Zum Hauptinhalt springen

Systemischer Lupus erythematodes (SLE)

Zuletzt bearbeitet: Zuletzt revidiert:
Zuletzt revidiert von:


Definition:
Der systemische Lupus erythematodes (SLE) ist eine chronische, in Schüben verlaufende Autoimmunerkrankung. Es können praktisch alle Organe in unterschiedlichem Schweregrad betroffen sein. Klinisches Bild, Verlauf und Prognose sind ausgesprochen variabel.
Prävalenz:
Sie beträgt in Deutschland etwa 40/100.000, das Verhältnis Frauen zu Männer liegt etwa bei 9:1.
Symptome:
Die Erkrankung kann alle Organsysteme betreffen. Neben Allgemeinsymptomen wie Abgeschlagenheit und Fieber betrifft die Symptomatik vor allem Gelenke, Haut, Nieren und zentrales Nervensystem.
Befunde:
Neben den Veränderungen im Bereich der betroffenen Organsysteme werden hämatologische (Anämie, Leukozytopenie, Thrombozytopenie) und immunologische (verschiedene Autoantikörper) Befunde erhoben.
Diagnostik:
Anhand eines Klassifikationssystems, das klinische, hämatologische und immunologische Kriterien umfasst.
Therapie:
Allgemeinmaßnahmen wie Sonnenschutz und Impfschutz. Medikamentös mit Antimalariamitteln, Glukokortikoiden, Immunsuppressiva und Biologika.
  • European League Against Rheumatism (EULAR). Update of the EULAR recommendations for the management of systemic lupus erythematosus. Stand 2019. www.eular.org 
  • European League Against Rheumatism (EULAR). Recommendations for the management of systemic lupus erythematosus with neuropsychiatric manifestations. Stand 2010. www.eular.org 
  • European League Against Rheumatism (EULAR). Update of the  recommendations for the management of lupus nephritis. Stand 2019. www.ard.bmj.com 
  • European League Against Rheumatism (EULAR). Recommendations for women's health and the management of family planning, assisted reproduction, pregnancy and menopause in patients with systemic lupus erythematosus and/or antiphospholipid syndrome. Stand 2017. www.eular.org 
  • T2T/SLE Working Group. Treat-to-target in systemic lupus erythematosus: recommendations from an international task force. Stand 2014. www.ard.bmj.com 

 

  1. Tsokos GC. Systemic lupus erythematosus. N Engl J Med 2011; 365: 2110-21. pmid:22129255. PubMed  
  2. Kuhn A, Bonsmann G, Anders H-J, Herzer P, Tenbrock K, Schneider M. The Diagnosis and Treatment of Systemic Lupus Erythematosus. Dtsch Arztebl Int. 2015 Jun 19;112(25):423-32. www.ncbi.nlm.nih.gov  
  3. Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 1997;40:1725. www.ncbi.nlm.nih.gov  
  4. Gergianaki I, Bertsias G. Systemic Lupus Erythematosus in Primary Care: An Update and Practical Messages for the General Practitioner. Front Med (Lausanne) 2018; 5:161. www.ncbi.nlm.nih.gov  
  5. Lam N, Ghetu M, Bieniek M. Systemic Lupus Erythematosus: Primary Care Approach to Diagnosis and Management. Am Fam Physician 2016; 94: 284- 294. www.aafp.org  
  6. American College of Rheumatology Ad Hoc Committee on Systemic Lupus Erythematosus Guidelines. Guidelines for referral and management of systemic lupus erythematosus in adults. Arthritis Rheum. 1999; 42(9):1785-1796. www.ncbi.nlm.nih.gov  
  7. D'Cruz DP. Systemic lupus erythematosus. BMJ 2006; 332: 890-4. PubMed  
