Download PDF
Klin Padiatr 2008; 220(1): 47-52
DOI: 10.1055/s-2007-972570
Mitteilung

© Georg Thieme Verlag KG Stuttgart · New York

Purpura Schoenlein-Henoch - Ergebnisse der Wörlitzer Konsensusgespräche 2005 zum diagnostischen und therapeutischen Vorgehen

Purpura Schoenlein-Henoch - Results of the Wörlitz 2005 Consensus Conference Focussing on Diagnosis and TherapyT. Hospach 1 [*] , G. Klaus 2 [*] , K. Holl-Ulrich 3 , G. E. Dannecker 1 , G. Horneff 4
  • 1Olgahospital Stuttgart, Pädiatrie 1, Stuttgart
  • 2Universitätskinderklinik Marburg
  • 3Universitätsklinikum Schleswig Holstein, Campus Lübeck, Institut für Pathologie
  • 4Zentrum für Allgemeine Pädiatrie und Neonatologie, Asklepios Klinik Sankt Augustin, Sankt Augustin
Further Information

Publication History

Publication Date:
20 December 2007 (online)

Also available at
    Permissions and Reprints

Zusammenfassung

Die Purpura Schoenlein-Henoch (PSH) ist die häufigste Vaskulitis des Klein- und Schulkindes. Die typischen Manifestationen betreffen die Haut, den Gastrointestinaltrakt sowie die Gelenke. Die ebenfalls häufig vorkommende renale Beteiligung ist meist milde und führt nur selten zu einer prognostisch ungünstigen PSH-Nephritis. Trotz der relativen Häufigkeit der Erkrankung stehen für das diagnostische und therapeutische Vorgehen bislang keine Empfehlungen zur Verfügung. Im Rahmen der 8. Konsensusgespräche in Wörlitz im Herbst 2005 wurden Empfehlungen diskutiert und in dieser Arbeit zusammengefasst.

Abstract

Henoch-Schoenlein Purpura (PSH) is the most frequent vasculitis in childhood. Skin, bowel and joints are characteristically involved. Although renal manifestations are common PSH-nephritis is infrequent but characterized by a poor prognosis. In autumn 2005 diagnosis and treatment were discussed at the 8th consensus meeting in Wörlitz. These results are summarized.

