[Adult-onset Still's disease : Diagnosis and treatment according to the new S2e guidelines of the German Society of Rheumatology (DGRh)]. / Adultes Still-Syndrom : Diagnostik und Therapie unter Berücksichtigung der neuen DGRh(Deutsche Gesellschaft für Rheumatologie)-S2e-Leitlinie.
Z Rheumatol
; 82(2): 134-142, 2023 Mar.
Article
em De
| MEDLINE
| ID: mdl-36515738
ABSTRACT
Adult-onset Still's disease (AOSD) is a rare autoinflammatory disease characterized by intermittent fever and a combination of symptoms, such as an evanescent rash synchronous with fever, arthralgia/arthritis, lymphadenopathy and hepatosplenomegaly. The diagnosis is based on a characteristic constellation of symptoms and the exclusion of infections, hemato-oncological diseases, infectious diseases and alternative rheumatological causes. The systemic inflammatory reaction is reflected by high levels of ferritin and Creactive protein (CRP). The pharmacological treatment concept includes glucocorticoids often in combination with methotrexate (MTX) and ciclosporine (CSA) for reduction of steroids. The interleukin 1 (IL-1) receptor antagonist anakinra, the IL-1beta antibody canakinumab or an IL6 receptor blockage with tocilizumab (off label for AOSD) are used where there is no response to MTX or CSA. Anakinra or canakinumab can be used as a primary option in AOSD in cases of moderate and severe disease activity.
Palavras-chave
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Artrite
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Reumatologia
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Doença de Still de Início Tardio
Tipo de estudo:
Diagnostic_studies
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Guideline
Limite:
Adult
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Humans
Idioma:
De
Revista:
Z Rheumatol
Ano de publicação:
2023
Tipo de documento:
Article