Aggiornamento monografico
La porpora di Schönlein-Henoch
HENOCH-SCHÖNLEIN PURPURA (HSP)
Lorenzo Calligaris, Federico Marchetti, Egidio Barbi, Eva da Dalt, Elisa Panontin, Loredana Lepore, Alessandro Ventura
Clinica Pediatrica, IRCCS “Burlo Garofolo”, Trieste
Gennaio 2009 - pagg. 19 -26
Abstract
Henoch-Schönlein purpura (HSP) is the most common vasculitic disease of childhood. HSP
is a multisystem immunoglobulin A-mediated vasculitis with a self-limited course usually affecting
the skin, joints, gastrointestinal tract, and kidneys. HSP occurs most often in children
before the age of 10 years, and classically presents with a unique distribution of the rash
to the lower extremities and the buttocks. One third of these patients will have one or more
recurrences of symptoms, usually within 6 weeks, but they may occur as late as 18
months later. The overall prognosis in HSP is excellent, but the long-term morbidity depends
mainly on the renal involvement. In our opinion, actually, there are no data to recommend
the routine use of corticosteroids in the management of uncomplicated HSP, in both
the acute and chronic settings.
Parole chiave
Suggerite dall'AI
Classificazione MeSH
Bibliografia
1. Peru H, Soylemezoglu O, Bakkaloglu SA, et
al. Henoch-Schönlein purpura in childhood:
clinical analysis of 254 cases over a 3-year period.
Clin Rheumatol 2008;27:1087-92.
2. Tizard EJ. Henoch-Schönlein purpura. Arch Dis Child 1999;80:380-3.
3. Ha T-S, Cha SH. Cerebral vasculitis in Henoch- Schönlein purpura: a case report with sequential magnetic resonance imaging. Pediatr Nephrol 1996;10:634-6.
4. Ostegaard JR, Storm K. Neurologic manifestations of Schönlein-Henoch purpura. Acta Pediatr Scand 2001;80:339-42.
5. Matsubayashi R, MatsubayashiT, Fujita N, Yokota T, Ohro Y, Enoki H. Pulmunary hemorrhage associated with Henoch-Schönlein purpura in a child. Clin Rheumatol 2008;27: 803-5.
6. Tae-Sun H, Jin-Seok L. Scrotal involvement in childhood Henoch-Schönlein purpura. Acta Paediatr 2007; 96:552-5.
7. Trapani S, Micheli A, Grisolia F et al. Henoch- Schönlein purpura in childhood: epidemiological and clinical analysis of 150 cases over a 5-year period and review of literature. Semin Arthritis Rheum 2005;35:143-53.
8. Ozen S, Ruperto N, Dillon MJ, et al. EULAR/ PreS endorsed consensus criteria for the classification of childhood vasculites. Ann Rheum Dis 2006;65:936-41.
9. Rabach I, Badina L, Lazzerini M, Longo G. Edema e rash. Medico e Bambino 2008;27: 325-6.
10. Miorin E, Meneghini A, Don B, Romanello C, Tenore A. Edema emorragico acuto del lattante: descrizione di un caso clinico e revisione della letteratura. Medico e Bambino pagine elettroniche 2002;5 http://www.medicoe bambino. com/?id=CL0203_10.html.
11. Saulsbury FT. Clinical update: Henoch- Schönlein purpura. Lancet 2007;369:976-8.
12. Chang WL, Yang YH, Lin YT, Chang BL. Gastrointestinal manifestation in Henoch- Schönlein purpura: a review of 261 patients. Acta Paediatr 2004;93:1427-31.
13. Sano H, Izumida M, Shimizu M, Ogawa Y. Risk factors on renal involvement and significant proteinuria in Henoch-Schönlein purpura. Eur J Pediatr 2002;161:193-201.
14. Salsbury FT. Henoch-Schönlein purpura in children: report of 100 patients and review of the literature. Medicine 1999;78:395-409.
15. 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.