Schlüsselwörter

Purpura - Schoenlein-Henoch - Diagnose - Therapie

Key words

purpura - Schoenlein-Henoch - diagnosis - therapy

Literatur

  • 1 Allen DM, Diamond LK, Howell DA.. Anaphylactoid purpura in children (Schoenlein-Henoch syndrome): review with follow-up ot the renal complications.  Am J Dis Child. 1960;  99 833-854
    PubMedGoogle Scholar
  • 2 Caksen H, Odabas D, Kösem M, Arslan S, Öner AF, Atas B, Akcay G, Ceylan N.. Report of eight infants with acute infantile hemor-rhagic edema and review of the literature.  J Dermatol. 2002;  29 290-295
    PubMedGoogle Scholar
  • 3 Chang WL, Yang YH, Wang LC, Lin YT, Chiang BL. Renal manifestations in Henoch-Schonlein purpura: a 10-year clinical study.  Pediatr Nephrol. 2005;  20 1269-1272 , . Epub 2005 Jun 10
    CrossrefPubMedGoogle Scholar
  • 4 Coppo R, Andrulli S, Amore A, Gianoglio B, Conti G, Peruzzi L, Locatelli F, Cagnoli L. Predictors of outcome in Henoch-Schonlein nephritis in children and adults.  Am J Kidney Dis. 2006;  47 993-1003
    CrossrefPubMedGoogle Scholar
  • 5 Coppo R, Mazzucco G, Cagnoli L, Lupo A, Schena FP.. Long-term prognosis of Henoch-Schonlein nephritis in adults and children. Italian Group of Renal Immunopathology Collaborative Study on Henoch-Schonlein purpura.  Nephrol Dial Transplant. 1997;  12 2277-2283
    PubMedGoogle Scholar
  • 6 Counahan R, Winterborn MH, White RH, Heaton JM, Meadow SR, Bluett NH, Swetschin H, Cameron JS, Chantler C. Prognosis of Henoch-Schonlein nephritis in children.  Br Med J. 1977;  2 11-14
    PubMedGoogle Scholar
  • 7 Dixit MP, Dixit NM, Scott K. Managing Henoch-Schonlein purpura in children with fish oil and ACE inhibitor therapy.  Nephrology (Carlton). 2004;  9 381-386
    CrossrefPubMedGoogle Scholar
  • 8 Foster BJ, Bernard C, Drummond KN, Sharma AK.. Effective therapy for severe Henoch-Schonlein purpura nephritis with prednisone and azathioprine: a clinical and histopathologic study.  J Pediatr. 2000;  136 370-375
    CrossrefPubMedGoogle Scholar
  • 9 Garcia-Porrua C, Calvino MC, Llorca J, Couselo JM, Gonzalez-Gay MA. Henoch-Schonlein purpura in children and adults: clinical differences in a defined population.  Semin Arthritis Rheum. 2002;  32 149-156
    CrossrefPubMedGoogle Scholar
  • 10 Gardner-Medwin JMM, Dolezalova P, Cummings C, Southwood TR. Incidence of Henoch-Schoenlein purupura, Kawasaki disease and rare vasculitides in children of different ethnic origins.  Lancet. 2002;  360 1197-1202
    CrossrefPubMedGoogle Scholar
  • 11 Glasier CM, Siegel MJ, MacAlister WH, Shackelford GD.. Henoch-Schonlein syndrome in children: gastrointestinal manifestations.  Am J Roentgenol. 198;  6 1081-10855
    PubMedGoogle Scholar
  • 12 Goldstein AR, White RH, Akuse R, Chantler C.. Long-term follow-up of childhood Henoch-Schonlein nephritis.  Lancet. 1992;  339 280-282
    CrossrefPubMedGoogle Scholar
  • 13 Halling SF, Soderberg MP, Berg UB. Henoch Schonlein nephritis: clinical findings related to renal function and morphology.  Pediatr Nephrol. 2005;  20 46-51 , . Epub 2004 Oct 22
    CrossrefPubMedGoogle Scholar
  • 14 Hattori M, Ito K, Konomoto T, Kawaguchi H, Yoshioka T, Khono M.. Plasmapheresis as the sole therapy for rapidly progressive Henoch-Schonlein purpura nephritis in children.  Am J Kidney Dis. 1999;  33 427-433
    PubMedGoogle Scholar
  • 15 Hospach T, Dannecker GE. Purpura Schoenlein-Henoch. In: Evidenzbasierte Pädiatrie Thieme Verlag. EG I 2004
  • 16 Huber AM, King J, MacLaine P, Klassen T, Pothos M. A randomized, placebo-controlled trial of prednisone in early Henoch Schonlein Purpura [ISRCTN85109383].  BMC Med. 2004;  2 7
    CrossrefPubMedGoogle Scholar