  8. Rees F, Doherty M, Grainge M, et al. The worldwide incidence and prevalence of systemic lupus erythematosus: a systematic review of epidemiological studies.. Rheumatology 2017; 56: 1945-1961. doi:10.1093/rheumatology/kex260 DOI  
  9. Bartels C. Systemic Lupus Erythematosus (SLE). Medscpae, Updated: Nov 14, 2017. Zugriff 28.10.17. emedicine.medscape.com  
  10. Schwarting A (Deutsche Gesellschaft für Innere Medizin). Systemischer Lupus erythematodes. e.Medpedia, Zugriff 15.08.20. www.springermedizin.de  
  11. Groot N, Graeff N, Avcin T, et al. European evidence-based recommendations for diagnosis and treatment of childhood-onset systemic lupus erythematosus: the SHARE initiative. Ann Rheum Dis 2017 Nov; 76(11): 1788-1796. pmid:28630236 PubMed  
  12. Tenbrock K, Horneff G. Systemischer Lupus erythematodes. Leitlinie der Gesellschaft für Kinder- und Jugendrheumatologie der Deutschen Gesellschaft für Kinder- und Jugendmedizin. AWMF-Nr. 027-061. Stand 2013. docplayer.org  
  13. La Paglia G, Leone M, Lepri G, et al. One year in review 2017: systemic lupus erythematosus. Clin Exp Rheumatol 2017; 35: 551-561. pmid:28721860 PubMed  
  14. Mok C, Lau S. Pathogenesis of systemic lupus erythematosus. J Clin Pathol 2003; 56: 481-490. www.ncbi.nlm.nih.gov  
  15. Choi J, Kim S, Craft J. The Pathogenesis of Systemic Lupus Erythematosus – An Update. Curr Opin Immunol 2012; 24: 651-657. www.ncbi.nlm.nih.gov  
  16. Arbuckle M, McClain M, Rubertone M, et al. Development of autoantibodies before the clinical onset of systemic lupus erythematosus. N Engl J Med 2003; 349: 1526-33. PubMed  
  17. Ramos P, Brown E, Langefeld C. Genetic Factors Predisposing to Systemic Lupus Erythematosus and Lupus Nephritis. Semin Nephrol 2010; 30: 164-176. www.ncbi.nlm.nih.gov  
  18. Moser, KL, Kelly, JA, Lessard CJ, Harley JB. Recent insights into the genetic basis of systemic lupus erythematosus. Genes Immun 2009; 10: 373-9. doi:10.1038/gene.2009.39 DOI  
  19. Lehmann P, Homey B. Clinic and pathophysiology of photosensitivity in lupus erythematosus. Autoimmun Rev 2009; 8: 456-61. pmid:19167524 PubMed  
  20. Bengtsson A, Rylander L, Hagmar L, et al. Risk factors for developing systemic lupus erythematosus: a case-control study in southern Sweden.. Rheumatology (Oxford) 2002; 41: 563-571. pmid:12011382 PubMed  
  21. Doria A, Cutolo M, Ghirardello A, et al. Steroid hormones and disease activity during pregnancy in systemic lupus erythematosus. Arthritis Rheum 2002; 47: 202-9. pmid:11954015 PubMed  
  22. Aringer M, Costenbader K, Daikh D, et al. 2019 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus. Arthritis & Rheumatology 2019; 71: 1400-1412. doi:10.1002/art.40930 DOI  
  23. Mucke J, Fischer-Betz R, Schneider M. State of the Art: systemischer Lupus erythematodes. Z Rheumatol 2019; 78: 500-510. doi:10.1007/s00393-019-0633-8 DOI  
  24. Neue Klassifikations­kriterien für Lupus erythematodes Aerzteblatt.de, Freitag 07. September 2018. Zugriff 01.11.2018. www.aerzteblatt.de  
  25. Vu Lam N-C, Ghetu MV, Bieniek ML. Systemic lupus erythematosus: Primary care approach to diagnosis and management. Am Fam Physician 2016 Aug 15; 94(4): 284-94. pmid:27548593 PubMed  
