Still’s disease

New recommendations for diagnosis and treatment

The EULAR/PReS recommendations published in the Annals of Rheumatic Diseases 2024 represent a significant advancement in the management of Still's disease. These new recommendations establish a unified approach to systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still's disease (AOSD), paving the way for more consistent and effective treatment across all age groups. The recommendations introduce a paradigm shift in how we conceptualize, diagnose, and treat Still's disease, effectively bridging the gap between pediatric and adult rheumatology.¹

Still´s – One disease¹

These recommendations recognize systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still's disease (AOSD) as a single entity: "Still's disease." This unification eliminates the arbitrary age threshold of 16 years, acknowledging the disease continuum and promoting consistent management strategies regardless of age at onset.

New diagnosis approach¹

A crucial change in the diagnostic approach is that arthritis is no longer required for diagnosis, preventing harmful diagnostic delays since arthritis typically appears about 1 month after disease onset.

Key diagnostic indicators include:

• Fever (≥39°C for at least 7 days)
• Transient rash coinciding with fever spikes
• Musculoskeletal involvement
• Elevated inflammation markers

Biomarkers like serum IL-18 and S100 proteins should be measured when available to support clinical diagnosis. As Still's disease is typically diagnosed by exclusion, alternative diagnoses should be carefully considered.

The new approach emphasizes early intervention with biologics (IL-1 or IL-6 inhibitors) alongside short-term glucocorticoids. This targets the "window of opportunity" to potentially alter disease course and improve outcomes.

Treatment aims to achieve Clinically Inactive Disease (CID) and ultimately drug-free remission, maintaining CID for at least 6 months without medication. A treat-to-target approach with defined milestones prioritizes biologics over prolonged glucocorticoid use. While both IL-1 and IL-6 inhibitors effectively control symptoms, anakinra (IL-1 inhibitor) is often preferred initially due to its short half-life, favorable safety profile, and efficacy against macrophage activation syndrome.

Generally, IL-1 inhibitors show fewer serious adverse events compared to IL-6 inhibitors, making them a slightly safer option overall. If a patient doesn't respond adequately to one inhibitor type, the guidelines recommend rotating to the other before exploring alternative treatments, allowing for a flexible, personalized approach to disease management.

Kineret – Early start, sustained inactive disease²

A prospective cohort study of 42 newly-diagnosed sJIA patients, who had an unsatisfactory response to non-steroidal anti-inflammatory drugs, demonstrated Kineret's effectiveness as first-line treatment. With a treat-to-target strategy, 59.5% achieved CID at 1 month, 76% at 1 year, and 96% maintained CID after 5 years. Notably, 57% required only Kineret and NSAIDs during the median 5.8-year follow-up, potentially reducing glucocorticoid dependency and preventing disease-related complications.

A valuable asset for the treatment of Still’s disease

Learn more about the efficacy and safety of Kineret for the treatment of Still´s disease.

READ MORE ABOUT STILL´S