OP0168 DERIVATION AND VALIDATION OF A NEW STILL ACTIVITY SCORE (SAS)
Authors: Umut Kalyoncu, Timuçin Kaşifoğlu, Ahmet Omma, Cemal Bes, Muhammet Çınar, Hakan Emmungil, Orhan Küçükşahin, Servet Akar, Kenan Aksu, Fatih Yıldız, Nilüfer Alpay Kanıtez, Abdulsamet Erden, Emre Bilgin, Sezin Turan, Ediz Dalkılıç, Selime Ermurat, Kadir Mutlu Hayran
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background</h3> Adult-onset Still's disease (AOSD) is a rare, chronic, multisystem and auto-inflammatory disorder. Although several classification criteria exist for the diagnosis of AOSD, there is no valid method for the assessment of disease activity for these patients. <h3>Objectives</h3> To determine a simple method for the assessment of AOSD activity. <h3>Methods</h3> We conducted a cross-sectional, multicenter study for assessment of disease activity in AOSD patients. All AOSD patients were fulfilled the Yamaguchi criteria. Age, sex, disease duration and current symptoms were recorded. Erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), leukocyte count, and ferritin levels were also recorded. Visual analog scale (VAS) (0–10 cm) and likert scale (as remission, low, moderate, severe, and more severe disease activity) were used for physician's (PhGA) and patient's (PtGA) global assessment of disease activity. Patients were enrolled to "derivation cohort" (n=125) and subsequently "validation cohort" (n=72). Ordinal logistic regression model was used to derive Still Activity score (SAS). <h3>Results</h3> Overall, 197 (66.5% female) AOSD patients were enrolled. Mean age was 39 (13) years and median disease duration was 2 (0–30) years. Cross-sectional frequency of AOSD findings were as follow; fever 69 (35%), rash 55 (27.9%), arthritis 57 (28.9%), arthralgia 112 (56.9%), sore throat 58 (29.4), myalgia 80 (40.6), lymphadenopathy 25 (12.7%), splenomegaly 37 (18.8%), hepatomegaly 23 (11.7%), abdominal pain 8 (4.1%), pleuritic pain 8 (4.1%), pericarditis 4 (2%), hemophagocytic syndrome 4 (2%). Mean (SD) ESR (mm/h), CRP (mg/dl), ferritin and leukocyte count were 32.5 (31), 43.4 (65.3), 1169 (3088) and 10356 (4821), respectively. Mean PtGA and PhGA was 3.86 (3.29), and 3.05 (3.06). SAS derived for the assessment of AOSD disease activity is given in Table 1. SAS were correlated with the PtGA of disease activity as 83% for the derivation cohort and 75% for the validation cohort, also, correlation of this score and patient's global VAS assessments were 79% and 73%, respectively. Sensitivity and specificity of SAS equal and/or higher than 4 to predict " active (severe) and highly active (more severe) AOSD activity" are 100% and 82.6%, respectively (Figure 1). <h3>Conclusion</h3> In this study, we created a Still activity score (SAS) with the routine clinical and laboratory features of patients. This score can detect patients who have high likelihood of being in active disease status. In the future perspective, SAS should be assessed in the clinical trials and routine practice. <h3>Disclosure of Interests</h3> Umut Kalyoncu Grant/research support from: MSD, Roche, UCB, Novartis and Pfizer, Consultant for: MSD, Abbvie, Roche, UCB, Novartis, Pfizer and Abdi Ibrahim, Speakers bureau: MSD, Abbvie, Roche, UCB, Novartis, Pfizer and Abdi Ibrahim, Timuçin Kaşifoğlu: None declared, Ahmet Omma: None declared, Cemal Bes: None declared, Muhammet Çınar: None declared, Hakan Emmungil Grant/research support from: MSD, Roche, Pfizer, Abbvie, Consultant for: Novartis, Roche, Speakers bureau: MSD, Roche, Pfizer, Abbvie,Celltrion, Novartis, Orhan Küçükşahin: None declared, Servet Akar Grant/research support from: MSD, Abbvie, Roche, UCB, Novartis, Pfizer, Amgen, Consultant for: MSD, Abbvie, Roche, UCB, Novartis, Pfizer, Amgen, Speakers bureau: Pfizer, Kenan Aksu: None declared, Fatih Yıldız: None declared, Nilüfer Alpay Kanıtez: None declared, Abdulsamet Erden: None declared, Emre Bilgin: None declared, Sezin Turan: None declared, Ediz Dalkılıç Grant/research support from: MSD and Abbvie, Consultant for: MSD, Abbvie,Roche, UCB, Pfizer and Novartis, Speakers bureau: MSD, Abbvie,Roche, UCB, Pfizer and Novartis, Selime Ermurat: None declared, Kadir Mutlu Hayran: None declared