علاج الخط الأول لمرض ستيل ذي البداية في البالغين

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.

View source ↗