Skip to main content
Log in

Ätiopathogenese des systemischen Lupus erythematodes

Aetiology and pathogenesis of systemic lupus erythematosus

  • Leitthema
  • Published:
Monatsschrift Kinderheilkunde Aims and scope Submit manuscript

Zusammenfassung

Der systemische Lupus erythematodes ist eine systemische Autoimmunerkrankung, die durch die Produktion von zahlreichen Autoantikörpern, insbesondere antinukleären Antikörpern und Antikörpern gegen Doppelstrang-DNA, aber auch von Autoantikörpern gegen verschiedene Organe und Zellsysteme charakterisiert ist. Ätiologisch von Bedeutung sind sowohl Umwelteinflüsse, zu denen neben weiblichen Geschlechtshormonen und der UV-Strahlung auch Infektionen zu zählen sind, als auch eine genetische Prädisposition. Hier sind genetische Polymorphismen in Immunglobulin-Fc-Rezeptor-Genen, Komplementgenen, Zytokingenen und in Genen, die die Apoptose regulieren, zu nennen. Die Pathogenese ist gekennzeichnet durch eine Entzündung, die von Autoantikörpern, Immunkomplexen, aktivierten und autoreaktiven Lymphozyten geprägt und durch Ablagerung von Immunkomplexen, Komplementumsatz und zellulären Infiltraten in Geweben und Gefäßen charakterisiert ist. Die vorliegende Übersicht soll einen Einblick in aktuelle Konzepte zur Pathogenese des SLE geben.

Abstract

Systemic lupus erythematosus (SLE) is a systemic episodic autoimmune disease characterized by numerous autoantibodies, especially antinuclear antibodies and antibodies to double-strand DNA but also autoantibodies against various organs and cell systems. The heterogeneity of the clinical manifestations reflects the complexity of the disease’s pathogenesis. The aetiology is not completely understood, but environmental factors, including female sex hormones, ultraviolet light exposure and infections, play an important part, as does a genetic predisposition with genetic polymorphisms in immunoglobulin-Fc receptor genes, cytokine genes, complement genes and genes regulating apoptosis. The pathogenesis is linked with an inflammatory process involving autoantibodies, immune complexes and activated and autoreactive lymphocytes and is characterized by deposits of immune complexes, complement turnover and vascular and parenchymal cellular infiltrates in numerous tissues. This article reviews current concepts of the pathogenesis of SLE.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Abb. 1

Literatur

  1. Ackerman LS (2006) Sex hormones and the genesis of autoimmunity. Arch Dermatol 142: 371–376

    CAS  PubMed  Google Scholar 

  2. Bennett L, Paluckaa AK, Arcee E et al. (2003) Interferon and granulopoiesis signatures in systemic lupus erythematosus blood. J Exp Med 197: 711–723

    Article  CAS  Google Scholar 

  3. Bijl M, Reefman E, Horst G et al. (2006) Reduced uptake of apoptotic cells by macrophages in systemic lupus erythematosus: correlates with decreased serum levels of complement. Ann Rheum Dis 65: 57–63

    Article  CAS  Google Scholar 

  4. Chen YF, Morel L (2005) Genetics of T cell defects in lupus. Cell Mol Immunol 2: 403–409

    CAS  PubMed  Google Scholar 

  5. Childs SG (2006) The pathogenesis of systemic lupus erythematosus. Orthop Nurs 25: 140–145

    PubMed  Google Scholar 

  6. Criswell LA, Amos CI (2000) Update on genetic risk factors for systemic lupus erythematosus and rheumatoid arthritis. Curr Opin Rheumatol 12: 85–90

    Article  CAS  Google Scholar 

  7. Crow MK, Kirou KA (2004) Interferon-α in systemic lupus erythematosus. Curr Opin Rheumatol 16: 541–547

    Article  CAS  Google Scholar 

  8. Dean GS, Tyrrell-Price J, Crawley E et al. (2000) Cytokines and systemic lupus erythematosus. Ann Rheum Dis 59: 243–251

    Article  CAS  Google Scholar 

  9. Dijstelbloem HM, Bijl M, Fijnheer R et al. (2000) Fcγ receptor polymorphisms in systemic lupus erythematosus: association with disease and in vivo clearance of immune complexes. Arthritis Rheum 43: 2793–2800

    Article  CAS  Google Scholar 

  10. Doria A, Iaccarino L, Arienti S et al. (2006) Th2 immune deviation induced by pregnancy: the two faces of autoimmune rheumatic diseases. Reprod Toxicol 22: 234–241

