Journal List > J Rheum Dis > v.19(5) > 1063986

Bae, Lee, Kim, Jung, Kim, Lee, Ahn, Park, Lee, Kang, Lee, Kim, and Suh: Initial Hematochezia and Kidney Involvement are Important Prognostic Factors of Adult Onset Henoch-Schönlein Purpura in Korea



Henoch-Schönlein purpura (HSP) is a systemic vasculitis, characterized by small-vessel leukocytoclastic vasculitis with the deposition of immune complexes containing IgA. It is the most common acute vasculitic disorder affecting children but is relatively uncommon in adults. We investigated the clinical features and factors affecting the prognosis of adult HSP in Korea.


From 1996 to 2011 seventy patients over 15 years of age with HSP were retrospectively analyzed.


Thirty eight patients (54.3%) were female and the age at disease onset ranged from 15 to 75 years (35.0±15.8 years). Purpuric skin rash was observed in all patients and arthralgia was present in 34 patients (48.6%). GI symptoms and kidney involvements were observed in 28 patients (40.0%) and 34 patients (48.6%), respectively. Complete remission was achieved in 46 patients (65.7%). The remission group showed a lower incidence of hematochezia (p=0.044), hematuria (p=0.008), and proteinuria (p=0.011) at diagnosis than the no remission group. About 10% of adult HSP patient progressed to chronic kidney disease (CKD), which showed higher a incidence of nephrotic range proteinuria. Only nephrotic range proteinuria at diagnosis was a significant risk factor for CKD (OR=16.7, p=0.008, 95% CI=2.1~133.1).


Hematochezia, hematuria and proteinuria at the diagnosis of HSP are important prognostic factors in predicting remission. In addition, HSP patients with nephrotic range proteinuria at diagnosis have an increased risk of renal failure.

Figures and Tables

Table 1
Clinical and demographic characteristics of 70 patients
Table 2
Clinical features of 70 patients
Table 3
Laboratory findings of 70 patients

WBC: white blood cell, ESR: erythrocyte sedimentation rate, CRP: C-reactive protein, AST: aspartate aminotransferase, ALT: alanine transaminases, C: complement, IgA: immunoglobulin A, ASO: anti-streptolysin O

Table 4
Outcome of 70 patients at last follow up
Table 5
Clinical characteristics and treatment according to complete remission

Steroid: prednisolone, Immunosuppressant: cyclophosphamide or azathioprine. These data were assessed using Fisher's exact test.

Table 6
Clinical characteristics and treatment of HSP patients with nephritis

CKD: chronic kidney disease, Renal biopsy findings classified by ISKDC, ISKDC: international study of kidney diseases in children, Steroid: prednisolone, Immunosuppressant: cyclophosphamide or azathioprine. These data were assessed using Fisher's exact test


