Adult-Onset Still Disease

Question · easy

A 27-year-old woman has 3 weeks of daily evening fevers to 39.8 °C, an evanescent salmon-colored truncal rash that appears with the fever, polyarthralgia, and severe sore throat. Labs: leukocytes 16,500/µL (86% neutrophils), mildly elevated AST/ALT, ferritin 9,400 ng/mL, negative ANA and rheumatoid factor, negative blood cultures. What is the most likely diagnosis?

Reactive arthritis Systemic lupus erythematosus Acute rheumatic fever Adult-onset Still disease Infective endocarditis

Question · medium

According to the Yamaguchi criteria, which of the following is required to classify adult-onset Still disease?

Positive ANA plus quotidian fever Four minor criteria plus biopsy-proven vasculitis At least 5 criteria including at least 2 major, after exclusion of mimics At least 3 major criteria regardless of exclusions Serum ferritin >10,000 ng/mL

Question · medium

A patient with known adult-onset Still disease on glucocorticoids develops persistent non-spiking fever, platelets falling from 380,000 to 90,000/µL, ferritin rising from 4,000 to 48,000 ng/mL, ESR falling from 90 to 12 mm/h, elevated triglycerides, and worsening transaminases. What is the most likely complication?

Secondary (AA) amyloidosis Septic arthritis Macrophage activation syndrome Progression to Hodgkin lymphoma Drug-induced lupus

Question · hard

A 34-year-old man with adult-onset Still disease has persistent systemic disease (daily fevers, pleuritis, ferritin 15,000 ng/mL) despite prednisone 60 mg/day, and the team wishes to taper glucocorticoids. Which is the best next step?

Add an IL-1 inhibitor (anakinra or canakinumab) Continue prednisone alone and reassess in 3 months Add azathioprine Add hydroxychloroquine Add infliximab

Question · hard

Which statement about the course and prognosis of adult-onset Still disease is correct?

The disease is non-erosive and never causes joint destruction Mortality is essentially zero Nearly all patients have a single self-limited episode with full recovery A chronic progressive course is the most common pattern (~two-thirds) and is associated with destructive arthritis and ankylosis TNF inhibitors are the preferred biologic for chronic systemic disease