Synonyms
• Wissler-Fanconi syndrome,
subsepsis hyperallergica
Definitions
• 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)
Location
○ Hand
– Interphalangeal (IP) joints
more commonly affected than metacarpophalangeal (MCP) joints
□ Proximal interphalangeal
(PIP) joint (50%)
□ Distal interphalangeal (DIP)
joint (20%)
□ MCP joint (33%)
○ Wrist
– 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
Etiology
○ Unknown; adult continuum of
JIA
– Same manifestations and
clinical course
○ Prodromal sore throat common
(70%)
○ Associated with variety of
viral infections
CLINICAL
ISSUES
Presentation
• Most common signs/symptoms
○ Triad of daily spiking
fever, evanescent rash, and chronic polyarthritis (usually hands, feet)
• Other signs/symptoms
○ Myalgia
○ Weight loss
○ Lymphadenopathy
○ Hepatosplenomegaly
○ Pleuritis, pericarditis
○ May have abrupt onset
myocardial injury
• No specific diagnostic test
○ Seronegative for rheumatoid
factor and ANA
Demographics
• Age
○ 75% have onset between 16
and 35 years
– 10% onset after 50 years of
age
• Gender
○ M = F
• Ethnicity
○ No predilection for race or
ethnic origin
• Epidemiology
○ Rare (0.16/100,000)
Natural
History & Prognosis
• If chronic, 50% develop
carpal ankylosis
• Intermittent flares
throughout life
• Systemic manifestations do
not ↑ mortality
Treatment
• Arthritis managed with same
drug therapy as RA
• Steroids for severe systemic
manifestations
DIAGNOSTIC
CHECKLIST
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.