16. Tizard EJ, Halilton-Ayres MJJ. Henoch- Schönlein purpura. Arch Dis Child Ed Pract Ed 2008;91:1-8.
17. Pennesi M, Biasotto E, Saccari A. Schönlein- Henoch purpura involving the penis. Arch Dis Child 2006;91:603.
18. Bonora G, Malorgio C. Una porpora di Schönlein-Henoch plurisintomatica. Medico e Bambino pagine elettroniche 2002;5 http: //www. medicoebambino.com/?id= CL0204 _10.html.
19. Misra AK, Biswas A, Das SK, Gharai PK, Roy T. Henoch-Schönlein Purpura with Intracerebral Haemorrhage. Japi 2004;52:833-4.
20. Chao HC, Kong MS, Lin SJ. Hepatobiliary involvement of Henoch-Schönlein purpura in children. Acta Paediatr Taiwan 2000;41:63-8.
21. Dillon MJ. Henoch-Schönlein purpura: recent advances. Clin Exp Rheum 2007;25: S66-8.
22. Ayoub EM, McBride J, Schmiederer M, Anderson B. Role of Bartonella henselae in the etiology of Henoch-Schönlein purpura. Pediatr Infect Dis J 2002;21:28-31.
23. Salsbury FT. Alteration in O-linked glycosilation of IgA1 in children with Henoch-Schönlein purpura. J Rheumatol 1997;24:2246-9.
24. Allen AC, Willis FR, Beattie TJ, Feehally J. Abnormal IgA glycosilation in Henoch-Schönlein purpura restricted to patients with clinical nephritis. Nephrol Dial Transplant 1998; 13:930-4.
25. Gershoni-Baruch R, BrozaY, Brik R. Prevalence and significance of mutations in the familial Mediterranean fever gene in Henoch- Schönlein purpura. J Pediatr 2003;143:658-61.
26. Amoli MM, Calvino MC, Garcia-Prrua C, Llorca J, Ollier WE, Gonzalez-Gay MA. Interleukin 1 beta gene polymorphism association with severe renal manifestation and renal sequelae in Henoch-Schönlein purpura. J Rheumatol 2004;31:295-8.
27. Amoli MM, Thomson W, Hajeer AH, et al. Interleukin 1 receptor antagonist gene polymorphism is associated with severe renal involvement and renal sequelae in Henoch- Schönlein purpura. J Rheumatol 2002;29: 1404-7.
28. Calvino MC, Llorca J, Garcia-Porrua C, Fernandez-Iglesias JL, Rodriguez-Ledo P, Gonzalez-Gay MA. Henoch-Schönlein purpura in childhood from northwestern Spain. Medicine 2001;80:279-90.
29. Mir S, Yavascan O, Mutlubas F, Yeniay B. Clinical outcome in children with Henoch- Schönlein nephritis. Pediatr Nephrol 2007; 22:64-70.
30. Ronkainen J, Nuutinen M, Koskimies O. The adult kidney 24 years after childhood Henoch- Schönlein purpura: a retrospective cohort study. Lancet 2002;360:666-70.
31. Pieu P, Noel LH. Henoch-Schönlein nephritis in children and adults. Morphological features and clinicopathological correlations. Ann Med Intern 1999;150:151-9.
32. Hung SH, Yang YH, Lee CC, Wang LC, Lin YT, Chiang BL. Clinical predictors of self-limited urinalysis abnormality in childhood Henoch- Schönlein purpura nephritis. Acta Paediatr 2006;95:306-11.
33. Shin JI, Park JM, Shin YH, Hwang DH, Kim JH, Lee JS. Predictive factors for nephritis, relapse and significant proteinuria in childhood Henoch-Schönlein purpura. Scand J Rheumatol 2006;35:56-60.
34. Mollica F, Li Volti S, Garrozzo R, Russo G. Effectiveness of early prednisone treatment in preventing the developement of nephropaty in anaphylactoid purpura. Eur J Pediatr 1992; 151:140-4.
35. Huber AM, King J, McLaine P, Klassen T, Pothos M. A randomized, placebo controlled trial of prednisone in early Henoch-Schönlein purpura. BMC Med 2004;2:7.
