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OBJECTIVES: This study aimed to understand the status quo of medical treatments of the primary disease and pregnancy outcomes in patients with Takayasu arteritis (TAK) and children's birth outcomes. METHODS: This study retrospectively enrolled patients with TAK who conceived after the disease onset and were managed at medical facilities participating in the Japan Research Committee of the Ministry of Health, Labor, and Welfare for Intractable Vasculitis. RESULTS: This study enrolled 51 cases and 68 pregnancies 2019-2021. Of these, 48 cases and 65 pregnancies (95.6%) resulted in delivery and live-born babies. The median age of diagnosis and delivery was 22 and 31, respectively. Preconception therapy included prednisolone (PSL) in 51 (78.5%, median 7.5 mg/day), immunosuppressants in 18 (27.7%), and biologics in 12 (18.5%) pregnancies. Six cases underwent surgical treatment before pregnancy. Medications during pregnancy included PSL in 48 (73.8%, median: 9 mg/day), immunosuppressants in 13 (20.0%), and biologics in 9 (13.8%) pregnancies. Enlargement of an aneurysm was reported in one pregnancy, which might be associated with increased circulating plasma volume. TAK relapsed in 4 (6.2%) and 8 (12.3%) pregnancies during pregnancy and after delivery, respectively. Additionally, 13/62 (20.9%) preterm infants and 17/59 (28.8%) low birth weight infants were observed, and none had serious postnatal abnormalities. Of the 51 confirmed infants, 42 (82.4%) were exclusively breastfed or mixed with formula. CONCLUSION: Most pregnancies in TAK were manageable with PSL at ≤10 mg/day. Relapse during pregnancy and postpartum occurred in <20% of pregnancies.
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OBJECTIVES: This study aimed at identifying challenges nurses face in caring for patients with juvenile idiopathic arthritis (JIA) in Japan. METHODS: Nurses certified by the Japan Rheumatism Foundation were asked to describe their challenges with caring for JIA patients. Data was analyzed using qualitative content analysis. RESULTS: Responses were collected from 89 participants. 58 issues were identified from 40 participants with experience caring for JIA patients. Sixteen categories emerged, grouped into five challenges: communication, understanding, expertise, system, and collaboration. Care for JIA patients included different challenges from adult patients with rheumatoid arthritis, such as complicated patient-parent relationships, inadequate patient independence and insufficient patient information. Moreover, 76 issues from 49 participants with no experience were identified. Seven categories emerged, grouped into two challenges: expertise and opportunity. Issues included in expertise between both groups were similar. Even with no experience caring for JIA patients, nurses recognized the importance of acquiring knowledge. CONCLUSION: This is the first study in Japan regarding the difficulties nurses face in caring for JIA patients. Multidisciplinary team care and a comprehensive understanding of the patient journey, including relationships with guardians, acquaintances, and healthcare providers, is crucial to improve treatment outcome and overall patient quality of life.
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OBJECTIVES: A quality indicator for the treatment of systemic lupus erythematosus during pregnancy and childbirth that is useful for sharing standard treatment policies has not yet been developed. This study aimed to develop a quality indicator for systemic lupus erythematosus associated with pregnancy and childbirth. METHODS: To identify candidate quality indicators, we conducted a systematic literature review on the development of quality indicators for systemic lupus erythematosus related to pregnancy and childbirth and on clinical practice guidelines. Candidate quality indicator items were extracted from the final selected articles, and a first evaluation, panel meeting, and second evaluation were conducted to determine whether the candidate items were appropriate as quality indicators. Items for which all panel members reached a consensus were designated pregnancy and childbirth-related systemic lupus erythematosus quality indicators. RESULTS: Four articles on systemic lupus erythematosus-quality indicator development and 28 practice guidelines were listed through abstract/text screening. Based on these studies, 52 candidate quality indicators were extracted that were limited to items related to pregnancy and childbirth, and 41 items were selected on which all panel members agreed. CONCLUSION: We developed pregnancy-related systemic lupus erythematosus quality indicators using the RAND/UCLA method and selected 41 items, which could be used clinically.