  26. Okon LG, Werth VP. Cutaneous Lupus Erythematosus: Diagnosis and treatment. Best Pract Res Clin Rheumatol 2013; 27: 391-404. pmid:24238695 PubMed  
  27. Unholzer A. Kutaner Lupus erythematodes: Symptome. Apotheken-Umschau online, aktualisiert am 05.01.17. Zugriff 02.11.18. www.apotheken-umschau.de  
  28. Rubin RL. Drug-induced lupus. Expert Opin Drug Saf 2015; 14: 361-78. pmid:25554102 PubMed  
  29. Kauffman C. Drug-induced lupus erythematosus. Medscape, updated: Jul 10, 2018. Zugriff 02.11.18. emedicine.medscape.com  
  30. Rheumaliga Schweiz. Systemischer Lupus erythematosus. Zugriff 01.11.18. www.rheumaliga.ch  
  31. Gluhovschi C, Gluhovschi G, Petrica L, et al. Pregnancy Associated with Systemic Lupus Erythematosus: Immune Tolerance in Pregnancy and Its Deficiency in Systemic Lupus Erythematosus--An Immunological Dilemma. J Immunol Res 2015. pmid:26090485 PubMed  
  32. Hon K, Leung H. Neonatal Lupus Erythematosus. Autoimmune Diseases 2012; 2012: 1-6. www.ncbi.nlm.nih.gov  
  33. Femia A. Neonatal and Pediatric Lupus Erythematosus. Medscape, updated: Jun 08, 2016. Zugriff 03.11.2018. emedicine.medscape.com  
  34. Lorenz H, Pieterse L, Rüter T , et al. Fieber bei systemischem Lupus erythematodes: Krankheitsschub oder Infektion?. Z Rheumatol 2020; 79: 325-331. doi:10.1007/s00393-020-00773-8 DOI  
  35. Fanouriakis A, Kostopoulou M, Alunno A, et al. 2019 update of the EULAR recommendations for the management of systemic lupus erythematosus. Ann Rheum Dis 2019;78:736–745. ard.bmj.com  
  36. van Vollenhoven R, Mosca M, Bertsias G, et al. Treat-to-target in systemic lupus erythematosus: recommendations from an international task force. Ann Rheum Dis 2014; 73: 958-967. doi:10.1136/annrheumdis-2013-205139 DOI  
  37. Mucke J, Düsing C, Chehab G, et al. Treat-to-Target beim systemischen Lupus erythematodes. Z Rheumatol 2020; 79: 351-358. doi:10.1007/s00393-020-00771-w DOI  
  38. Aringer M, Leuchten N, Schneider M. Treat-to-Target bei systemischem Lupus erythematodes. Kompass Autoimmun 2020; 2: 45-51. doi:10.1159/000507644 DOI  
  39. Bertsias G, Ioannidis J, Boletis J, et al. EULAR recommendations for the management of systemic lupus erythematosus. Ann Rheum Dis 2008; 67: 195-205. doi:10.1136/ard.2007.070367 DOI  
  40. Bertsias G, Ioannidis J, Aringer M, et al. EULAR recommendations for the management of systemic lupus erythematosus with neuropsychiatric manifestations. Ann Rheum Dis 2010; 69: 2074-2082. doi:10.1136/ard.2010.130476 DOI  
  41. Bertsias G, Tektonidou M, Amoura Z, et al. Joint European League Against Rheumatism and European Renal Association–European Dialysis and Transplant Association (EULAR/ERA-EDTA) recommendations for the management of adult and paediatric lupus nephritis. Ann Rheum Dis 2012; 71: 1771-1782. doi:10.1136/annrheumdis-2012-201940 DOI  
  42. Andreoli L, Bertsias G, Agmon-Levin N, et al. EULAR recommendations for women’s health and the management of family planning, assisted reproduction, pregnancy and menopause in patients with systemic lupus erythematosus and/or antiphospholipid syndrome. Ann Rheum Dis 2017; 76: 476-485. doi:10.1136/annrheumdis-2016-209770 DOI  