36. Ronkainen J, Koskimibs O, Ala-Houalala M, et al. Early prednisone therapy in Henoch- Schönlein purpura: a randomized doubleblind, placebo-controlled trial. J Pediatr 2006; 149:241-7.
37. Wiss PF, Feinstein JA, Luan X, Burnham JM, Feudtner C. Effects of corticosteroids on Henoch-Schönlein purpura: a systemic review. Pediatrics 2007;120:1079-87.
38. Buchanec J, Galanda V, Belakova S, Miunarik M, Zibolen M. Incidence of renal complications in Henoch-Schönlein purpura syndrome independence of early administrations of steroids. Int J Nephrol 1988;20:409-12.
39. Saulsbury FT. Corticosteroid therapy does not prevent nephritis in Henoch-Schönlein purpura. Pediatr Nephrol 1993;7:69-71.
40. Dudley J, Smith G, Llewellyn A, Tizard J. Randomised placebo controlled trial to assess the role of early prednisolone on the development of Henoch-Schönlein purpura nephritis. J Am Soc Nephrol 2007;18:47A.
41. Chartapisak W, Opastiraku SL, Willis NS, Craig JC, Hodson EM. Prevention and treatment of renal disease in Henoch-Schönlein purpura: a systematic review. Arch Dis Child 2009;94;132-137
42. Haroon M. Should children with Henoch- Schönlein purpura and abdominal pain be treated with steroids? Arch Dis Child 2005; 90:1196-8.
43. Reif S, Jain A, Santiago J, Rossi T. Protein losing enteropathy as a manifestation of Henoch- Schönlein purpura. Acta Paediatr Scand 1991;80:482-5.
44. Kawasaki Y, Suzuki J, Nozawa R, Suzuki S, Suzuki H. Efficacy of methylprednisolone and urokinase pulse therapy for severe Henoch- Schönlein nephritis. Pediatrics 2003;111:785-9.
45. Kawasaki Y, Suzuki J, Murai M, et al. Plasmapheresis therapy for rapidly progressive Henoch-Schönlein nephritis. Pediatr Nephrol 2004;19:920-3.
46. Shin JI, Park JM, Lee JS, Kim JH, Kim PK, Jeong HJ. Successful use of cyclosporine A in severe Schönlein-Henoch nephritis resistant to both methylprednisolone pulse and azathioprine. Clin Rheumatol 2006;25:759-60.
47. Shin JI, Park JM, Shin YH, Kim JH, Lee JS, Jeong HJ. Henoch-Schönlein purpura nephritis with nephrotic-range proteinuria: histological regression possibly associated with cyclosporin A and steroid treatment. Scand J Rheumatol 2005;34:392-5.
48. Ronkainen J, Ala-Houhala M, Antikainen M, et al. Cyclosporine A (CyA) versus MP pulses (MP) in the treatment of severe Henoch- Schönlein Nephritis (HSN). Pediatr Nephrol 2006;21:1531.
49. Shin JI, Park JM, Shin YH, et al. Can azathioprine and steroids alter the progression of severe nephritis in children. Pediatr Nephrol 2005;20:1087-92.
50. Tarshish P, Bernstein J, Edelman CM. Henoch- Schönlein purpura nephritis: course of disease and efficacy of cyclophosphamide. Pediatr Nephrol 2004;19:51-6.
51. Dede F, Onec B, Ayll D, Gonul II, Onec K. Mycophenolate mofetil traeatment of crescentic Henoch-Schönlein nephritis with IgA depositions. Scand J Urol Nephrol 2008;42: 178-80.
52. Shenoy M, Bradbury MG, Lewis MA, Webb NJA. Outcome of Henoch-Schönlein purpura nephritis treated with long-term immunosuppression. Pediatr Nephrol 2007;22: 1717-22.
53. Lamireau T, Rebouissoux L, Hehunstre JP. Intravenous immunoglobulin therapy for severe digestive manifestations of Henoch-Schönlein purpura. Acta Pediatr 2001;90:1081-2.