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OBJECTIVES: To access the real-world clinical management of physicians who treat Takayasu arteritis (TAK) and giant cell arteritis (GCA) after the publication of the Japanese Circulation Society (JCS) 2017 Guidelines for the Management of Vasculitis Syndrome. METHODS: This descriptive, cross-sectional study utilized self-administered electronic questionnaires, which were answered in February 2022 by physicians treating TAK or GCA and registered with Macromill Inc. RESULTS: The 329 survey respondents comprised 110 cardiologists, 110 rheumatologists, 34 cardiovascular surgeons, 24 surgeons, 35 internal medicine physicians, 13 nephrologists, and 7 pediatricians. The 2017 JCS Guidelines were the most commonly referenced information source for resolving clinical questions, accessed by 70% of respondents. Ophthalmoscopy was performed in only 50% of patients with TAK, and in 70% for GCA. The median percentages of patients who underwent 18F-fluorodeoxyglucose-positron emission tomography/computed tomography for TAK and GCA patients were 23% and 20% at diagnosis, respectively, and 10% each at follow-up within 12 months. Tocilizumab was the most frequently used medication in combination with glucocorticoids for both TAK and GCA, especially in remission induction therapy for relapsed patients. CONCLUSIONS: The majority of physician treating TAK and GCA referred to the 2017 JCS guidelines. This report clarified the current clinical practice for large vessel vasculitis in Japan, providing information for the next revision of the guidelines.
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OBJECTIVE: Although treatments for juvenile idiopathic arthritis (JIA) have seen considerable advancements, there remains a lack of clear guidelines on withdrawing medications. This study aimed to investigate the current strategies for discontinuing non-systemic JIA treatment. METHODS: A web-based questionnaire was distributed to Pediatric Rheumatology Association of Japan members. RESULTS: According to 126 responses, the most significant factors influencing JIA treatment tapering were the duration of clinically inactive disease, medication toxicity, and a history of arthritis flares. Respondents were often cautious about discontinuing medication if symptoms, e.g., 'morning stiffness' or 'intermittent joint pain', persisted. Among subtypes, oligoarticular JIA was more amenable to treatment tapering, whereas rheumatoid factor-positive polyarticular JIA proved less amenable. Most respondents started medication tapering after a continuous clinical inactive duration exceeding 12 months, and >50% of them required >6 months to achieve treatment discontinuation. Additionally, 40% of respondents consistently underwent imaging before treatment tapering. CONCLUSIONS: The relative risks of treatment continuation and withdrawal should be considered, and decisions should be made accordingly. To obtain improved understanding of and more robust evidence for the optimal strategies for safely discontinuing JIA treatment, it is crucial to continue investigations, including long-term outcomes.
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OBJECTIVE: We assess the clinical characteristics of patients with cryopyrin-associated periodic syndrome (CAPS) in Japan and evaluate the real-world efficacy and safety of interleukin-1 (IL-1) inhibitors, primarily canakinumab. METHODS: Clinical information was collected retrospectively, and serum concentrations of canakinumab and cytokines were analyzed. RESULTS: A total of 101 patients were included, with 86 and 15 carrying heterozygous germline and somatic mosaic mutations, respectively. We identified 39 mutation types, and the common CAPS-associated symptoms corresponded with those in previous reports. Six patients (5.9% of all patients) died, with four of the deaths caused by CAPS-associated symptoms. Notably, 73.7% of patients (100%, 79.6%, and 44.4% of familial cold autoinflammatory syndrome, Muckle-Wells syndrome, and chronic infantile neurological cutaneous articular syndrome/neonatal onset multisystem inflammatory disease, respectively) achieved complete remission with canakinumab, and early therapeutic intervention was associated with better auditory outcomes. In some patients, canakinumab treatment stabilized the progression of epiphysial overgrowth and improved height gain, visual acuity, and renal function. However, 23.7% of patients did not achieve inflammatory remission with crucial deterioration of organ damage, with two dying while receiving high-dose canakinumab treatment. Serological analysis of canakinumab and cytokine concentrations revealed that the poor response was not related to canakinumab shortage. Four inflammatory nonremitters developed inflammatory bowel disease (IBD)-unclassified during canakinumab treatment. Dual biologic therapy with canakinumab and anti-tumor necrosis factor-α agents was effective for IBD- and CAPS-associated symptoms not resolved by canakinumab monotherapy. CONCLUSION: This study provides one of the largest epidemiologic data sets for CAPS. Although early initiation of anti-IL-1 treatment with canakinumab is beneficial for improving disease prognosis, some patients do not achieve remission despite a high serum concentration of canakinumab. Moreover, IBD may develop in CAPS after canakinumab treatment.
