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[Update on Adult-Onset Still's Disease: Diagnosis, Therapy and Guideline].

Authors :
Vordenbäumen S
Feist E
Source :
Deutsche medizinische Wochenschrift (1946) [Dtsch Med Wochenschr] 2023 Jun; Vol. 148 (12), pp. 788-792. Date of Electronic Publication: 2023 May 31.
Publication Year :
2023

Abstract

Adult-onset Still's disease (ASOD) is an autoinflammatory disease of unknown etiology which is pathogenetically characterized by an involvement of the innate immune response with activation of neutrophils and an increased secretion of IL-1, IL-6, IL-18, type 1 interferons. Still's disease may occur at any age with distinct variability in signs and symptoms. Recently, the German Society of Rheumatology (DGRh) has issued an AOSD guideline which recommends diagnosing AOSD based on a characteristic combination of symptoms including intermittent fever, rash, arthralgia, and arthritis after exclusion of infections, neoplasms and other rheumatological conditions. Classification criteria according to Yamaguchi may support the clinical diagnosis. Therapy is recommended to include glucocorticosteroids and methotrexate or ciclosporin, at higher activity levels IL1-receptor antagonist anakinra, IL-1β antibody canakinumab, or IL6-receptor antibody tocilizumab. At a high disease activity, anakinra or canakinumab may be employed primarily. Local drug licensing policies may have to be considered, as these substances are not universally approved in these scenarios. Important complications to consider consist in perimyocarditis, a multi-faceted pulmonary involvement, and macrophage activation syndrome (MAS). MAS features multi-organ involvement and cytopenias. Besides supportive measures often requiring intensive care, high dose glucocorticosteroids as well as above named biologics, and if necessary, also etoposide based therapeutic regimen are used.<br />Competing Interests: Prof. Stefan Vordenbäumen gibt keine Interessenkonflikte an. Prof. Eugen Feist gibt an, Berater-, Gutachtertätigkeit und/oder Forschungsprojekte mit Abbvie, BMS, MSD, Roche, Lilly, Sanofi, Sobi, Novartis, Pfizer durchgeführt zu haben.<br /> (Thieme. All rights reserved.)

Details

Language :
German
ISSN :
1439-4413
Volume :
148
Issue :
12
Database :
MEDLINE
Journal :
Deutsche medizinische Wochenschrift (1946)
Publication Type :
Academic Journal
Accession number :
37257482
Full Text :
https://doi.org/10.1055/a-2000-3446