  43. Carvalho MR, Sato EI, Tebexreni AS, Heidecher RT, Schenkman S, Neto TL. Effects of supervised cardiovascular training program on exercise tolerance, aerobic capacity, and quality of life in patients with systemic lupus erythematosus. Arthritis Rheum 2005; 53: 838-44. PubMed  
  44. Berufsverband Deutscher Rheumatologen. Systemischer Lupus erythematodes. Zugriff 01.11.18. www.bdrh.de  
  45. Ruiz-Irastorza G, Ramos-Casals M, Brito-Zeron P, Khamashta MA. Clinical efficacy and side effects of antimalarials in systemic lupus erythematosus: a systematic review. Ann Rheum Dis 2010; 69: 20-8. PubMed  
  46. Hannah J, Casian A, D'Cruz D. Tacrolimus use in lupus nephritis: A systematic review and meta-analysis. Autoimmun Rev 2015. pmid:26427983 PubMed  
  47. Sakthiswary R, Suresh E. Methotrexate in systemic lupus erythematosus: a systematic review of its efficacy. Lupus. 2014 Mar;23(3):225-35. doi: 10.1177/0961203313519159. DOI  
  48. Navarra SV, Guzmán RM, Gallacher AE, et al. Efficacy and safety of belimumab in patients with active systemic lupus erythematosus: a randomised, placebo-controlled, phase 3 trial. Lancet 2011; 377: 721-31. PubMed  
  49. Furie R, Petri M, Zamani O, et al. A phase III, randomized, placebo-controlled study of belimumab, a monoclonal antibody that inhibits B lymphocyte stimulator, in patients with systemic lupus erythematosus. Arthritis Rheum 2011; 63: 3918-30. PubMed  
  50. Deutsche Gesellschaft für Rheumatologie. Empfehlungen zum Einsatz von Belimumab beim Systemischen Lupus erythematodes. Zugriff 19.08.20. dgrh.de  
  51. Srivastava A. Belimumab in Systemic Lupus Erythematosus. Indian J Dermatol 2016; 61: 550-553. www.e-ijd.org  
  52. Hoyer B. Neue Therapien beim systemischen Lupus erythematodes. Z Rheumatol 2020; 79: 342-350. doi:10.1007/s00393-020-00788-1 DOI  
  53. Lettau M, Schrezenmeier E, Specker C, et al. Lupus und Thrombophilie - Antiphospholipidsyndrom. Z Rheumatol 2020; 79: 332-341. doi:10.1007/s00393-020-00786-3 DOI  
  54. Boser M, Kielstein J. Rolle von Plasmapherese und Immunadsorption in der Rescue-Therapie rheumatologischer Erkrankungen. Z Rheumatol 2016; 75: 964–972. doi:10.1007/s00393-016-0219-7 DOI  
  55. Fischer-Betz R, Herzer P, Schneider M. Systemischer Lupus erythematodes. Dtsch Med Wochenschr 2005; 130: 2451-2458. doi:10.1055/s-2005-918589 DOI  
  56. Fanouriakis A, Kostopoulou M, Cheema K, et al. 2019 Update of the Joint European League Against Rheumatism and European Renal Association– European Dialysis and Transplant Association (EULAR/ ERA–EDTA) recommendations for the management of lupus nephritis. Ann Rheum Dis 2020;79:713–723. ard.bmj.com  
  57. Muangchan C, van Vollenhoven RF, Bernatsky SR, et al. Treatment algorithms in systemic lupus erythematosus. Arthritis Care Res (Hoboken) 2015. pmid:25777803 PubMed  
  58. Weening J, D’Agati V, Schwartz M, et al. The Classification of Glomerulonephritis in Systemic Lupus Erythematosus Revisited. Kidney Int 2004; 65: 521–530. www.ncbi.nlm.nih.gov  
  59. Henderson L, Masson P, Craig JC, Flanc RS, Roberts MA, Strippoli GFM, Webster AC. Treatment for lupus nephritis. Cochrane Database of Systematic Reviews 2012, Issue 12. Art. No.: CD002922. DOI: 10.1002/14651858.CD002922.pub3. pubmed.ncbi.nlm.nih.gov  
