Medizinische Universität Graz Austria/Österreich - Forschungsportal - Medical University of Graz
Gewählte Publikation:
Ruff, J.
Therapeutic strategies in Adult-onset Still's disease
Humanmedizin; [ Diplomarbeit ] Graz Medical University; 2018. pp. 87
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- Autor*innen der Med Uni Graz:
- Betreuer*innen:
- Heinemann Akos
- Altmetrics:
- Abstract:
- Adult-onset Still’s disease is a rare autoinflammatory syndrome of unknown etiology, which affects mostly young adults. Its clinical presentation is heterogenous, but usually characterized by a typical salmon-pink rash, high spiking fever and arthritis. Organ involvement such as hepato-/splenomegaly, lymphadenopathy and serositis occur frequently. High levels of ferritin and inflammatory markers are often present, especially glycosylated ferritin seems to be affected and may serve as a marker for disease activity. Currently, new findings concerning the patients’ cytokine profiles lead to the assumption that there exist two subtypes of AOSD with different clinical manifestation and immunologic profile: A systemic course with high levels of IL-18,IL-6 und IL-1ß, INF-¿, high fever, organ involvement and exaggerate levels of ferritin, with a greater risk of severe complications such as Macrophage Activation Syndrome; and an articular course with arthritis as the predominating symptom and high levels of IL-8, IL-17,IL-6,IL-23 und TNF-a. This course is more likely to become chronical. Until now there are no general diagnostic criteria available, but Yamaguchi criteria are the most widely used ones. There exist no general guidelines concerning therapeutic strategies, either. Most physicians choose steroids as first line therapy, which often results in steroid dependence as a severe side effect. New biologic DMARD already showed good results in off-label use, but their efficacy could only be evaluated by retrospective data analyses and case reports. Patients with systemic disease course seem to benefit particularly from Interleukin-1 inhibitors and a promising interleukin-18 inhibitor will be provided soon. For articular manifestations TNF-a inhibitors and the early use of methotrexat show good results. Steroids still remain first-line therapy, but therapeutic strategies must be adapted to disease course and cytokine profile of the patients to successfully treat refractory AOSD. Large cohort controlled randomized trials are indispensable to further elucidate the efficacy of biologicals in AOSD. A focus should also lie on the development of general guidelines for the management of AOSD patients and further research concerning cytokine – and genetic profiles.