54. Fagbemi AA, Torrente F, Hilson AJ, Thomson MA, Heuschkel RB, Murch SH. Massive gastrointestinal haemorrhage in isolated intestinal Henoch-Schönlein purpura with response to intravenous immunoglobulin infusion. Eur J Pediatr 2007;166:915-9.
55. Martin S, Cramer CH, Heikenen J, Gitomer JJ. Gastrointestinal symptoms of Henoch- Schönlein purpura treated with mycophenolate mofetil. J Pediatr Gastroenterol Nutr 2006; 43:245-7.
56. Wen YK, Yang Y, Chang CC. Cerebral vasculitis and intracerebral hemorrhage in Henoch- Schönlein purpura treated with plasmapheresis. Pediatr Nephrol 2005;20:223-5.
2. Tizard EJ. Henoch-Schönlein purpura. Arch Dis Child 1999;80:380-3.
3. Ha T-S, Cha SH. Cerebral vasculitis in Henoch- Schönlein purpura: a case report with sequential magnetic resonance imaging. Pediatr Nephrol 1996;10:634-6.
4. Ostegaard JR, Storm K. Neurologic manifestations of Schönlein-Henoch purpura. Acta Pediatr Scand 2001;80:339-42.
5. Matsubayashi R, MatsubayashiT, Fujita N, Yokota T, Ohro Y, Enoki H. Pulmunary hemorrhage associated with Henoch-Schönlein purpura in a child. Clin Rheumatol 2008;27: 803-5.
6. Tae-Sun H, Jin-Seok L. Scrotal involvement in childhood Henoch-Schönlein purpura. Acta Paediatr 2007; 96:552-5.
7. Trapani S, Micheli A, Grisolia F et al. Henoch- Schönlein purpura in childhood: epidemiological and clinical analysis of 150 cases over a 5-year period and review of literature. Semin Arthritis Rheum 2005;35:143-53.
8. Ozen S, Ruperto N, Dillon MJ, et al. EULAR/ PreS endorsed consensus criteria for the classification of childhood vasculites. Ann Rheum Dis 2006;65:936-41.
9. Rabach I, Badina L, Lazzerini M, Longo G. Edema e rash. Medico e Bambino 2008;27: 325-6.
10. Miorin E, Meneghini A, Don B, Romanello C, Tenore A. Edema emorragico acuto del lattante: descrizione di un caso clinico e revisione della letteratura. Medico e Bambino pagine elettroniche 2002;5 http://www.medicoe bambino. com/?id=CL0203_10.html.
11. Saulsbury FT. Clinical update: Henoch- Schönlein purpura. Lancet 2007;369:976-8.
12. Chang WL, Yang YH, Lin YT, Chang BL. Gastrointestinal manifestation in Henoch- Schönlein purpura: a review of 261 patients. Acta Paediatr 2004;93:1427-31.
13. Sano H, Izumida M, Shimizu M, Ogawa Y. Risk factors on renal involvement and significant proteinuria in Henoch-Schönlein purpura. Eur J Pediatr 2002;161:193-201.
14. Salsbury FT. Henoch-Schönlein purpura in children: report of 100 patients and review of the literature. Medicine 1999;78:395-409.
15. 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.
16. Tizard EJ, Halilton-Ayres MJJ. Henoch- Schönlein purpura. Arch Dis Child Ed Pract Ed 2008;91:1-8.
17. Pennesi M, Biasotto E, Saccari A. Schönlein- Henoch purpura involving the penis. Arch Dis Child 2006;91:603.
18. Bonora G, Malorgio C. Una porpora di Schönlein-Henoch plurisintomatica. Medico e Bambino pagine elettroniche 2002;5 http: //www. medicoebambino.com/?id= CL0204 _10.html.
19. Misra AK, Biswas A, Das SK, Gharai PK, Roy T. Henoch-Schönlein Purpura with Intracerebral Haemorrhage. Japi 2004;52:833-4.
20. Chao HC, Kong MS, Lin SJ. Hepatobiliary involvement of Henoch-Schönlein purpura in children. Acta Paediatr Taiwan 2000;41:63-8.
