First-Line Treatment of Adult-onset Still Disease
Adult-onset Still disease requires prompt initiation of therapy once the diagnosis is established.
Presentations range from milder arthritis and fever to high disease activity with widespread polyarthritis,
pericarditis, and risk of macrophage activation syndrome — and the treatment strategy is calibrated accordingly.
Treatment Approach
Evidence-based guidance supports initiating biologic therapy — an IL-1 inhibitor or an IL-6 inhibitor —
as early as possible, irrespective of disease severity. In patients with high disease activity,
glucocorticoids may also be part of the regimen. The complete protocol details which agent is preferred
in which clinical circumstances, how therapies are combined, and how glucocorticoids are tapered over time —
all of which remain in the full regimen.
Clinical Treatment Targets
- Day 7 — resolution of fever and >50% reduction in CRP
- Week 4 — no fever, >50% reduction in active joint count, normal CRP, physician and patient global assessment <20/100
- Month 3 — clinically inactive disease with glucocorticoids <0.1 mg/kg/day (adults) or <0.2 mg/kg/day (children)
- Month 6 — clinically inactive disease off glucocorticoids
References
DOI: 10.1136/ard-2024-225851
- At onset or during a flare a patient should receive an IL-1 or IL-6 inhibitor as early as possible.
- Since there is no clear predictor at disease onset to identify patients who will develop a chronic disease course, the TF recommends considering first-line biologic therapy irrespective of disease severity.
- High-dose GCs are indicated in patients with high disease severity (high spiking fever, wide-spread polyarthritis, high levels of pain (VAS >6-7/10), pericarditis, impending MAS (elevated LFT and/or high serum ferritin levels).
- In milder presentations, GC may be used at low or intermediate doses, but are not mandatory.
- When they are started, GC should be progressively tapered as soon as possible with the aim of achieving CID on low dose GC at 3 months and, subsequently, CID off GC at 6 months from treatment initiation.
- At day 7, resolution of fever and reduction of CRP by >50%.
- At week 4, no fever, reduction of active (or swollen) joint count by >50%, normal CRP and physician and patient/parent global assessment less than 20 on a 0-100 VAS.
- At month 3, CID with GCs less than 0.1 (adults) or 0.2 (children) mg/kg/day.
- At month 6, CID without GCs.
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