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Start free with EleplanAdult-onset Still disease
ORPHA:829Disease
Also called AOSD · Wissler-Fanconi syndrome
What it is
A rare inflammatory multisystem disorder characterized clinically by four cardinal signs: fever of unknown origin, arthralgia or arthritis, hyperleucocytosis, and typical skin rash.
Key facts
- Prevalence
- 1-9 / 100 000 (Europe)
- Age of onset
- Adult, Elderly
- Inheritance
- Not applicable
- Classified as
- Disease
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
Signs and symptoms
Very common80–99%
14- ArthralgiaDiagnostic criterion
- Arthralgia/arthritisDiagnostic criterion
- ArthritisDiagnostic criterion
- Elevated circulating C-reactive protein concentration
- Elevated erythrocyte sedimentation rate
- ErythemaDiagnostic criterion
- Fatigue
- FeverDiagnostic criterion
- Increased circulating ferritin concentration
- Increased total leukocyte countDiagnostic criterion
- Joint swelling
- NeutrophiliaDiagnostic criterion
- Pruritus
- Skin rashDiagnostic criterion
Common30–79%
18- Abdominal pain
- Anemia
- Asthenia
- Elevated circulating alkaline phosphatase concentration
- Elevated circulating hepatic transaminase concentrationDiagnostic criterion
- Generalized lymphadenopathy
- HepatomegalyDiagnostic criterion
- Koebner Phenomenon
- LymphadenopathyDiagnostic criterion
- Maculopapular exanthema
- Myalgia
- PharyngalgiaDiagnostic criterion
- PharyngitisDiagnostic criterion
- Pleural effusion
- Pleuritis
- SplenomegalyDiagnostic criterion
- Transient pulmonary infiltrates
- Weight loss
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
ICD-10 codes
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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