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
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
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
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
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
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
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
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
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
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
11
Glasier CM, Siegel MJ, MacAlister WH, Shackelford GD..
Henoch-Schonlein syndrome in children: gastrointestinal manifestations.
Am J Roentgenol.
198;
6
1081-10855
12
Goldstein AR, White RH, Akuse R, Chantler C..
Long-term follow-up of childhood Henoch-Schonlein nephritis.
Lancet.
1992;
339
280-282
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
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
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
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
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
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
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
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
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
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
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
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
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
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
28
Mrusek S, Krüger M, Greiner P, Kleinschmidt M, Brandis M, Ehl S.
Henoch-Schoenlein purpura.
Lancet.
2004;
363
1116
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
30
Niaudet P, Habib R.
Schonlein-Henoch purpura nephritis: prognostic factors and therapy.
Ann Med Interne (Paris).
1994;
145
577-580
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
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
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
34
Ozen S.
The spectrum of vasculitis in children.
Best Pract Res Clin Rheumatol.
2002;
3
411-425
35
Prigent F, Chevalier B, Bensman A. et al .
LŽalitement, traitment du purpura rhumatoide?.
Ann Dermatol Venereol.
1997;
124
296-298
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
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
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
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
40
Rosenblum ND, Winter HS.
Steroid effects on the course of abdominal pain in Henoch-Schoenlein purpura.
Pediatrics.
1987;
79
1081-1021
41
Rostoker G.
Schoenlein-Henoch Purpura in children and adults.
BioDrugs.
2001;
2
99-138
42
Sams WM.
Hypersensitivity angiitis.
J Invest Dermatol.
1989;
93
78-81
43
Saulsbury FT.
Corticosteroid therapy does not prevent nephritis in Henoch-Schonlein purpura.
Pediatr Nephrol.
1993;
7
69-71
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
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
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
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
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
49
Tizard EJ.
Henoch-Schoenlein purpura.
Arch Dis Child.
1999;
80
380-383
50 White RHR. Schoenlein Henoch Purpura. In Churg A, Churg J (eds)
Systemic vasculitides . New York: Igaku-Shoin 1991: 201-217
51
Yarvuz H, Arslan A.
Henoch-Schoenlein purura-related intestinal perforation: a steroid complication?.
Pediatr Int.
2001;
43
423-425
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