Various Manifestations of Rheumatic Disorders: Still Disease

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.)