    Article  CAS  Google Scholar 

  11. Gaipl US, Kuhn A, Sheriff A et al. (2006) Clearance of apoptotic cells in human SLE. Curr Dir Autoimmun 9: 173–187

    CAS  PubMed  Google Scholar 

  12. Gattorno M, Picco P, Barbano G et al. (1998) Differences in tumor necrosis factor alpha soluble receptor serum concentrations between patients with Henoch-Schoenlein purpura and pediatric systemic lupus erythematosus. J Rheumatol 25: 361–365

    CAS  PubMed  Google Scholar 

  13. Hagiwara E, Gourley MF, Lee S et al. (1996) Disease severity in patients with systemic lupus erythematosus correlates with an increased ratio of interleukin-10: interferon-γ-secreting cells in the peripheral blood. Arthritis Rheum 39: 379–385

    Article  CAS  Google Scholar 

  14. Kuhn A, Beissert S (2005) Photosensitivity in lupus erythematosus. Autoimmunity 38: 519–529

    Article  CAS  Google Scholar 

  15. Kyttaris VC, Tsokos GC (2004) T lymphocytes in systemic lupus erythematosus: an update. Curr Opin Rheumatol 16: 548–552

    Article  Google Scholar 

  16. Lee YJ, Shin KS, Kang SW et al. (2004) Association of the oestrogen receptor α gene polymorphisms with disease onset in systemic lupus erythematosus. Ann Rheum Dis 63: 1244–1249

    Article  CAS  Google Scholar 

  17. Looney RJ, Anolik J, Sanz I (2004) B lymphocytes in systemic lupus erythematosus: lessions from therapy targeting B cells. Lupus 13: 1–10

    Article  Google Scholar 

  18. Maddison PJ (1999) Nature and nurture in systemic lupus erythematosus. Adv Exp Med Biol 455: 7–13

    Article  CAS  Google Scholar 

  19. Magnusson V, Nakken B, Bolstad AI et al. (2001) Cytokine polymorphisms in systemic lupus erythematosus and Sjögren’s syndrome. Scand J Immunol 54: 55–61

    Article  CAS  Google Scholar 

  20. Manger K, Repp R, Jansen M et al. (2002) Fcγ receptor IIa, IIIa, and IIIb polymorphisms in German patients with systemic lupus erythematosus: association with clinical symptoms. Ann Rheum Dis 61: 786–792

    Article  CAS  Google Scholar 

  21. Mason LJ, Isenberg DA (1998) Immunopathogenesis of SLE. Baillieres Clin Rheumatol 12: 385–403

    Article  CAS  Google Scholar 

  22. Petri M, Robinson C (1997) Oral contraceptives and systemic lupus erythematosus. Arthritis Rheum 40: 797–803

    Article  CAS  Google Scholar 

  23. Petty RE (1998) Etiology and pathogenesis of rheumatic diseases of adolescence. Adolesc Med 9: 11–24

    CAS  PubMed  Google Scholar 

  24. Prokunina L, Alarcon-Riquelme M (2004) The genetic basis of systemic lupus erythematosus – knowledge of today and thoughts for tomorrow. Hum Mol Genet 13: R143–R148

    Article  CAS  Google Scholar 

  25. Reveille JD (2005) Genetic studies in the rheumatic diseases: present status and implications for the future. J Rheumatol Suppl 72: 10–13

    PubMed  Google Scholar 

  26. Singh AK (1993) Abnormalities in the regulation of variable region genes that encode for antibodies to DNA may be a central factor in the pathogenesis of systemic lupus erythematosus. Ann Rheum Dis 52: 378–383

    Article  CAS  Google Scholar 

  27. Smyth LJC, Snowden N, Carthy D et al. (1997) Fcγ RIIa polymorphism in systemic lupus erythematosus. Ann Rheum Dis 56: 744–746

    Article  CAS  Google Scholar 

  28. Tsao BP (2004) Update on human systemic lupus erythematosus genetics. Curr Opin Rheumatol 16: 513–521

    Article  CAS  Google Scholar 

  29. Vaishnaw AK, McNally JD, Elkon KB (1997) Apoptosis in the rheumatic diseases. Arthritis Rheum 40: 1917–1927

    Article  CAS  Google Scholar 

Download references

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.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to G. Horneff.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Horneff, G. Ätiopathogenese des systemischen Lupus erythematodes. Monatsschr Kinderheilkd 154, 1177–1188 (2006). https://doi.org/10.1007/s00112-006-1442-0

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00112-006-1442-0

Schlüsselwörter

Keywords

Navigation