Extreme synovial inflammation resulted
in the articular degradation seen in
the hands of this 40-year-old woman
(A, B) who has Still disease, or juvenile
rheumatoid arthritis (JRA). Complete
destruction of the small finger
joints results in “flippers”-unlike the
classic swan-neck deformity of adult
rheumatoid arthritis, where vasculitic
lesions also are present (C).
Still disease results from an autoimmune
reaction to unknown stimuli.
The diagnosis is based on the history,
physical presentation, and meticulous
elimination of other possible
causes (eg, septic or viral arthritis, osteomyelitis,
systemic lupus erythematosus,
or other mixed connective
tissue diseases).
From 5% to 10% of all cases of
JRA are caused by rheumatoid factor–
positive polyarticular variant, and
such patients are predominantly female.
Onset is typically in late childhood,
the arthritis is severe, and the
prognosis is poor.
(Case and photographs A and B courtesy of
Drs Leslie Trope, Eric Capulla, and Avi Trope.
Photograph C courtesy of Dr Navin M. Amin.)
