AB0934 Clinical Features and Prognosis of Adult-Onset Still's Disease: S Single Center Study in Japan

Background Adult-onset Still's disease (AOSD) is an inflammatory diseases characterized by spiking fever, arthritis and evanescent rash. Although AOSD is considered as a benign disease, relapses are common and life-threatening severe complications occur in some patients. Objectives In this study, we reviewed the clinical features and outcomes of AOSD patients to seek out risk factors for unfavorable outcomes. Methods We retrospectively reviewed medical charts of AOSD patients who were admitted to our department between 1997 and 2013. For the statistical analyses, Mann Whitney U test and Fisher's exact test were carried out. Multivariate logistic analysis was used to identify factors independently associated with death. It was considered statistically significant if p value was less than 0.05. Results A total of 27 patients (8 male and 19 female) were available to analysis. The median age was 33 years (range, 15-64) and the median follow-up period was 2.2 years (range, 0.1-9.1). Corticosteroids and immunosuppressants were used in 92.6% and 33.3% of the patients, respectively. Methylprednisolone pulse therapy was applied in 18.5% of the patients and the median initial dose of steroids was 50 mg/day (range, 30-70; equivalent dose of prednisolone, excluding the dose of pulse therapy). All patients responded to the initial therapy, including 2 patients treated with nonsteroidal anti-inflammatory drugs (NSAIDs) alone. Twelve patients (44.4%) continued remission, whereas 15 patients (55.6%) had at least one relapse (median 2 times; range, 1-6). Five patients (18.5%) died from multiple organ failure in 3, sepsis in 1 and respiratory failure in 1 patient. The comparison of baseline characteristics between dead and alive patients was shown in Table. The complications of hemophagocytic syndrome (HPS) and disseminated intravascular coagulation (DIC) were significantly more frequent, and the hemoglobin level was significantly lower in dead patients than patients who survived. Multivariate logistic analysis showed a presence of DIC was an independent risk factor for death (OR 20.0, 95%CI 1.613-247.981, p=0.020). Table 1. Comparison of baseline characteristics between dead and alive patients Dead patients (n=5) Alive patients (n=22) P value Age (year) 46.0 (31.0–64.0) 29.0 (15.0–63.0) 0.1112 Time to diagnosis (days) 77.0 (0–498.0) 29.0 (0–120.0) 0.0802 HPS (patients) 3 (60.0%) 1 (4.5%) 0.0008 DIC (patients) 4 (80.0%) 4 (18.2%) 0.0048 Serum ferritin (ng/ml) 3889 (1039.9–8