To date, the treatment of
adult-onset Still's disease (AOSD) has been largely empirical; therefore, this study was conducted to investigate the response to
therapy and prognostic factors of AOSD. Fifty-four Korean patients with AOSD were enrolled based on Yamaguchi's criteria. We retrospectively analyzed the treatments and prognosis. Thirty-nine patients (72.2%) were female, and the average age at disease onset was 37.3 years. Twenty-nine patients had a monocyclic disease (53.7%), five had a polycyclic (9.3%) and fifteen had a chronic articular disease (27.7%) and five died (9.3%). The elevated ESR and
corticosteroids refractoriness were associated with poor prognosis (P = 0.023 and P = 0.009, respectively). The patients that died were older than those survived (49.2 ± 11.8 vs. 42.2 ± 14 year old, P = 0.024). Forty-two patients were treated with non-steroidal anti-inflammatory drugs; however, they also needed
corticosteroids and
intravenous immunoglobulin (
IVIG). Among 50 patients treated with high-dose
corticosteroids, 21 patients (42%) were resistant to
corticosteroids and treated with
IVIG or anti-
tumor necrosis factor (TNF) agents. Of the 23 patients medicated with
IVIG, the prognosis was better in
IVIG-responsive patients, indicating a
therapeutic effect.
Methotrexate was the most commonly used disease modifying
anti-rheumatic drugs (27 patients, 50%), and the
corticosteroid requirements were lower in the
methotrexate-responsive patients. Approximately half of AOSD patients had a poor prognosis and were
corticosteroids resistance. An elevated ESR and non-response to
corticosteroids were associated with poor prognosis. Patients who died were older than those survived.