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Q1: Presentation & diagnosis (2 min)
A 26-year-old presents with three weeks of daily spiking evening fevers, a salmon-pink rash that comes and goes with the fevers, wrist and knee arthralgia, and a sore throat. Take us through your approach. Why is this a diagnosis of exclusion? Cover: the classic tetrad (quotidian spiking fever, evanescent salmon-pink rash, arthritis, sore throat) in a young adult with very high ferritin, neutrophilic leucocytosis and negative ANA/RF; the principle of first excluding infection, malignancy (lymphoma) and other rheumatic disease; the Yamaguchi and Fautrel clinical criteria; and the supportive role of a glycosylated ferritin under 20 percent.
Q2: Pathophysiology (2 min)
What is the underlying mechanism of adult Still's disease, and why does it matter for treatment? Cover: an autoinflammatory disorder of innate immunity driven by IL-1, IL-6 and IL-18 from activated macrophages/neutrophils — explaining the negative autoantibodies, the very high ferritin, and the dramatic response to cytokine-blocking biologics. Distinguish this from an autoimmune (adaptive-antibody) disease. Explain why IL-18 links AOSD to macrophage activation syndrome.
Q3: Management (3 min)
How do you treat adult Still's disease step by step? Cover: NSAIDs + glucocorticoids (prednisolone 0.5 to 1 mg/kg; IV methylprednisolone pulses for severe disease) first-line; methotrexate to spare steroids; IL-1 inhibitors (anakinra, canakinumab) as the preferred first-line biologic for refractory systemic disease; tocilizumab (IL-6) for the chronic articular pattern; emerging IL-18 and JAK inhibitors. State the treat-to-target principle (remission, then taper) and pre-biologic screening (TB, hepatitis B and C, HIV).
Q4: Macrophage activation syndrome (2 min)
What is the feared complication, and how do you recognise and treat it? Cover: macrophage activation syndrome / secondary HLH complicating about 10 to 15 percent; recognised by persistent fever with falling platelets and white cells, falling fibrinogen, and rising ferritin, LDH, triglycerides and transaminases; a haemophagocytic marrow is supportive but not required. Treat as an emergency with high-dose IV methylprednisolone pulses plus anakinra, supportive care, and ICU monitoring.
Q5: Patterns and prognosis (1 min)
What are the clinical patterns and what determines outcome? Cover: monocyclic (self-limited, best prognosis), polycyclic (relapsing-remitting) and chronic articular (joint destruction, greatest morbidity). Predictors of the chronic articular pattern include polyarthritis and shoulder/hip involvement at onset. Mortality is driven mainly by MAS, severe infection and hepatic failure.