The European Alliance of Associations for Rheumatology (EULAR), in partnership with the Paediatric Rheumatology European Society (PReS), has developed new recommendations for the diagnosis and management of Still’s disease, comprising the two diseases formerly known as systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still’s disease (AOSD).
sJIA and AOSD share the same features and the same four major symptoms. However, the two conditions have traditionally been separated by a person’s age at diagnosis. Until now, before the age of 16, a patient would be diagnosed with sJIA, and after 16 they would be diagnosed with AOSD. Critically, this has translated into different treatment options, noted EULAR.
In recent years, several drugs have been approved in sJIA but not in AOSD – mainly due to the rarity of the adult-onset form, which has made it difficult to run trials, according to EULAR. This has meant that effective, advanced therapies have been accessible only for people whose disease started before the age of 16 years. People with AOSD, whilst experiencing the same disease, have been left behind. To address this, EULAR has worked with PReS to develop new recommendations to unify these two diseases and give advice on diagnosis and management. The work was completed by a taskforce of researchers, healthcare professionals, and patient representatives. The group completed a literature review to collect up-to-date evidence.
The work, published in the Annals of the Rheumatic Diseases, includes 14 individual recommendations and four overarching principles. All overarching principles and recommendations were agreed by over 80 per cent of the taskforce experts with a high level of agreement.
Crucially, the first of the overarching principles stresses that EULAR and PReS see sJIA and AOSD as the same disease and recommend that they are called by the same name: Still’s disease instead of sJIA or AOSD. The principles go on to say that treatment targets and the strategy for looking after people with Still’s disease should be based on shared decision-making between the patient (and their parents or caregivers for young patients) and the treating team.
Recommendations
Of the 14 recommendations, three are around diagnosis, two focus on timing and targets, three are on treatment choices, and the remaining six look at specific complications of Still’s disease.
The document emphasises that drug-free remission is the goal for patients across all ages and is now feasible for many given the efficacy of IL-1 or IL-6 inhibitors. Therefore, a treat-to-target strategy, similar to other rheumatological conditions, is advised.
Two therapeutic targets were defined: Clinically inactive disease (CID) and remission, that is, CID maintained for at least six months. The optimal therapeutic strategy relies on early use of IL-1 or IL-6 inhibitors associated to short-duration glucocorticoids (GC). The document acknowledges that inhibiting IL-6 or IL-1 may not cover the full spectrum of the pathogenic pathways involved in Still’s disease and some may not respond. There is definitely some heterogeneity in Still’s disease, both from a clinical and a pathogenic perspective, EULAR notes.
There is a risk of macrophage activation syndrome (MAS), a severe and potentially fatal complication, in people with Still’s disease, and this should be detected promptly and treated rapidly, the new recommendations emphasise.
MAS treatment should rely on high-dose GC, IL-1 inhibitors, ciclosporin and interferon-γ inhibitors. In recent years, a specific concern has arisen regarding cases of severe lung disease in children with Still’s disease, for which T-cell directed immunosuppressants are suggested. The recommendations emphasised the key role of expert centres for difficult-to-treat patients.
These are the first consensus recommendations for
the diagnosis and management of children and adults with Still’s disease
Conclusion
These are the first consensus recommendations for the diagnosis and management of children and adults with Still’s disease.
EULAR hope the new recommendations will help to unify two previously separate diseases and improve outcomes for all patients, no matter their age at diagnosis.
Source
Fautrel B, et al. EULAR/PReS recommendations for the diagnosis and management of Still’s disease, comprising systemic juvenile idiopathic arthritis and adult-onset Still’s disease. Ann Rheum Dis. 2024 Nov 14;83(12):1614-1627
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