martino M (2005) Henoch Schonlein purpura in childhood: epidemiological and clinical analysis of 150 cases over a 5-year period and review of literature. Semin Arthritis Rheum 35(3):143–153. CrossRefGoogle Scholar
  27. 27.
    Shim JO, Han K, Park S, Kim GH, Ko JS, Chung JY (2018) Ten-year nationwide population-based survey on the characteristics of children with Henoch-Schnlein purpura in Korea. J Korean Med Sci 33(25):e174. CrossRefGoogle Scholar
  28. 28.
    Weiss PF, Klink AJ, Luan X, Feudtner C (2010) Temporal association of Streptococcus, Staphylococcus, and parainfluenza pediatric hospitalizations and hospitalized cases of Henoch-Schonlein purpura. J Rheumatol 37(12):2587–2594. CrossRefGoogle Scholar
  29. 29.
    Lippl F, Huber W, Werner M, Nekarda H, Berger H, Weigert N (2001) Life-threatening gastrointestinal bleeding due to a jejunal lesion of Henoch-Schonlein purpura. Endoscopy 33(9):811–813. CrossRefGoogle Scholar
  30. 30.
    Chen SY, Kong MS (2004) Gastrointestinal manifestations and complications of Henoch-Schonlein purpura. Chang Gung Med J 27(3):175–181Google Scholar
  31. 31.
    Zaffanello M, Brugnara M, Franchini M (2007) Therapy for children with henoch-schonlein purpura nephritis: a systematic review. ScientificWorldJournal 7:20–30. CrossRefGoogle Scholar
  32. 32.
    Donghi D, Schanz U, Sahrbacher U, Recher M, Trueb RM, Mullhaupt B, French LE, Hafner J (2009) Life-threatening or organ-impairing Henoch-Schonlein purpura: plasmapheresis may save lives and limit organ damage. Dermatology 219(2):167–170. CrossRefGoogle Scholar
  33. 33.
    Shah R, Ramakrishnan M, Vollmar A, Harrell A, Van Trump R, Masoud A (2017) Henoch-Schonlein purpura presenting as severe gastrointestinal and renal involvement with mixed outcomes in an adult patient. Cureus 9(3):e1088. Google Scholar
  34. 34.
    Basaran O, Cakar N, Uncu N, Celikel BA, Kara A, Cayci FS, Taktak A, Gur G (2015) Plasma exchange therapy for severe gastrointestinal involvement of Henoch Schonlein purpura in children. Clin Exp Rheumatol 33(2 Suppl 89):S-176-180Google Scholar
  35. 35.
    Ebert EC (2008) Gastrointestinal manifestations of Henoch-Schonlein Purpura. Dig Dis Sci 53(8):2011–2019. CrossRefGoogle Scholar
  36. 36.
    Zhang Q, Guo Q, Gui M, Ren Z, Hu B, Lu L, Deng F (2018) Henoch-Schonlein purpura with acute pancreatitis: analysis of 13 cases. BMC Pediatr 18(1):159. CrossRefGoogle Scholar
  37. 37.
    Buscatti IM, Casella BB, Aikawa NE, Watanabe A, Farhat SCL, Campos LMA, Silva CA (2018) Henoch-Schonlein purpura nephritis: initial risk factors and outcomes in a Latin American tertiary center. Clin Rheumatol 37(5):1319–1324. CrossRefGoogle Scholar
  38. 38.
    Jauhola O, Ronkainen J, Koskimies O, Ala-Houhala M, Arikoski P, Holtta T, Jahnukainen T, Rajantie J, Ormala T, Turtinen J, Nuutinen M (2010) Renal manifestations of Henoch-Schonlein purpura in a 6-month prospective study of 223 children. Arch Dis Child 95(11):877–882. CrossRefGoogle Scholar
  39. 39.
    Chang WL, Yang YH, Wang LC, Lin YT, Chiang BL (2005) Renal manifestations in Henoch-Schonlein purpura: a 10-year clinical study. Pediatr Nephrol 20(9):1269–1272. CrossRefGoogle Scholar
  40. 40.
    Wang K, Sun X, Cao Y, Dai L, Sun F, Yu P, Dong L (2018) Risk factors for renal involvement and severe kidney disease in 2731 Chinese children with Henoch-Schonlein purpura: a retrospective study. Medicine (Baltimore) 97(38):e12520. CrossRefGoogle Scholar
  41. 41.
    Teng MC, Wang LC, Yu HH, Lee JH, Yang YH, Chiang BL (2012) Kawasaki disease and Henoch-Schonlein purpura—10 years’ experience of childhood vasculitis at a university hospital in Taiwan. J Microbiol Immunol Infect 45(1):22–30. CrossRefGoogle Scholar
  42. 42.
    Calvo-Rio V, Hernandez JL, Ortiz-Sanjuan F, Loricera J, Palmou-Fontana N, Gonzalez-Vela MC, Gonzalez-Lamuno D, Gonzalez-Lopez MA, Armesto S, Blanco R, Gonzalez-Gay MA (2016) Relapses in patients with Henoch-Schonlein purpura: analysis of 417 patients from a single center. Medicine (Baltimore) 95(28):e4217. CrossRefGoogle Scholar
  43. 43.
    Prais D, Amir J, Nussinovitch M (2007) Recurrent Henoch-Schonlein purpura in children. J Clin Rheumatol 13(1):25–28. CrossRefGoogle Scholar
  44. 44.
    Ozcakar ZB, Yalcinkaya F, Cakar N, Acar B, Kasapcopur O, Uguten D, Soy D, Kara N, Uncu N, Arisoy N, Ekim M (2008) MEFV mutations modify the clinical presentation of Henoch-Schonlein purpura. J Rheumatol 35(12):2427–2429CrossRefGoogle Scholar
  45. 45.
    Bayram C, Demircin G, Erdogan O, Bulbul M, Caltik A, Akyuz SG (2011) Prevalence of MEFV gene mutations and their clinical correlations in Turkish children with Henoch-Schonlein purpura. Acta Paediatr 100(5):745–749. CrossRefGoogle Scholar
  46. 46.
    Gershoni-Baruch R, Broza Y, Brik R (2003) Prevalence and significance of mutations in the familial Mediterranean fever gene in Henoch-Schonlein purpura. J Pediatr 143(5):658–661. CrossRefGoogle Scholar
  47. 47.
    Salah S, Rizk S, Lotfy HM, El Houchi S, Marzouk H, Farag Y (2014) MEFV gene mutations in Egyptian children with Henoch-Schonlein purpura. Pediatr Rheumatol Online J 12:41. CrossRefGoogle Scholar

Copyright information

© International League of Associations for Rheumatology (ILAR) 2019

Authors and Affiliations

  • Şerife Gül Karadağ
    • 1
  • Ayşe Tanatar
    • 1
  • Hafize Emine Sönmez
    • 1
  • Figen Çakmak
    • 1
  • Aysel Kıyak
    • 2
  • Sevgi Yavuz
    • 2
  • Mustafa Çakan
    • 1
  • Nuray Aktay Ayaz
    • 1
    Email author
  1. 1.Kanuni Sultan Süleyman Research and Training Hospital, Department of Pediatric RheumatologyUniversity of Health ScienceIstanbulTurkey
  2. 2.Kanuni Sultan Süleyman Research and Training Hospital, Department of Pediatric NephrologyUniversity of Health ScienceIstanbulTurkey

Personalised recommendations