Trends in actual medication use for child-onset systemic lupus erythematosus using the Japanese health insurance database 2009-18.

OBJECTIVES: Immunosuppressive therapy is the mainstay of treatment for child-onset systemic lupus erythematosus (cSLE). Since epidemiological data on Japanese cSLE patients are not available, we evaluated the trends in how treatment choices have changed over time in Japan. METHODS: Using the Japanese health insurance database provided by Medical Data Vision Co., Ltd, we identified cSLE patients and evaluated changes in the use of corticosteroids and immunosuppressive medications and maximum daily doses of prednisolone from 2009 to 2018. RESULTS: Of 182 cSLE patients, 86% were female, and the median age was 14 years. Oral prednisolone was used in more than 97% of cSLE patients during the study period, and the median of the maximum daily dose in each patient decreased over time. Intravenous cyclophosphamide was used less frequently after 2016, while mycophenolate mofetil and hydroxychloroquine were used frequently after 2016. The use of mizoribine reduced after 2014, whereas the other immunosuppressive medications showed no significant change over time; the use of biological agents was very limited. CONCLUSIONS: Oral prednisolone was the mainstay of treatment for cSLE, and the maximum daily dose has reduced over the past decade. The most frequently prescribed immunosuppressive therapy has shifted to mycophenolate mofetil over time.

Validation of the Japanese Transition Readiness Assessment Questionnaire.

BACKGROUND: For patients with childhood-onset chronic illnesses, the transition to adult care requires an understanding of transition readiness and the effectiveness of evaluation methods. However, no such psychometrically verified scales exist in Japan. This study aimed to develop a Japanese version of the Transition Readiness Assessment Questionnaire (TRAQ) and verify its validity and reliability. METHODS: The Japanese TRAQ was developed in accordance with international guidelines, followed by a preliminary survey to verify face validity among six participants who fulfilled the inclusion criteria. For the main survey 107 patients who fulfilled the same inclusion criteria were asked to complete the questionnaire and provide basic information. After descriptive statistics analysis, the construct validity of the Japanese TRAQ was tested using the t-test and Pearson's correlation coefficients. Cronbach's alpha coefficients were calculated to assess reliability. RESULTS: In the main survey, 76 participants with no missing data were included in the complete data analysis (40 males 36 females; mean age, 17.8 and 18.2 years, respectively). The mean total Japanese TRAQ score was 3.9. Cronbach's alpha coefficients were 0.94 overall and 0.8-0.96 for each of the four domains. The known-groups analysis revealed that older participants (r = 0.23, P = 0.044), those having knowledge of the disease name (yes [4.0] vs no [3.4]; P < 0.001), and making unaccompanied hospital visits (with parent/others [3.7] vs alone [4.4]; P < 0.001) had significantly higher total TRAQ scores. CONCLUSION: We confirmed preliminarily the validity and reliability of the Japanese TRAQ.

Updated version of Japanese Childhood Health Assessment Questionnaire (CHAQ).

Objectives: The Childhood Health Assessment Questionnaire (CHAQ) is one of the most widely used self- report questionnaires to measure functional status in Juvenile idiopathic arthritis (JIA). The Japanese version of the CHAQ (JCHAQ) has been revised to meet requirements of clinical international trials which need the same number of questions in each functional area of the CHAQ.Methods: The original JCHAQ consisted of 36 items, measuring eight functional areas. This was changed to 30 items of questionnaire so that each functional area has same number of questions as the original US English version. The revised version was professionally translated from English to Japanese, reviewed, and validated with Japanese JIA patients.Results: A total of 42 JIA patients were enrolled in the validation: seven systemic, 30 polyarticular/oligoarticular and five enthesis related. Most patients were well controlled and the median disability index (DI) scores was 0.0 [0-0.03]; however, significant correlation was seen with visual analog scale (VAS) of pain, VAS overall well-being, physician VAS, DAS (Disease Activity Score) 28-ESR, and JADAS (Juvenile Arthritis Disease Activity Score)-27. In comparison of two groups of disease activity, remission or inactive/low disease activity vs. moderate/high disease activity, both DAS28-ESR and JADAS-27 showed significant correlation with DI.Conclusion: The updated JCHAQ was a reliable and valid tool for the functional assessment of children with JIA. It is more suitable for international and transitional comparison.