Synopsis
Recognise the fever, rash, arthritis and systemic pattern of adult-onset Still disease, exclude infection and malignancy, identify macrophage activation syndrome, and escalate cytokine-directed therapy with specialist oversight.
- Adult-onset Still disease is a diagnosis of pattern and exclusion: high spiking fever, evanescent salmon-pink rash, inflammatory arthritis, neutrophilia, sore throat, lymphadenopathy and liver inflammation are typical clues.
- Rash may appear with fever and fade before examination; ask for photographs and examine during a temperature spike without relying on colour description alone across skin tones.
- Marked ferritin supports intense inflammation but is not specific; sepsis, liver injury, malignancy and macrophage activation also raise it.
Key red flags
Persistent fever with falling cell counts, fibrinogen, coagulopathy, hepatic or neurological deterioration and rising ferritin is an immediate inflammatory emergency.
Investigation priorities
Characterise systemic inflammation and detect evolving macrophage activation syndrome.
Management branches
Spiking fever, rash, inflammatory joints and high inflammatory markers suggest Still disease without current shock.
- Obtain repeated cultures, infection and travel testing, full blood and liver assessment and imaging guided by nodes, organs and constitutional features.
- Seek rheumatology and haematology input early and arrange tissue sampling when malignancy or unusual infection remains plausible.
Key medicines
PrednisoloneA common specialist initial range is 0.5–1 mg/kg orally once daily for significant systemic disease, followed by a response-led taper and steroid-sparing plan.
AnakinraAt body weight 50 kg or more, use 100 mg subcutaneously once daily; below 50 kg, start 1–2 mg/kg/day. In severe renal impairment with creatinine clearance below 30 mL/min, consider dosing every other day.