21. Dillon MJ. Henoch-Schönlein purpura: recent advances. Clin Exp Rheum 2007;25: S66-8.
22. Ayoub EM, McBride J, Schmiederer M, Anderson B. Role of Bartonella henselae in the etiology of Henoch-Schönlein purpura. Pediatr Infect Dis J 2002;21:28-31.
23. Salsbury FT. Alteration in O-linked glycosilation of IgA1 in children with Henoch-Schönlein purpura. J Rheumatol 1997;24:2246-9.
24. Allen AC, Willis FR, Beattie TJ, Feehally J. Abnormal IgA glycosilation in Henoch-Schönlein purpura restricted to patients with clinical nephritis. Nephrol Dial Transplant 1998; 13:930-4.
25. Gershoni-Baruch R, BrozaY, Brik R. Prevalence and significance of mutations in the familial Mediterranean fever gene in Henoch- Schönlein purpura. J Pediatr 2003;143:658-61.
26. Amoli MM, Calvino MC, Garcia-Prrua C, Llorca J, Ollier WE, Gonzalez-Gay MA. Interleukin 1 beta gene polymorphism association with severe renal manifestation and renal sequelae in Henoch-Schönlein purpura. J Rheumatol 2004;31:295-8.
27. Amoli MM, Thomson W, Hajeer AH, et al. Interleukin 1 receptor antagonist gene polymorphism is associated with severe renal involvement and renal sequelae in Henoch- Schönlein purpura. J Rheumatol 2002;29: 1404-7.
28. Calvino MC, Llorca J, Garcia-Porrua C, Fernandez-Iglesias JL, Rodriguez-Ledo P, Gonzalez-Gay MA. Henoch-Schönlein purpura in childhood from northwestern Spain. Medicine 2001;80:279-90.
29. Mir S, Yavascan O, Mutlubas F, Yeniay B. Clinical outcome in children with Henoch- Schönlein nephritis. Pediatr Nephrol 2007; 22:64-70.
30. Ronkainen J, Nuutinen M, Koskimies O. The adult kidney 24 years after childhood Henoch- Schönlein purpura: a retrospective cohort study. Lancet 2002;360:666-70.
31. Pieu P, Noel LH. Henoch-Schönlein nephritis in children and adults. Morphological features and clinicopathological correlations. Ann Med Intern 1999;150:151-9.
32. Hung SH, Yang YH, Lee CC, Wang LC, Lin YT, Chiang BL. Clinical predictors of self-limited urinalysis abnormality in childhood Henoch- Schönlein purpura nephritis. Acta Paediatr 2006;95:306-11.
33. Shin JI, Park JM, Shin YH, Hwang DH, Kim JH, Lee JS. Predictive factors for nephritis, relapse and significant proteinuria in childhood Henoch-Schönlein purpura. Scand J Rheumatol 2006;35:56-60.
34. Mollica F, Li Volti S, Garrozzo R, Russo G. Effectiveness of early prednisone treatment in preventing the developement of nephropaty in anaphylactoid purpura. Eur J Pediatr 1992; 151:140-4.
35. Huber AM, King J, McLaine P, Klassen T, Pothos M. A randomized, placebo controlled trial of prednisone in early Henoch-Schönlein purpura. BMC Med 2004;2:7.
36. Ronkainen J, Koskimibs O, Ala-Houalala M, et al. Early prednisone therapy in Henoch- Schönlein purpura: a randomized doubleblind, placebo-controlled trial. J Pediatr 2006; 149:241-7.
37. Wiss PF, Feinstein JA, Luan X, Burnham JM, Feudtner C. Effects of corticosteroids on Henoch-Schönlein purpura: a systemic review. Pediatrics 2007;120:1079-87.
38. Buchanec J, Galanda V, Belakova S, Miunarik M, Zibolen M. Incidence of renal complications in Henoch-Schönlein purpura syndrome independence of early administrations of steroids. Int J Nephrol 1988;20:409-12.
39. Saulsbury FT. Corticosteroid therapy does not prevent nephritis in Henoch-Schönlein purpura. Pediatr Nephrol 1993;7:69-71.