  60. Tunnicliffe DJ, Palmer SC, Henderson L, et al. Immunosuppressive treatment for proliferative lupus nephritis. Cochrane Database of Systematic Reviews 2018, Issue 6. Art. No.: CD002922. DOI: 10.1002/14651858.CD002922.pub4 DOI  
  61. Garcia D, Erkan D. Diagnosis and Management of the Antiphospholipid Syndrome. N Engl J Med 2018; 378: 2010-2021. doi:10.1056/NEJMra1705454 DOI  
  62. Buyon JP, Kim MY, Guerra MM, et al. Predictors of Pregnancy Outcomes in Patients With Lupus: A Cohort Study. Ann Intern Med 2015; 163: 153-63. pmid:26098843 PubMed  
  63. Stojan G, Baer AN. Flares of systemic lupus erythematosus during pregnancy and the puerperium: prevention, diagnosis and management. Expert Rev Clin Immunol 2012; 8: 439-53. pmid:22882219 PubMed  
  64. Fischer-Betz R, Haase I. Schwangerschaft bei Lupus erythematodes – ein Update. Z Rheumatol 2020; 79: 359-366. doi:10.1007/s00393-020-00772-9 DOI  
  65. Petri M, Kim MY, Kalunian KC, et al.; OC-SELENA Trial. Combined oral contraceptives in women with systemic lupus erythematosus. N Engl J Med 2005; 353: 2550-8. New England Journal of Medicine  
  66. ACOG Committee on Practice Bulletins-Gynecology. ACOG practice bulletin. No. 73: Use of hormonal contraception in women with coexisting medical conditions. Obstet Gynecol 2006; 107: 1453-72. PubMed  
  67. Schoenfeld SR, Kasturi S, Costenbader KH. The epidemiology of atherosclerotic cardiovascular disease among patients with SLE: a systematic review. Semin Arthritis Rheum. (2013) 43:77–95. doi:10.1016/j.semarthrit.2012.12.002 DOI  
  68. El-Magadmi M, Bodill H, Ahmad Y, Durrington PN, Mackness M, Walker M, et al. Systemic lupus erythematosus: an independent risk factor for endothelial dysfunction in women. Circulation 2004; 110: 399-404. PubMed  
  69. Rivest C, Lew RA, Welsing PM, Sangha O, Wright EA, Roberts WN, et al. Association between clinical factors, socioeconomic status, and organ damage in recent onset systemic lupus erythematosus. J Rheumatol 2000;27:680-4. PubMed  
  70. Yurkovich M, Vostretsova K, Chen W, Aviña-Zubieta JA. Overall and cause-specific mortality in patients with systemic lupus erythematosus: a meta-analysis of observational studies. Arthritis Care Res (Hoboken) 2014; 66: 608-16. pmid:24106157 PubMed  
  71. Ahn G, Ramsey-Goldman R.. Fatigue in systemic lupus erythematosus. Int J Clin Rheumtol 2012; 7: 217-227. www.ncbi.nlm.nih.gov  
  72. Jump RL, Robinson ME, Armstrong AE, Barnes EV, Kilbourn KM, Richards HB. Fatigue in systemic lupus erythematosus: contributions of disease activity, pain, depression, and perceived social support. J Rheumatol 2005; 32: 1699-705. PubMed  
  73. Wallace D. Improving the prognosis of SLE without prescribing lupus drugs and the primary care paradox. Lupus 2008. doi:10.1177/0961203307086267 DOI  
  • Michael Handke, Prof. Dr. med., Facharzt für Innere Medizin, Kardiologie und Intensivmedizin, Freiburg i. Br.

Frühere Autor*innen

  • Die ursprüngliche Version dieses Artikels basiert auf einem entsprechenden Artikel im norwegischen hausärztlichen Online-Handbuch Norsk Elektronisk Legehåndbok(NEL, https://legehandboka.no/).