Monatsschrift Kinderheilkunde

, Volume 154, Issue 9, pp 919–929 | Cite as

Systemischer Lupus erythematodes im Kindes- und Jugendalter

  • J. Brunner
  • C. Sergi
  • T. Jungraithmayr
  • L. B. Zimmerhackl
CME Weiterbildung • Zertifizierte Fortbildung

Zusammenfassung

Der systemische Lupus erythematodes (SLE) zeichnet sich durch eine Entzündung der Blutgefäße und des Bindegewebes aus, gehört zu den Kollagenosen und gilt als Prototyp der Autoimmunerkrankungen. Er ist eine entzündliche, nicht infektiöse Erkrankung bei Patienten mit genetischer Disposition. Differenzialdiagnostisch muss er gegenüber Malignomen, Infekten und anderen rheumatischen Erkrankungen abgegrenzt werden. Der SLE kann multiple Organmanifestationen zeigen. Als Manifestationen sind neben Allgemeinsymptomen, Hauterscheinungen, Nierenbeteiligung, Arthritiden, eine zerebrale Manifestation sowie eine Beteiligung der Leber und des hämatologischen Systems und des Herzens bekannt. Begleitet wird der SLE häufig von Fieber, Müdigkeit und Gewichtsabnahme. Die Therapie basiert auf einer exakten Diagnostik der Schwere und Ausdehnung der Erkrankung. Die Intensität der Therapie ist gegen ihre Nebenwirkungen abzuwägen.

Schlüsselwörter

Komplement T-Zellen Lupus-Nephritis 

Systemic lupus erythematosus in childhood and adolescence

Abstract

Systemic lupus erythematosus (SLE) is a heterogeneous multisystem autoimmune disease. Differential diagnoses to be considered include malignoma, infectious disease, and other rheumatic diseases. Rashes are a typical clinical feature at the time of diagnosis, followed by polyarthritis and renal pathology. Generalised lymphadenopathy and hepatosplenomegaly are common features, while neurological, pulmonary, and cardiac manifestations are infrequent. Renal involvement with SLE is more frequent in children than adults. Improved understanding of the immunopathogenesis of this disorder over the past two decades now affects therapeutic strategies, with treatment being directed at specific immunological targets. Treatment intensity needs to be balanced against its side effects.

Keywords

Complement T-cells Lupus nephritis 

Notes

Interessenkonflikt

Es besteht kein Interessenkonflikt. Der korrespondierende Autor versichert, dass keine Verbindungen mit einer Firma, deren Produkt in dem Artikel genannt ist, oder einer Firma, die ein Konkurrenzprodukt vertreibt, bestehen. Die Präsentation des Themas ist unabhängig und die Darstellung der Inhalte produktneutral.