40. Dudley J, Smith G, Llewellyn A, Tizard J. Randomised placebo controlled trial to assess the role of early prednisolone on the development of Henoch-Schönlein purpura nephritis. J Am Soc Nephrol 2007;18:47A.
41. Chartapisak W, Opastiraku SL, Willis NS, Craig JC, Hodson EM. Prevention and treatment of renal disease in Henoch-Schönlein purpura: a systematic review. Arch Dis Child 2009;94;132-137
42. Haroon M. Should children with Henoch- Schönlein purpura and abdominal pain be treated with steroids? Arch Dis Child 2005; 90:1196-8.
43. Reif S, Jain A, Santiago J, Rossi T. Protein losing enteropathy as a manifestation of Henoch- Schönlein purpura. Acta Paediatr Scand 1991;80:482-5.
44. Kawasaki Y, Suzuki J, Nozawa R, Suzuki S, Suzuki H. Efficacy of methylprednisolone and urokinase pulse therapy for severe Henoch- Schönlein nephritis. Pediatrics 2003;111:785-9.
45. Kawasaki Y, Suzuki J, Murai M, et al. Plasmapheresis therapy for rapidly progressive Henoch-Schönlein nephritis. Pediatr Nephrol 2004;19:920-3.
46. Shin JI, Park JM, Lee JS, Kim JH, Kim PK, Jeong HJ. Successful use of cyclosporine A in severe Schönlein-Henoch nephritis resistant to both methylprednisolone pulse and azathioprine. Clin Rheumatol 2006;25:759-60.
47. Shin JI, Park JM, Shin YH, Kim JH, Lee JS, Jeong HJ. Henoch-Schönlein purpura nephritis with nephrotic-range proteinuria: histological regression possibly associated with cyclosporin A and steroid treatment. Scand J Rheumatol 2005;34:392-5.
48. Ronkainen J, Ala-Houhala M, Antikainen M, et al. Cyclosporine A (CyA) versus MP pulses (MP) in the treatment of severe Henoch- Schönlein Nephritis (HSN). Pediatr Nephrol 2006;21:1531.
49. Shin JI, Park JM, Shin YH, et al. Can azathioprine and steroids alter the progression of severe nephritis in children. Pediatr Nephrol 2005;20:1087-92.
50. Tarshish P, Bernstein J, Edelman CM. Henoch- Schönlein purpura nephritis: course of disease and efficacy of cyclophosphamide. Pediatr Nephrol 2004;19:51-6.
51. Dede F, Onec B, Ayll D, Gonul II, Onec K. Mycophenolate mofetil traeatment of crescentic Henoch-Schönlein nephritis with IgA depositions. Scand J Urol Nephrol 2008;42: 178-80.
52. Shenoy M, Bradbury MG, Lewis MA, Webb NJA. Outcome of Henoch-Schönlein purpura nephritis treated with long-term immunosuppression. Pediatr Nephrol 2007;22: 1717-22.
53. Lamireau T, Rebouissoux L, Hehunstre JP. Intravenous immunoglobulin therapy for severe digestive manifestations of Henoch-Schönlein purpura. Acta Pediatr 2001;90:1081-2.
54. Fagbemi AA, Torrente F, Hilson AJ, Thomson MA, Heuschkel RB, Murch SH. Massive gastrointestinal haemorrhage in isolated intestinal Henoch-Schönlein purpura with response to intravenous immunoglobulin infusion. Eur J Pediatr 2007;166:915-9.
55. Martin S, Cramer CH, Heikenen J, Gitomer JJ. Gastrointestinal symptoms of Henoch- Schönlein purpura treated with mycophenolate mofetil. J Pediatr Gastroenterol Nutr 2006; 43:245-7.
56. Wen YK, Yang Y, Chang CC. Cerebral vasculitis and intracerebral hemorrhage in Henoch- Schönlein purpura treated with plasmapheresis. Pediatr Nephrol 2005;20:223-5.
Corrispondenza: lorenzo.calligaris@inwind.it