  • 17 Hung SH, Yang YH, Lee CC, Wang LC, Lin YT, Chiang BL. Clinical predictors of self-limited urinalysis abnormality in childhood Henoch-Schonlein purpura nephritis.  Acta Paediatr. 2006;  95 306-311
    CrossrefPubMedGoogle Scholar
  • 18 Prenzel F. Pfäffle R. Thiele F. Schuster V. Decreased Factor XIII Activity during Severe Henoch-Schoenlein Purpura - Does It Play a Role.  Klin Pädiatr. 2006;  218 174-176
    PubMedGoogle Scholar
  • 19 Kawana S, Shen GH, Kobayashi Y, Nishiyama S. Membrane attack complex of complements in Henoch-Schonlein Purpura skin and nephritis.  Arch Dermatol Res. 1990;  282 183-187
    CrossrefPubMedGoogle Scholar
  • 20 Kawasaki Y, Suzuki J, Murai M, Takahashi A, Isome M, Nozawa R, Suzuki S, Suzuki H.. Plasmapheresis therapy for rapidly progressive Henoch-Schonlein nephritis.  Pediatr Nephrol. 2004;  19 920-923 , . Epub 2004 Jun 9
    PubMedGoogle Scholar
  • 21 Kawasaki Y, Suzuki J, Nozawa R, Suzuki S, Suzuki H. Efficacy of methylprednisolone and urokinase pulse therapy for severe Henoch-Schonlein nephritis.  Pediatrics. 2003;  111 785-789
    CrossrefPubMedGoogle Scholar
  • 22 Kawasaki Y, Suzuki J, Sakai N, Nemoto K, Nozawa R, Suzuki S, Suzuki H.. Clinical and pathological features of children with Henoch-Schoenlein purpura nephritis: risk factors associated with poor prognosis.  Clin Nephrol. 2003;  60 153-160
    PubMedGoogle Scholar
  • 23 Kawasaki Y, Suzuki J, Suzuki H.. Efficacy of methylprednisolone and urokinase pulse therapy combined with or without cyclophosphamide in severe Henoch-Schoenlein nephritis: a clinical and histopathological study.  Nephrol Dial Transplant. 2004;  19 858-864
    CrossrefPubMedGoogle Scholar
  • 24 Ladell K. Was ist Leukoyzytoklasie? Eine histologische und elektronenmikroskopische Untersuchung an kutaner leukozytoklastischer Vaskulitis.  Dissertation. 2001;  http://edoc.hu-berlin.de/dissertationen/ladell-krstin-2001-05-25/HTML/front.htmlfront
    PubMedGoogle Scholar
  • 25 Mills JA, Michel BA, Bloch DA. et al . The American College of Rheumatology 1990 criteria for the classification of Henoch-Schoenlein purpura.  Arth Rheum. 1990;  33 1114-1121
    PubMedGoogle Scholar
  • 26 Mohan N, Edwards ET, Cupps TR, Slifman N, Lee JH, Siegel JN, Braun MM.. Leukocytoclastic Vasculitis associated with tumor necrosis factor-α blocking agents.  J Rheumatol. 2004;  31 1955-1958
    PubMedGoogle Scholar
  • 27 Mollica F, Li Volti S, Garozzo R, Russo G. Corticosteroid therapy does not prevent nephritis in Henoch-Schonlein purpura.  Pediatr Nephrol. 1994;  8 131
    PubMedGoogle Scholar
  • 28 Mrusek S, Krüger M, Greiner P, Kleinschmidt M, Brandis M, Ehl S. Henoch-Schoenlein purpura.  Lancet. 2004;  363 1116
    CrossrefPubMedGoogle Scholar
  • 29 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;  90 916-920 , . Epub 2005 May 4
    CrossrefPubMedGoogle Scholar
  • 30 Niaudet P, Habib R. Schonlein-Henoch purpura nephritis: prognostic factors and therapy.  Ann Med Interne (Paris). 1994;  145 577-580
    PubMedGoogle Scholar
  • 31 Niaudet P, Habib R. Methylprednisolone pulse therapy in the treatment of severe forms of Schonlein-Henoch purpura nephritis.  Pediatr Nephrol. 1998;  12 238-243
    CrossrefPubMedGoogle Scholar
  • 32 Niaudet P, Levy M, Broyer M, Habib R.. Clinicopathologic correlations in severe forms of Henoch-Schonlein purpura nephritis based on repeat biopsies.  Contrib Nephrol. 1984;  40 250-254
    PubMedGoogle Scholar
  • 33 Ozen S. et al . , EULAR/PRES endorsed Consensus Criteria for the Classification of Childhood Vasculitides under review by the ACR Ann Rheum Dis published online 1 Dec.  2005;  , doi:10.1136/ard.2005.046300
