Adult-Onset Still Disease
Rheumatology · Other Rheumatologic Diseases 🎯 Practice these cards
Yamaguchi major criteria: fever ≥____ °C with daily spikes, arthralgia/arthritis >____ weeks, salmon-colored rash, and leukocytes >____/µL with ≥____% neutrophils.
The four Yamaguchi MAJOR criteria for AOSD: fever ≥39.0 °C with daily evening spikes; arthralgia/arthritis >2 weeks; nonpruritic salmon-colored macular/maculopapular rash (trunk/extremities); leukocytes >10,000/µL with ≥80% neutrophils.
Yamaguchi minor criteria: ____, lymphadenopathy/splenomegaly, elevated ____, and negative ____.
The four Yamaguchi MINOR criteria: sore throat; lymphadenopathy and/or splenomegaly; elevated AST, ALT, or LDH; negative ANA and rheumatoid factor.
Yamaguchi classification requires ≥____ criteria including ≥____ major, after exclusion of infection, malignancy, and ____.
Classification of AOSD requires ≥5 Yamaguchi criteria including ≥2 major — AND exclusion of infections, malignancies, and other systemic autoimmune diseases (AOSD is a diagnosis of exclusion).
AOSD signature tetrad: quotidian fever, evanescent ____-colored rash, arthritis, and ____.
Classic AOSD presentation: quotidian (daily evening-spiking) fever, evanescent nonpruritic salmon-colored rash peaking with fever, arthritis/arthralgia, and leukocytosis with neutrophilia. Sore throat + extreme hyperferritinemia are characteristic extras.
AOSD: serum ____ is often extremely elevated; a glycosylated fraction <____% supports the diagnosis.
Serum ferritin is often extremely elevated (thousands–tens of thousands) and is a key diagnostic clue; a glycosylated ferritin fraction <20% is relatively specific for Still disease.
AOSD prevalence is ____ cases per million; it reflects overexpression of ____ and is conceptualized as a polygenic autoinflammatory syndrome.
AOSD prevalence is 1–10 cases per million. It reflects overexpression of IL-1β and other cytokines and is conceptualized as a polygenic autoinflammatory syndrome; AOSD and sJIA form one disease continuum (Still disease).
AOSD course: monocyclic, polycyclic, or ____; the most common (~____ of patients) carries the poorest prognosis.
Three course patterns: monocyclic (single self-limited episode), polycyclic (relapsing–remitting), and chronic progressive — the most common (~two-thirds of patients) and the one with the poorest prognosis (destructive arthritis, ankylosis).
MAS in AOSD: unremitting fever, cytopenias, soaring ferritin, hypertriglyceridemia, and a paradoxically ____ ESR (from ____ consumption).
Macrophage activation syndrome in AOSD: unremitting fever, cytopenias, soaring ferritin, hypertriglyceridemia, liver dysfunction, DIC, and a paradoxically FALLING ESR (fibrinogen consumption). Most common rheumatic cause of MAS; emergency.
For systemic AOSD, preferred biologics block ____ (anakinra, canakinumab) or ____ (tocilizumab); ____ inhibitors are inferior for systemic disease.
Preferred biologics for systemic AOSD: IL-1 blockade (anakinra, canakinumab) or IL-6 blockade (tocilizumab). TNF inhibitors are inferior for systemic disease — reserved for persistent arthritis without systemic features. Methotrexate is an adjunct for arthritis-predominant disease.
In June ____, the FDA approved ____ as the first treatment for active Still disease including AOSD (ages ≥____).
In June 2020 the FDA expanded canakinumab (Ilaris) to active Still disease in patients ≥2 years old, including AOSD — the first FDA-approved treatment for AOSD (based on a 36-patient RCT consistent with sJIA data).
In June 2025 the FDA approved ____, an anti–____ antibody, as the first treatment for MAS in Still disease.
In June 2025 the FDA approved emapalumab-lzsg (Gamifant), an anti–interferon-γ antibody, for HLH/MAS in known or suspected Still disease (glucocorticoid failure/intolerance or recurrent MAS) — first approved MAS therapy; 54% complete response, 82% remission at week 8 (EMERALD/NI-0501-06).
The 2024 EULAR/PReS task force unified sJIA and AOSD under one name — ____ — and set the treatment target as clinically inactive disease/remission maintained ≥____ months.
The 2024 EULAR/PReS task force (Fautrel et al., Ann Rheum Dis 2024;83:1614-1627) unified sJIA and AOSD as one disease — “Still disease” — with 14 recommendations; treatment target = clinically inactive disease/remission maintained ≥6 months via early IL-1/IL-6 blockade + short glucocorticoid course.
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