Home of Radiologists


Recent Topics

جاري التحميل ...

Adult Still Disease



Wissler-Fanconi syndrome, subsepsis hyperallergica



Systemic inflammatory disease, featuring triad

Quotidian fevers (spiking, daily)

Evanescent rash

Chronic polyarthritis

Considered adult continuum of juvenile idiopathic arthritis (JIA)


Best diagnostic clue

Diagnosis of exclusion

25% develop destructive polyarthritis, behaving like rheumatoid arthritis (RA)




Interphalangeal (IP) joints more commonly affected than metacarpophalangeal (MCP) joints

Proximal interphalangeal (PIP) joint (50%)

Distal interphalangeal (DIP) joint (20%)

MCP joint (33%)



Involved in 74% of cases

Pericapitate (midcarpal and carpometacarpal) disease is classic, but any pattern of carpal involvement may be seen


Foot: Midtarsal and tarsal-metatarsal joints


Knee: 84% involvement, at least with arthralgias


Radiographic Findings

Uniform cartilage loss

Erosions similar to RA

Carpus may be remarkable for joint space narrowing, without significant erosive change

Ankylosis, especially at carpus

Distribution of involved joints differentiates from RA

No periostitis; may differentiate from psoriatic




Differential Diagnosis

Psoriatic Arthritis

IP > MCP joint involvement is similar

Ankylosis is similar

Rash and constitutional symptoms differentiate adult Still disease from psoriatic arthritis


Chronic Reactive Arthritis

Constitutional symptoms may be suggestive

Location of arthritic changes is likely different in adult Still, with hand/wrist predominating

Foot/ankle may be involved, similar to chronic reactive arthritis


Inflammatory Bowel Disease

Constitutional symptoms may be suggestive

Distribution of destructive arthralgias usually different; axial and proximal joints predominate in inflammatory bowel disease




Unknown; adult continuum of JIA

Same manifestations and clinical course

Prodromal sore throat common (70%)

Associated with variety of viral infections




Most common signs/symptoms

Triad of daily spiking fever, evanescent rash, and chronic polyarthritis (usually hands, feet)


Other signs/symptoms


Weight loss



Pleuritis, pericarditis

May have abrupt onset myocardial injury


No specific diagnostic test

Seronegative for rheumatoid factor and ANA




75% have onset between 16 and 35 years

10% onset after 50 years of age



M = F



No predilection for race or ethnic origin



Rare (0.16/100,000)


Natural History & Prognosis

If chronic, 50% develop carpal ankylosis

Intermittent flares throughout life

Systemic manifestations do not mortality



Arthritis managed with same drug therapy as RA

Steroids for severe systemic manifestations



Image Interpretation Pearls

Pericapitate predominance in carpal disease and IP predominance in hand disease, with ankylosis, is highly suggestive

Primary differential with these findings is psoriatic arthritis; constitutional symptoms differentiates


PA radiograph of the fingers in a patient with constitutional symptoms suggests adult Still disease. There is joint space narrowing and erosive change of a distal interphalangeal (IP) joint. The other IP joints showed narrowing but no erosions. The MCP joints were normal.


PA radiograph of the carpus in the same patient is shown. While there is diffuse joint space narrowing, it is most prominent in  pericapitate distribution . No erosions are seen, but there may be ankylosis. This is classic adult Still disease.



 PA radiograph of the hand in a patient with rash and daily fevers is shown. There are significant IP joint erosions and ankylosis of a single distal interphalangeal joint . The appearance may suggest psoriatic arthritis, but the constitutional symptoms help make the diagnosis of adult Still disease.


 PA radiograph of the carpus in the same patient is shown. There is diffuse cartilage loss and erosive change. This is a nonspecific appearance and does not serve to differentiate psoriatic from adult Still disease.


عن الكاتب


اتصل بنا

جميع الحقوق محفوظة