Factors and glucocorticoid usage affecting the prognosis of systemic JIA.

Background The rate of glucocorticoid (GC) usage is significantly higher in systemic juvenile idiopathic arthritis (SJIA) than other JIA subtypes. There is no consensus on the duration and dosage of GC treatment. We aimed to investigate the risk factors of polyphasic/persistent disease course and the effect of dose and duration of GC treatment on SJIA prognosis. Methods Forty-two patients diagnosed with SJIA and the duration of disease was longer than two years included. Patients were divided as monophasic and others (polyphasic/persistent disease course). Risk factors of polyphasic/persistent disease course, which were clinical and laboratory findings of the patients, treatment options, dose, and duration of GCs were evaluated for the first active disease periods and for all flares in the entire disease course. Results Of the 42 SJIA patients, 21 had monophasic, and 21 had polyphasic/persistent disease. Cumulative dosages and durations of glucocorticoid treatment were similar in two groups at first flare(OR:1,032p:0,671)(OR:1,113p:0,115).Durations of the first active disease period were longer in polyphasic/persistent group(OR:1,275,p:0,01). Active disease duration cut-off value of 1.5 months with sensitivity 85.7%, specificity 52.4% were observed on ROC curve analysis. Presence of hepatosplenomegaly at first flare was detected as an independent risk factor of polyphasic/persistent disease both dosage and duration of steroid included multivariate analysis (HR:4,129, p:0,034), (HR:3,992, p:0,038). Multivariate recurrent events survival analysis determined ALT levels as risk factor affecting polyphasic/persistent disease (HR:0,986, p:0,037). Conclusion Glucocorticoid dose and duration did not affect the active disease periods and disease course in SJIA. An active disease period that longer than 1.5 months, presentation of hepatosplenomegaly at initial disease course, and high ALT levels at the recurrences should warn physicians of polyphasic/persistent disease.