1. Tsai CC, Giangiacomo J, Zuckner J. Letter: Dermal IgA deposits in Henoch-Schönlein purpura and Berger's nephritis. Lancet. 1975. 1:342–343.
2. Davin JC, Weening JJ. Henoch-Schönlein purpura nephritis: an update. Eur J Pediatr. 2001. 160:689–695.
3. Tizard EJ. Henoch-Schönlein purpura. Arch Dis Child. 1999. 80:380–383.
4. Ilan Y, Naparstek Y. Henoch-Schönlein purpura in children and adults: is it one entity? Semin Arthritis Rheum. 2002. 32:139–140.
5. Blanco R, Martínez-Taboada VM, Rodríguez-Valverde V, García-Fuentes M, González-Gay MA. Henoch-Schönlein purpura in adulthood and childhood: two different expressions of the same syndrome. Arthritis Rheum. 1997. 40:859–864.
6. Michel BA, Hunder GG, Bloch DA, Calabrese LH. Hypersensitivity vasculitis and Henoch-Schönlein purpura: a comparison between the 2 disorders. J Rheumatol. 1992. 19:721–728.
7. García-Porrúa C, Calviño MC, Llorca J, Couselo JM, González-Gay MA. Henoch-Schönlein purpura in children and adults: clinical differences in a defined population. Semin Arthritis Rheum. 2002. 32:149–156.
8. Silverstein DM, Greifer I, Folkert V, Bennett B, Corey HE, Spitzer A. Sequential occurrence of IgA nephropathy and Henoch-Schönlein purpura: support for common pathogenesis. Pediatr Nephrol. 1994. 8:752–753.
9. Pillebout E, Thervet E, Hill G, Alberti C, Vanhille P, Nochy D. Henoch-Schönlein Purpura in adults: outcome and prognostic factors. J Am Soc Nephrol. 2002. 13:1271–1278.
10. Allen AC, Willis FR, Beattie TJ, Feehally J. Abnormal IgA glycosylation in Henoch-Schönlein purpura restricted to patients with clinical nephritis. Nephrol Dial Transplant. 1998. 13:930–934.
11. Rieu P, Noël LH. Henoch-Schönlein nephritis in children and adults. Morphological features and clinicopathological correlations. Ann Med Interne (Paris). 1999. 150:151–159.
12. Glasgow EF. Renal changes in Henoch-Schönlein purpura. Arch Dis Child. 1970. 45:151.
13. Counahan R, Winterborn MH, White RH, Heaton JM, Meadow SR, Bluett NH, et al. Prognosis of Henoch-Schönlein nephritis in children. Br Med J. 1977. 2:11–14.
14. Kim KE, Shin YH, Shin JI, Park JM, Lee JS, Jeong HJ. Clinical comparison of henoch-schonlein purpura nephritis in children and adults. J Korean Soc Pediatr Nephrol. 2003. 7:157–165.
15. Kang Y, Ha YJ, Lee KH, Jung SY, Lee SW, Lee SK, et al. Clinical manifestations of korean adult patients with Henoch-Schönlein purpura. J Korean Rheum Assoc. 2010. 17:133–142.
16. Ozen S, Pistorio A, Iusan SM, Bakkaloglu A, Herlin T, Brik R, et al. Paediatric Rheumatology International Trials Organisation (PRINTO). 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. 69:798–806.
17. Culic S, Jakl R, Metlicic V, Paukovic-Sekulic B, Resic B, Culic V, et al. Platelet function analysis in children with Schönlein-Henoch syndrome. Arch Med Res. 2001. 32:268–272.
18. Trygstad CW, Stiehm ER. Elevated serum IgA globulin in anaphylactoid purpura. Pediatrics. 1971. 47:1023–1028.
19. Casanueva B, Rodriguez-Valverde V, Merino J, Arias M, Garcia-Fuentes M. Increased IgA-producing cells in the blood of patients with active Henoch-Schnlein purpura. Arthritis Rheum. 1983. 26:854–860.
20. Davin JC, Ten Berge IJ, Weening JJ. What is the difference between IgA nephropathy and Henoch-Schönlein purpura nephritis? Kidney Int. 2001. 59:823–834.
21. Szer IS. Henoch-Schönlein purpura. Curr Opin Rheumatol. 1994. 6:25–31.
22. Baselga E, Drolet BA, Esterly NB. Purpura in infants and children. J Am Acad Dermatol. 1997. 37:673–705.
23. Robson WL, Leung AK. Henoch-Schönlein purpura. Adv Pediatr. 1994. 41:163–194.
24. al-Sheyyab M, Batieha A, el-Shanti H, Daoud A. Henoch-Schonlein purpura and streptococcal infection: a prospective case-control study. Ann Trop Paediatr. 1999. 19:253–255.
25. Kwon EH, Kim SJ, Na MA, Jung YS, Lee DW, Lee SB, et al. A clinicopathological study of the adult Henoch-Schönlein Purpura. Korean J Med. 2003. 65:323–334.
26. Choong CK, Beasley SW. Intra-abdominal manifestations of Henoch-Schönlein purpura. J Paediatr Child Health. 1998. 34:405–409.
27. Rigante D, Candelli M, Federico G, Bartolozzi F, Porri MG, Stabile A. Predictive factors of renal involvement or relapsing disease in children with Henoch-Schönlein purpura. Rheumatol Int. 2005. 25:45–48.
28. Austin HA 3rd, Balow JE. Henoch-Schönlein nephritis: prognostic features and the challenge of therapy. Am J Kidney Dis. 1983. 2:512–520.
29. White RH. Henoch-Schönlein nephritis. A disease with significant late sequelae. Nephron. 1994. 68:1–9.
30. Saulsbury FT. Clinical update: Henoch-Schönlein purpura. Lancet. 2007. 369:976–978.
31. Coppo R, Andrulli S, Amore A, Gianoglio B, Conti G, Peruzzi L, et al. Predictors of outcome in Henoch-Schönlein nephritis in children and adults. Am J Kidney Dis. 2006. 47:993–1003.
32. Jeong YK, Ha HK, Yoon CH, Gong G, Kim PN, Lee MG, et al. Gastrointestinal involvement in Henoch-Schönlein syndrome: CT findings. AJR Am J Roentgenol. 1997. 168:965–968.
33. Goldstein AR, White RH, Akuse R, Chantler C. Long-term follow-up of childhood Henoch-Schönlein nephritis. Lancet. 1992. 339:280–282.
34. Islek I, Muslu A, Dagdemir A, Dilber C. Is low serum complement 3 in Henoch Schönlein purpura unusual and of prognostic value? Eur J Pediatr. 2001. 160:397–398.
35. Mollica F, Li Volti S, Garozzo R, Russo G. Effectiveness of early prednisone treatment in preventing the development of nephropathy in anaphylactoid purpura. Eur J Pediatr. 1992. 151:140–144.
36. Ronkainen J, Koskimies O, Ala-Houhala M, Antikainen M, Merenmies J, Rajantie J, et al. Early prednisone therapy in Henoch-Schönlein purpura: a randomized, double-blind, placebo-controlled trial. J Pediatr. 2006. 149:241–247.
37. Hong EJ, Ha TS. The effect of steroid on renal involvement in Henoch-Schonlein Purpura. J Korean Soc Pediatr Nephrol. 2007. 11:185–194.
38. Huang DC, Yang YH, Lin YT, Chiang BL. Cyclosporin A therapy for steroid-dependent Henoch-Schönlein purpura. J Microbiol Immunol Infect. 2003. 36:61–64.
39. Allen DM, Diamond LK, Howell DA. Anaphylactoid purpura in children (Schonlein-Henoch syndrome): review with a follow-up of the renal complications. AMA J Dis Child. 1960. 99:833–854.
40. Counahan R, Cameron JS. Henoch-Schönlein nephritis. Contrib Nephrol. 1977. 7:143–165.
41. Koskimies O, Mir S, Rapola J, Vilska J. Henoch-Schönlein nephritis: long-term prognosis of unselected patients. Arch Dis Child. 1981. 56:482–484.
Similar articles