Adult-onset Still's disease; clinical and laboratory features, treatment
and progress of 45 cases.

Wouters JM, van de Putte LB
Department of Rheumatology, University Hospital, Nijmegen, The
Netherlands.

The clinical and laboratory features, treatment, prognosis,
complications and disability of 45 patients with adult-onset Still's
disease were studied. Sixty per cent of the patients were female. Median
age at onset was 25 years and median observation period after onset was
41 months. For 28 patients detailed data were available of the first
month of illness. In only 43 per cent of these did the disease present
with the classical triad of fever, arthritis and rash, although in the
whole group eventually, fever with temperatures of 40 degrees C or more
occurred in 84 per cent, arthritis in 98 per cent, and the typical rash
in 82 per cent. In 32 per cent of the patients with rash, this was
pruritic. Other features seen frequently were: lymphadenopathy (71 per
cent), splenomegaly (36 per cent), pleuritis and/or pneumonitis (31 per
cent), pericarditis (22 per cent), leucocytosis (98 per cent) and
hepatic abnormalities (84 per cent). The high spiking fever was reduced
to normal in six of 21 cases (29 per cent) by aspirin, in 19 of 27 cases
(70 per cent) by indomethacin and in six of seven cases (86 per cent) by
naproxen. In 16 of 21 cases (76 per cent) glucocorticoids reduced the
systemic and/or joint symptoms. In three patients who remained febrile
on glucocorticoids, indomethacin reduced temperature to normal. Eight
patients whose joint disease improved on steroids later developed severe
joint destructions. Thirteen patients received one or several
slow-acting antirheumatic drugs. Only in eight of 18 trials (44 per
cent) with one of these drugs did symptoms and signs improve. Fifty-one
per cent of the 45 patients had self-limiting disease and 49 per cent
had persistent disease with continuous activity for at least one year.
At the time of evaluation 47 per cent of the 45 patients were in
remission without medication, 33 per cent were in remission while on
medication and 20 per cent had active disease. Three life-threatening
complications occurred: two patients developed signs of cardiac
tamponade and one almost died from diffuse intravascular coagulation.
Disability was determined primarily by the course of the arthritis. At
the time of review 43 per cent of the 45 patients had no joint
destruction (Group 1), 24 per cent had destruction of at least one
joint, but had no evidence of disease of the root joints (Group 2) and
33 per cent had destruction in at least one root joint (Group 3).

PMID: 3659248, UI: 880168-11