Systemischer Lupus erythematodes

Zusammenfassung

Der systemische Lupus erythematodes (SLE) ist der Prototyp einer systemischen Autoimmunerkrankung mit potenzieller Multiorganbeteiligung und weist als solcher auch häufig einen „schweren Verlauf“ auf. Dieser kann z. B. durch die Prognose definiert werden, durch Art und Ausmaß der Organbeteiligung, wobei insbesondere eine Beteiligung von Nieren und zentralem Nervensystem (ZNS) einen schweren Verlauf kennzeichnen, oder durch die notwendige Therapie. Dabei ist der „schwere Verlauf“ keine statische und auch keine unumkehrbare Definition. Diese muss sich auch an dem Therapieansprechen orientieren und neben der sich daraus ergebenden Prognose auch chronische Krankheits- oder Therapiefolgen berücksichtigen. Die Definition eines „schweren Verlaufes“ entspricht somit eher einem Composit-Score über die Zeit. Man wird einen SLE insbesondere dann als „schwer verlaufend“ einstufen, wenn dieser viele Organe, vor allem Niere und ZNS in Mitleidenschaft zieht, klinisch und immunologisch hoch aktiv ist, einer aggressiven Immunsuppression bedarf (vor allem mit Cyclophosphamid und hohen Dosen von Glukokortikoiden) und dabei schlecht auf die Therapie anspricht bzw. durch frühe oder häufige Rezidive gekennzeichnet ist.

Summary

Systemic lupus erythematosus (SLE) is the prototype of a systemic autoimmune disease with potential multiple organ involvement and shows as such also often a “severe” disease course. Severeness can be defined by prognosis, type and extent of organ involvement, where particularly involvement of the kidneys and the central nervous system mark a course as “severe” or the therapy to control the disease. A “severe” course is no static and no irreversible definition. This must also be based on the response to treatment and take into account not only the resulting prognosis but also chronic damage by disease or its treatment. Thus definition of a “severe” course is more or less a composite score over time. One would consider SLE as “severe” in its course if the disease involves multiple organs, especially kidneys and central nervous system, is clinically and immunologically highly active, requires aggressive immunosuppression (especially with cyclophosphamide and high doses of corticosteroids) and if it responds poorly to therapy and is characterized by early or frequent flares.

Schlüsselwörter

Systemischer Lupus erythematodes - Autoimmunerkrankung - schwerer Verlauf - Multiorganbeteiligung

Keywords

Systemic lupus - prognosis - systemic autoimmune disease - severe disease course - multiple organ involvement

• Literatur

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