Literatur

  1. 1.
    Smith CD, Cyr M (1988) The history of lupus erythematosus. From Hippocrates to Osler. Rheum Dis Clin North Am 14: 1–14Google Scholar
  2. 2.
    Kaposi M (1872) New reports on knowledge of lupus erythematosus. Arch Dermat u Syph 4: 36–72CrossRefGoogle Scholar
  3. 3.
    Libman E, Sacks B (1924) A hitherto undescribed form of valvular and mural endocarditis. Arch Intern Med 33: 701–737Google Scholar
  4. 4.
    Hargraves MM, Richmond H, Morton R (1948) Presentation of two bone marrow elements: The “tart” cell and the “LE” cell. Mayo Clin Proc 23: 25Google Scholar
  5. 5.
    Hochberg MC (1997) Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 40: 1725Google Scholar
  6. 6.
    Wagner N, Dannecker G (2003) Systemischer Lupus erythematodes und nicht-infektiöse Entzündungskrankheiten. In: Lentze MJ, Schaub J, Schulte FJ et al. (Hrsg) Pädiatrie, 2. Aufl. Springer, Berlin Heidelberg New York, S 656–662Google Scholar
  7. 7.
    Petty RE, Cassidy JT (2005) Systemic lupus erythematosus. In: Cassidy JT, Petty RE (eds) Textbook of pediatric rheumatology, 4th edn. Saunders, Philadelphia, pp 396–449Google Scholar
  8. 8.
    Gladman D, Urowitz M (2004) Clinical features. In: Hochberg MC, Silman AA, Smolen JS et al. (eds) Rheumatology, 3rd edn. Mosby, Edinburgh, pp 1359–1378Google Scholar
  9. 9.
    Alarcon GS, Friedman AW, Straaton KV et al. (1999) Systemic lupus erythematosus in three ethnic groups: III. A comparison of characteristics early in the natural history of the LUMINA cohort. LUpus in MInority populations: NAture vs. Nurture. Lupus 8: 197–209CrossRefPubMedGoogle Scholar
  10. 10.
    Williams W, Shah D, Sargeant LA (2004) The clinical and epidemiologic features in 140 patients with lupus nephritis in a predominantly black population from one center in Kingston, Jamaica. Am J Med Sci 327: 324–329CrossRefPubMedGoogle Scholar
  11. 11.
    Brunner HI, Silverman ED, Bombardier C et al. (2003) European Consensus Lupus Activity Measurement is sensitive to change in disease activity in childhood-onset systemic lupus erythematosus. Arthritis Rheum 49: 335–341CrossRefPubMedGoogle Scholar
  12. 12.
    Jolly M, Utset TO (2004) Can disease specific measures for systemic lupus erythematosus predict patients health related quality of life? Lupus 13: 924–926CrossRefPubMedGoogle Scholar
  13. 13.
    Shai R, Quismorio FP Jr, Li L et al. (1999) Genome-wide screen for systemic lupus erythematosus susceptibility genes in multiplex families. Hum Mol Genet 8: 639–644CrossRefPubMedGoogle Scholar
  14. 14.
    Tsao BP (2003) The genetics of human systemic lupus erythematosus. Trends Immunol 24: 595–602CrossRefPubMedGoogle Scholar
  15. 15.
    Grammer AC, Lipsky PE (2003) B cell abnormalities in systemic lupus erythematosus. Arthritis Res Ther 5(Suppl 4): S22–S27Google Scholar
  16. 16.
    Anand A, Dean GS, Quereshi K et al. (2002) Characterization of CD3+ CD4− CD8− (double negative) T cells in patients with systemic lupus erythematosus: activation markers. Lupus 11: 493–500CrossRefPubMedGoogle Scholar
  17. 17.
    Zhang J, Roschke V, Baker KP et al. (2001) Cutting edge: a role for B lymphocyte stimulator in systemic lupus erythematosus. J Immunol 166: 6–10PubMedGoogle Scholar
  18. 18.
    Singh RR (2005) SLE: translating lessons from model systems to human disease. Trends Immunol 26: 572–579CrossRefPubMedGoogle Scholar
  19. 19.
    Honda M, Mengesha E, Albano S et al. (2001) Telomere shortening and decreased replicative potential, contrasted by continued proliferation of telomerase-positive CD8+CD28(lo) T cells in patients with systemic lupus erythematosus. Clin Immunol 99: 211–221CrossRefPubMedGoogle Scholar
  20. 20.
    Tebbe B (2004) Clinical course and prognosis of cutaneous lupus erythematosus. Clin Dermatol 22: 121–124CrossRefPubMedGoogle Scholar
  21. 21.
    Gutierrez MA, Molina JF, Jara LJ et al. (1996) Prolactin-induced immunoglobulin and autoantibody production by peripheral blood mononuclear cells from systemic lupus erythematosus and normal individuals. Int Arch Allergy Immunol 109: 229–235PubMedGoogle Scholar
  22. 22.
    Waldman M, Madaio MP (2005) Pathogenic autoantibodies in lupus nephritis. Lupus 14: 19–24CrossRefPubMedGoogle Scholar