    PubMedGoogle Scholar
  • 34 Ozen S. The spectrum of vasculitis in children.  Best Pract Res Clin Rheumatol. 2002;  3 411-425
    PubMedGoogle Scholar
  • 35 Prigent F, Chevalier B, Bensman A. et al . LŽalitement, traitment du purpura rhumatoide?.  Ann Dermatol Venereol. 1997;  124 296-298
    PubMedGoogle Scholar
  • 36 Reinehr T, Bürk G, Andler W.. Does steroid treatment of abdominal pain prevent renal involvement in Henoch-Schoenlein purpura.  J Pediatr Gastroenterol Nutr. 2000;  3 323-324
    PubMedGoogle Scholar
  • 37 Ronkainen J, Ala-Houhala M, Huttunen NP, Jahnukainen T, Koskimies O, Ormala T, Nuutinen M. Outcome of Henoch-Schoenlein nephritis with nephrotic-range proteinuria.  Clin Nephrol. 2003;  60 80-84
    PubMedGoogle Scholar
  • 38 Ronkainen J, Nuutinen M, Koskimies O. The adult kidney 24 years after childhood Henoch-Schonlein purpura: a retrospective cohort study.  Lancet. 2002;  360 666-670
    CrossrefPubMedGoogle Scholar
  • 39 Ronkainen J, Koskimies O, Ala-Houhala M, Antikainen M, Merenmies J, Rajantie J, Ormala T, Turtinenen J, Nuutinen M. Early prednisone therapy in Henoch-Schonlein purpura: a randomise, double-blind, placebo-controlled trial.  J Pediatr. 2006;  149 241-247
    PubMedGoogle Scholar
  • 40 Rosenblum ND, Winter HS. Steroid effects on the course of abdominal pain in Henoch-Schoenlein purpura.  Pediatrics. 1987;  79 1081-1021
    PubMedGoogle Scholar
  • 41 Rostoker G. Schoenlein-Henoch Purpura in children and adults.  BioDrugs. 2001;  2 99-138
    PubMedGoogle Scholar
  • 42 Sams WM. Hypersensitivity angiitis.  J Invest Dermatol. 1989;  93 78-81
    CrossrefPubMedGoogle Scholar
  • 43 Saulsbury FT. Corticosteroid therapy does not prevent nephritis in Henoch-Schonlein purpura.  Pediatr Nephrol. 1993;  7 69-71
    CrossrefPubMedGoogle Scholar
  • 44 Schärer K, Krmar R, Querfeld U, Ruder H, Waldherr R, Schaefer F. Clinical outcome of Schonlein-Henoch purpura nephritis in children.  Pediatr Nephrol. 1999;  13 816-823
    CrossrefPubMedGoogle Scholar
  • 45 Schwartz GJ, Brion LP, Spitzer A. The use of plasma creatinine concentration for estimating glomerular filtration rate in infants, children, and adolescents.  Pediatr Clin North Am. 1987;  34 571-590
    PubMedGoogle Scholar
  • 46 Shin JI, Park JM, Shin YH, Hwang DH, Kim JH, Lee JS.. Predictive factors for nephritis, relapse, and significant proteinuria in childhood He-noch-Schonlein purpura.  Scand J Rheumatol. 2006;  35 56-60
    CrossrefPubMedGoogle Scholar
  • 47 Shin JI, Park JM, Shin YH, Kim JH, Kim PK, Lee JS, Jeong HJ. Cyclosporin A therapy for severe Henoch-Schonlein nephritis with nephritic syndrome.  Pediatr Nephrol. 2005;  20 1093-1097 , . Epub 2005 Jun 10
    CrossrefPubMedGoogle Scholar
  • 48 Shin JI, Park JM, Shin YH, Kim JH, Lee JS, Kim PK, Jeong HJ. Can azathioprine and steroids alter the progression of severe Henoch-Schonlein nephritis in children?.  Pediatr Nephrol. 2005;  20 1087-1092 , . Epub 2005 May 12
    CrossrefPubMedGoogle Scholar
  • 49 Tizard EJ. Henoch-Schoenlein purpura.  Arch Dis Child. 1999;  80 380-383
    PubMedGoogle Scholar
  • 50 White RHR. Schoenlein Henoch Purpura. In Churg A, Churg J (eds) Systemic vasculitides. New York: Igaku-Shoin 1991: 201-217
    Google Scholar
  • 51 Yarvuz H, Arslan A. Henoch-Schoenlein purura-related intestinal perforation: a steroid complication?.  Pediatr Int. 2001;  43 423-425
    CrossrefPubMedGoogle Scholar

1 zu gleichen Teilen erarbeitet

Korrespondenzadresse

T. Hospach

Olgahospital

Bismarckstr. 8

70176 Stuttgart

Phone: +49/711/9920,+49/711/992 24 17

Fax: +49/711/992 24 19

Email: a.hospach@olgahospital.de

      >