Ann Rheum Dis 1990 May;49(5):283-5

Comparison of long term evolution of adult onset and juvenile onset
Still's disease, both followed up for more than 10 years.

Cabane J, Michon A, Ziza JM, Bourgeois P, Bletry O, Godeau P, Kahn MF

Department of Internal Medicine, Pitie-Salpetriere Hospital, Paris,
France.

Still's disease is a clinical entity of unknown origin, which can appear
before 15 years of age (juvenile onset Still's disease) or later (adult
onset Still's disease). There are few reported data about the long term
prognosis of Still's disease and no study compares the long term
evolution of adult onset and juvenile onset Still's disease. Eighteen
patients fulfilling the American Rheumatism Association criteria for
Still's disease were followed up for more than 10 years. Ten (group 1)
had juvenile onset Still's disease and eight (group 2) adult onset
Still's disease. A comparison of the groups showed no significant
differences in the initial systemic manifestations of Still's disease,
or in the joint lesions. Both groups had severe sequelae, which appeared
between six and 10 years after the initial flare up of Still's disease.
Nine patients had articular damage and nine had only arthritis without
apparent x ray abnormalities. Nine patients had bilateral hip
destruction in less than four years. Of these nine, seven required 13
total hip replacements before the age of 45. In the whole group of 18
patients bilateral involvement of the following joints was also seen:
carpus (seven patients), knee (four), tarsus (four), ankle (three);
three patients had ankylosis of the cervical spine. The occurrence of
amyloidosis (three cases, two deaths) was restricted to group 2. This
was the only difference between the groups, as the treatments were
identical. It is concluded that the articular prognosis of Still's
disease is poor, be it adult onset or juvenile onset, with severe joint
destruction in half of the patients.

PMID: 2344206, UI: 90262276