Various Manifestations of Rheumatic Disorders: Still Disease

Extreme synovial inflammation resultedin the articular degradation seen inthe hands of this 40-year-old woman(A, B) who has Still disease, or juvenilerheumatoid arthritis (JRA). Completedestruction of the small fingerjoints results in "flippers"-unlike theclassic swan-neck deformity of adultrheumatoid arthritis, where vasculiticlesions also are present (C).Still disease results from an autoimmunereaction to unknown stimuli.The diagnosis is based on the history,physical presentation, and meticulouselimination of other possiblecauses (eg, septic or viral arthritis, osteomyelitis,systemic lupus erythematosus,or other mixed connectivetissue diseases).From 5% to 10% of all cases ofJRA are caused by rheumatoid factor-positive polyarticular variant, andsuch patients are predominantly female.Onset is typically in late childhood,the arthritis is severe, and theprognosis is poor.

(Case and photographs A and B courtesy ofDrs Leslie Trope, Eric Capulla, and Avi Trope.Photograph C courtesy of Dr Navin M. Amin.)