  23. 23.
    Sekikawa T, Kashihara N, Maruyama K et al. (1998) Expression of interleukin-8 in human glomerulonephritis. Res Commun Mol Pathol Pharmacol 99: 217–224PubMedGoogle Scholar
  24. 24.
    Weening JJ, D’Agati VD, Schwartz MM et al. (2004) The classification of glomerulonephritis in systemic lupus erythematosus revisited. Kidney Int 65: 521–530CrossRefPubMedGoogle Scholar
  25. 25.
    Tucker LB, Menon S, Schaller JG et al. (1995) Adult- and childhood-onset systemic lupus erythematosus: a comparison of onset, clinical features, serology, and outcome. Br J Rheumatol 34: 866–872PubMedGoogle Scholar
  26. 26.
    Daugas E, Nochy D, Huong du LT et al. (2002) Antiphospholipid syndrome nephropathy in systemic lupus erythematosus. J Am Soc Nephrol 13: 42–52CrossRefPubMedGoogle Scholar
  27. 27.
    Tucker LB, Menon S, Schaller JG et al. (1995) Adult- and childhood-onset systemic lupus erythematosus: a comparison of onset, clinical features, serology, and outcome. Br J Rheumatol 34: 866–872PubMedGoogle Scholar
  28. 28.
    Hanly JG (2005) Neuropsychiatric lupus. Rheum Dis Clin North Am 31: 273–298CrossRefPubMedGoogle Scholar
  29. 29.
    Carreno L, Lopez-Longo FJ, Gonzalez CM et al. (2002) Treatment options for juvenile-onset systemic lupus erythematosus. Paediatr Drugs 4: 241–256PubMedGoogle Scholar
  30. 30.
    Wahn V, Ruder H (2001) Systemischer Lupus erythematodes. In: Wahn V, Oppermannn J, Huppertz H-I et al. (Hrsg) Rheumatische Erkrankungen im Kindes- und Jugendalter, 1. Aufl. Hans Marseille, München, S 248–268Google Scholar
  31. 31.
    Carneiro JR, Sato EI (1999) Double blind, randomized, placebo controlled clinical trial of methotrexate in systemic lupus erythematosus. J Rheumatol 26: 1275–1279PubMedGoogle Scholar
  32. 32.
    Lehman TJ (1995) A practical guide to systemic lupus erythematosus. Pediatr Clin North Am 42: 1223–1238PubMedGoogle Scholar
  33. 33.
    Silverman ED, Lang B (1997) An overview of the treatment of childhood SLE. Scand J Rheumatol 26: 241–246PubMedGoogle Scholar
  34. 34.
    Contreras G, Pardo V, Leclercq B et al. (2004) Sequential therapies for proliferative lupus nephritis. N Engl J Med 350: 971–980CrossRefPubMedGoogle Scholar
  35. 35.
    Looney RJ, Anolik JH, Campbell D et al. (2004) B cell depletion as a novel treatment for systemic lupus erythematosus: a phase I/II dose-escalation trial of rituximab. Arthritis Rheum 50: 2580–2589CrossRefPubMedGoogle Scholar
  36. 36.
    Edelbauer M, Jungraithmayr T, Zimmerhackl LB (2005) Rituximab in childhood systemic lupus erythematosus refractory to conventional immunosuppression: case report. Pediatr Nephrol 20: 811–813CrossRefPubMedGoogle Scholar
  37. 37.
    Ostendorf B, Iking-Konert C, Kurz K et al. (2005) Preliminary results of safety and efficacy of the interleukin 1 receptor antagonist anakinra in patients with severe lupus arthritis. Ann Rheum Dis 64: 630–633CrossRefPubMedGoogle Scholar
  38. 38.
    Kuiper-Geertsma DG, Derksen RH (2003) Newer drugs for the treatment of lupus nephritis. Drugs 63: 167–180CrossRefPubMedGoogle Scholar
  39. 39.
    Bader-Meunier B, Armengaud JB, Haddad E et al. (2005) Initial presentation of childhood-onset systemic lupus erythematosus: a French multicenter study. J Pediatr 146: 648–653CrossRefPubMedGoogle Scholar
  40. 40.
    Glidden RS, Mantzouranis EC, Borel Y (1983) Systemic lupus erythematosus in childhood: clinical manifestations and improved survival in fifty-five patients. Clin Immunol Immunopathol 29: 196–210CrossRefPubMedGoogle Scholar
  41. 41.
    Platt JL, Burke BA, Fish AJ et al. (1982) Systemic lupus erythematosus in the first two decades of life. Am J Kidney Dis 2(Suppl 1): 212–222Google Scholar

Copyright information

© Springer Medizin Verlag 2006

Authors and Affiliations

  • J. Brunner
    • 1
  • C. Sergi
    • 2
  • T. Jungraithmayr
    • 1
  • L. B. Zimmerhackl
    • 1
  1. 1.Pädiatrische Rheumatologie, Klinik für Pädiatrie IDepartment für Kinder- und Jugendheilkunde, Medizinische Universität InnsbruckInnsbruckÖsterreich
  2. 2.Institut für Pathologie, Medizinische Universität InnsbruckInnsbruckÖsterreich

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