Can Patient-Reported Outcomes and Disease Activity Scores Predict Patient Acceptable Symptom State in Adult-Onset Still's Disease?

Emre Bilgin ¹, Timuçin Kaşifoğlu ², Ahmet Omma ³, Cemal Bes ⁴, Muhammet Çınar ⁵, Hakan Emmungil ⁶, Orhan Küçükşahin ⁷, Servet Akar ⁸, Kenan Aksu ⁹, Fatih Yıldız ¹⁰, Nilüfer Kanıtez ¹¹, Abdulsamet Erden ¹², Sezin Turan ¹³, Ediz Dalkılıç ¹⁴, Selime Ermurat ¹⁵, Mutlu Hayran ¹⁶ and Umut Kalyoncu¹⁷, ¹Hacettepe University Vasculitis Centre, Ankara, Turkey, Ankara, Turkey, ²Eskişehir Osmangazi University, Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Eskişehir, Turkey, ³Ankara Numune Education and Research Hospital, Department of Internal Medicine, Division of Rheumatology, Ankara, Turkey, ⁴University of Medical Sciences, İstanbul, Turkey, ⁵University of Medical Sciences, Ankara, Turkey, ⁶Trakya University, Edirne, Turkey, ⁷Liv Hospital, Ankara, Turkey, ⁸Izmir Katip Celebi University, Faculty of Medicine, Division of Rheumatology, İzmir, Turkey, ⁹Ege University, Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, İzmir, Turkey, ¹⁰Kahramanmaraş Sütçü İmam University, Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Kahramanmaraş, Turkey, ¹¹Koç University, Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, İstanbul, Turkey, ¹²Ministry of Health Ankara City Hospital, Department of Internal Medicine, Division of Rheumatology, Ankara, Turkey, ¹³Trakya University, Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Edirne, Turkey, ¹⁴Uludağ University, Bursa, Turkey, ¹⁵Uludağ University, Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Bursa, Turkey, ¹⁶Hacettepe University, Faculty of Medicine, Department of Preventive Oncology, Ankara, Turkey, ¹⁷Hacettepe University Department of Rheumatology, Ankara, Turkey

Meeting: 2019 ACR/ARP Annual Meeting

Keywords: Adult-onset Still's disease, PASS and Disease Activity, patient-reported outcome measures

Session Information

Date: Monday, November 11, 2019

Title: Miscellaneous Rheumatic & Inflammatory Disease Poster II: Autoinflammation Related Diseases & Therapies

Session Type: Poster Session (Monday)

Session Time: 9:00AM-11:00AM

Background/Purpose: Adult-onset Still’s disease (AOSD) is a rare, chronic and auto-inflammatory disorder with multisystemic involvement. Patient Acceptable Symptom State (PASS) is the highest level of disease activity that patients feel quite well. Till now, there is no valid information for the possible cut-off values of patient-reported outcomes (PRO) or activity scores for AOSD patients to predict PASS. To determine cut-off values of PROs and activity scores to predict PASS in AOSD patients, we conducted this study.

Methods: We conducted a cross-sectional, multicenter study. All AOSD patients were fulfilled the Yamaguchi criteria. Still activity score (SAS) [1 point for neutrophilia ≥65%, 1 point for ferritin≥350 ng/ml, 2 point for fever and 2 point for arthralgia-additional 1 point if swollen joint number is equal or grater than 2], Pouchet’s systemic score (SS) [1 point for each of the following manifestation: fever, typical rash, pleuritis, pneumonia, pericarditis, hepatomegaly, or abnormal liver function tests, splenomegaly, lymphadenopathy, leukocytosis >15,000/mm2, sore throat, myalgia, and abdominal pain], visual analog scale (VAS) (0–10 cm) for physician’s (PhGA) and patient’s (PtGA) global assessment were used to determination of the disease activity. To assess PASS, we asked patients whether they would be okay or not, if the disease activity will be the same as now during the next month. Receiver operator curve (ROC) analysis was done to find cut-off values.

Results: Total of 72 (71% female) AOSD patients were enrolled. Mean age was 41 (13) years and median disease duration was 2 (0–30) years. Median SS and SAS were 3 (0-10) and 4 (0-7), respectively. Mean PtGA and PhGA were 4.29 (3.15) and 3.54 (3.07), respectively. Cross-sectional frequency of AOSD findings were as follow; fever 34 (47%), rash 27 (37.5%), arthritis 25 (34.7%), arthralgia 52 (72.2%), sore throat 30 (41.7), myalgia 38 (52.8), lymphadenopathy 13 (18.1%), splenomegaly 20 (27.8%), hepatomegaly 16 (22.2%), pleuritic pain 5 (6.9%), pericarditis 4 (5.6%), hemophagocytic syndrome 2 (2.8%). Mean (SD) ESR (mm/h), CRP (mg/dl), ferritin level and leukocyte count were 36.8 (34), 5.4 (7.3), 1758 (4070) and 9960 (4178), respectively. ROC analysis, area under curve, confidence intervals, sensitivity and specificity for different cut-off values were given in Table 1 and Figure 1.

Conclusion: In this study, all PROs and activity scores performed well to predict PASS. Still activity score seems to perform better over SS. To the best of our knowledge, this is the best study reporting cut-off values for PROs and activity scores to predict PASS.

PASS-Table1

Table 1. Sensitivity, specificity, AUC for several cut-off values of PROs and disease activity scores

PASS-figure1

Figure 1. ROC analysis for PROs and disease activity scores

Disclosure: E. Bilgin, None; T. Kaşifoğlu, None; A. Omma, None; C. Bes, None; M. Çınar, None; H. Emmungil, None; O. Küçükşahin, None; S. Akar, Abbvie, 2, 5, Amgen, 2, 5, MSD, 2, 5, Novartis, 2, 5, Pfizer, 2, 5, 8, Roche, 2, 5, UCB, 2, 5; K. Aksu, None; F. Yıldız, None; N. Kanıtez, None; A. Erden, None; S. Turan, None; E. Dalkılıç, None; S. Ermurat, None; M. Hayran, None; U. Kalyoncu, UCB, 5.

To cite this abstract in AMA style:

Bilgin E, Kaşifoğlu T, Omma A, Bes C, Çınar M, Emmungil H, Küçükşahin O, Akar S, Aksu K, Yıldız F, Kanıtez N, Erden A, Turan S, Dalkılıç E, Ermurat S, Hayran M, Kalyoncu U. Can Patient-Reported Outcomes and Disease Activity Scores Predict Patient Acceptable Symptom State in Adult-Onset Still’s Disease? [abstract]. Arthritis Rheumatol. 2019; 71 (suppl 10). https://acrabstracts.org/abstract/can-patient-reported-outcomes-and-disease-activity-scores-predict-patient-acceptable-symptom-state-in-adult-onset-stills-disease/. Accessed .

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