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.