Utility of the EULAR Sjögren syndrome disease activity index in Japanese children: a retrospective multicenter cohort study

Pediatric Rheumatology

Naomi Iwata¹  Yasunori Sato²  Ichiro Kobayashi³  Yusaburo Inoue⁴  Hiroaki Umebayashi⁵  Masaaki Mori⁶  Yukiko Nonaka⁷  Yasuhiko Ito⁸  Nami Okamoto⁹  Minako Tomiita^1,10  Ryoki Hara^1,11 

[1] Department of Infection and Immunology, Aichi Children’s Health and Medical Center, Obu, Japan;The Japan Pediatric Sjögren’s Syndrome Study Group, Tokyo, Japan;Department of Preventive Medicine and Public Health, Keio University School of Medicine, Tokyo, Japan;The Japan Pediatric Sjögren’s Syndrome Study Group, Tokyo, Japan;Center for Pediatric Allergy and Rheumatology, KKR Sapporo Medical Center, Sapporo, Japan;The Japan Pediatric Sjögren’s Syndrome Study Group, Tokyo, Japan;Department of Allergy and Rheumatology, Chiba Children’s Hospital, Chiba, Japan;The Japan Pediatric Sjögren’s Syndrome Study Group, Tokyo, Japan;Department of General Pediatrics, Miyagi Children’s Hospital, Sendai, Japan;The Japan Pediatric Sjögren’s Syndrome Study Group, Tokyo, Japan;Department of Lifetime Clinical Immunology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan;The Japan Pediatric Sjögren’s Syndrome Study Group, Tokyo, Japan;Department of Pediatrics, Kagoshima University Hospital, Kagoshima, Japan;The Japan Pediatric Sjögren’s Syndrome Study Group, Tokyo, Japan;Department of Pediatrics, Nippon Medical School, Tokyo, Japan;The Japan Pediatric Sjögren’s Syndrome Study Group, Tokyo, Japan;Department of Pediatrics, Osaka Medical College, Osaka, Japan;The Japan Pediatric Sjögren’s Syndrome Study Group, Tokyo, Japan;Department of Pediatrics, Shimoshizu National Hospital, Yotsukaido, Japan;The Japan Pediatric Sjögren’s Syndrome Study Group, Tokyo, Japan;Department of Pediatrics, Yokohama City University School of Medicine, Yokohama, Japan;

关键词: Primary Sjögren’s syndrome;    Children;    ESSDAI;    Treatment;   

DOI  :  10.1186/s12969-020-00458-1

来源: Springer

【 摘 要 】

BackgroundThe European League Against Rheumatism (EULAR) Sjögren Syndrome Disease Activity Index (ESSDAI) has been utilized to assess Sjögren syndrome-related systemic involvement in adult patients. To date, however, the ESSDAI has not been validated in children with primary Sjögren’s syndrome. This study evaluated the applicability of the ESSDAI to Japanese children with primary Sjögren’s syndrome.MethodsThe medical records of children who had been diagnosed with Sjogren syndrome at age ≤ 16 years between June 2011 and October 2016 were collected, and their ESSDAIs at initial presentation were calculated. Clinical symptoms and treatment regimens were surveyed by questionnaire, and patients were divided into groups based on ESSDAI and glucocorticoid dosages. The associations of ESSDAI scores with treatment regimens were analyzed statistically.ResultsThe study subjects included 31 children (3 boys, 28 girls) with primary Sjögren’s syndrome. Their median age at disease onset was 10 years (interquartile range [IQR], 8–13 years), and their median initial ESSDAI was 7.0 (IQR; 5.0–15.0). ESSDAI-determined disease activity was high in nine patients (29.0%), moderate in 15 (48.4%), and low in seven (22.6%). During the first year after their initial visit, 14 patients (45.2%) were treated with prednisolone (PSL) and six (19.4%) with immunosuppressants. Dose of PSL was significantly associated with ESSDAI score. Median ESSDAI score was significantly higher in patients treated with high/medium- than with no/low-dose PSL (16.5 [IQR 10.5–18.0] vs 5.0 [IQR 3.0–8.5]). Eight (66.7%) of 12 patients administered medium/high-dose PSL and one (5.3%) of 19 administered no/low-dose PSL had high disease activity on ESSDAI.ConclusionDisease activity assessed by ESSDAI tended to be consistent with disease activity assessed by pediatric rheumatologists in determining treatment regimens. ESSDAI is useful for assessing disease activity in Japanese children with primary Sjögren’s syndrome.

【 授权许可】

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