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Anticorpos Monoclonais Humanizados , Síndromes Periódicas Associadas à Criopirina , Humanos , Síndromes Periódicas Associadas à Criopirina/tratamento farmacológico , Síndromes Periódicas Associadas à Criopirina/genética , Anticorpos Monoclonais Humanizados/uso terapêutico , Japão , Feminino , Masculino , Estudos Retrospectivos , Criança , Pré-Escolar , Adulto , Adolescente , Adulto Jovem , Resultado do Tratamento , Pessoa de Meia-Idade , Lactente , Proteína 3 que Contém Domínio de Pirina da Família NLR/genética , Mutação , Indução de RemissãoRESUMO
OBJECTIVE: The European League Against Rheumatism recommends that the disease activity of systemic lupus erythematosus should be stable before pregnancy because complications and disease flares increase if pregnancy occurs while disease activity is high. However, some patients have ongoing serological activity even after treatment. Herein, we investigated how physicians decide on the acceptability of pregnancy in patients showing only serological activity. METHODS: A questionnaire was administered from December 2020 to January 2021. It included the characteristics of physicians, facilities, and the allowance for pregnancies of patients using vignette scenarios. RESULTS: The questionnaire was distributed to 4946 physicians, and 9.4% responded. The median age of respondents was 46 years, and 85% were rheumatologists. Pregnancy allowance was significantly affected by the duration of the stable period and status of serological activity [duration: proportion difference 11.8 percentage points (p.p.), P < .001; mild activity: proportion difference -25.8 p.p., P < .001; high activity: proportion difference -65.6 p.p., P < .001]. For patients with high-level serological activity, 20.5% of physicians allowed pregnancy if there were no clinical symptoms for 6 months. CONCLUSIONS: Serological activity had a significant effect on the acceptability of pregnancy. However, some physicians allowed patients with serological activity alone to become pregnant. Further observational studies are required to clarify such prognoses.
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Lúpus Eritematoso Sistêmico , Médicos , Complicações na Gravidez , Gravidez , Feminino , Humanos , Pessoa de Meia-Idade , Complicações na Gravidez/diagnóstico , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Resultado da Gravidez , Estudos RetrospectivosRESUMO
To optimize patient prognosis, patient needs, including unmet needs, should be adequately assessed. However, such needs are more challenging to report and, consequently, more likely to go unmet compared with the needs reported by physicians. We aimed to determine the appropriate direction of future research on unmet medical needs in rheumatic diseases in Japan by conducting a literature review. We searched PubMed and Web of Science using 23 terms linked to unmet medical needs for major rheumatic diseases in Japan. Further, we collected articles on health-related quality of life and investigated the scales used for assessment, as well as whether the terms "unmet needs" or "unmet medical needs" were used. We identified 949 papers on 10 diseases, including systemic lupus erythematosus, systemic sclerosis, dermatomyositis, juvenile idiopathic arthritis, adult-onset Still's disease, antiphospholipid syndrome, mixed connective tissue disease, Takayasu arteritis, Sjögren's syndrome, and Behçet's disease; 25 of the 949 papers were selected for full-text review. Fifteen articles on five diseases were related to health-related quality of life. The term "unmet needs" was used in only one article. Six out of 15 studies used the 36-item short form survey, whereas the scales used in other studies differed. The optimal treatment plan determined by a physician may not necessarily align with the best interests of the patient. In clinical research, cross sectional and standardized indicators of health-related quality of life should be employed along with highly discretionary questionnaires to assess and optimize resource allocation in healthcare and simultaneously achieve patient-desired outcomes.
