Treatment of Difficult-to-Treat Adult-onset Still Disease
When Adult-onset Still disease (AOSD) proves difficult to treat, the clinical situation requires careful systematic evaluation and specialist-level decision-making before advancing therapy.
This protocol addresses the difficult-to-treat (D2T) presentation of Adult-onset Still disease — a distinct clinical situation in which disease control has not been adequately achieved and management must be escalated with expert involvement.
The approach begins with ruling out active infections. Central to further management is discussion in a multidisciplinary round at a Still's disease specialist or ERN reference centre. Certain advanced and experimental therapeutic options may then be considered — the full structured regimen is available via the link below.
References
DOI: 10.1136/ard-2024-225851
- A patient who fails to achieve CID off GC after IL-1/IL-6 inhibitor rotation should be considered as D2T and should be discussed in multidisciplinary rounds with an expert in Still's disease, for example, at a reference centre (in Europe through the ERN-RITA, https://ern-rita.org/); the use of less well-established therapeutic options should be discussed.
- These include JAK inhibitors, haematopoietic stem cell transplantation and immunosuppressants.
- Experimental therapy may include JAK inhibitors, emapalumab, bispecific antibody to IL-1/IL-18.
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