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Artrite Juvenil , Doenças Reumáticas , Adulto , Humanos , Japão , Estudos Transversais , Qualidade de Vida , Doenças Reumáticas/diagnóstico , Doenças Reumáticas/terapiaRESUMO
OBJECTIVES: This study aimed to identify the challenges faced by nurses engaged in rheumatology care in Japan. METHODS: We conducted a questionnaire survey of nurses in Japan in 2020 on challenges experienced in rheumatology care. The participants were nurses certified by the Japan Rheumatism Foundation. Participants' answers were coded and categorized based on relevant phrases and words. Content analysis was performed on the findings. RESULTS: Responses were collected from 162 participants, and a total of 228 issues were identified. Eighteen categories with 56 subcategories emerged from the data analysis, which were grouped into five types of challenges: (1) communication, (2) understanding, (3) expertise, (4) system, and (5) collaboration. In particular, the results highlighted deficiencies in needs-based multidisciplinary team care. CONCLUSION: This study elucidated issues experienced by rheumatology nurses in clinical settings in Japan. Furthermore, this investigation revealed the necessity of patient-centered multidisciplinary team care, including health professionals, patients, and other relevant individuals. This study provided practical directions to facilitate the implementation of effective care focused on improving patients' quality of life.
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OBJECTIVES: In clinical trials, tocilizumab (TCZ) is efficacious in patients with giant cell arteritis (GCA). This study evaluated the real-world tolerability and effectiveness of TCZ in Japanese patients with GCA. METHODS: In this multicentre, prospective, phase 4, large-scale, observational study, patients with GCA (with no TCZ treatment 6 months before the study) were recruited from 71 centres across Japan. Patients received subcutaneous TCZ 162 mg weekly (observation period, 52 weeks). RESULTS: Of the 117 patients (female, 70.1%; mean age, 74.2 years; mean disease duration, 1.4 years; treated for new-onset GCA, 71.8%; presence of large-vessel lesions [LVLs], 61.5%; previous immunosuppressant use, 28.2%; glucocorticoids at baseline, 95.7% [mean: 22.4 mg/day]), 38.5% reported adverse events. The most common adverse events of special interest were neutropenia and leukopenia (7.7%), followed by serious infection (6.0%). The relapse-free proportion was 85.0%; relapse after remission, 6.0%; and no remission, 9.0%. At the last observation, 94.2% of relapse-free patients received a concomitant glucocorticoid dose of <10 mg/day. Fatigue, headache, neck pain and absence of LVLs were positively associated with the relapse. CONCLUSIONS: TCZ was effective and well tolerated in Japanese patients with GCA and may be an effective treatment option combined with glucocorticoids.
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Coronavirus disease 2019 (COVID-19) in children can be compounded by concurrent diseases and immunosuppressants. For the first time, we aimed to report the clinical features of concurrent COVID-19 and pediatric rheumatic disease (PRD) in Japan. Pediatric Rheumatology Association of Japan members were surveyed between 1 April 2020 and 31 August 2022. Outcome measurements included the clinical features of concurrent PRD and COVID-19. Questionnaire responses were obtained from 38 hospitals. Thirty-one hospitals (82%) had children with PRD and COVID-19. The female-to-male ratio in these children (n = 156) was 7:3, with half aged 11-15 years. The highest proportion of children with PRD and COVID-19 was accounted for by juvenile idiopathic arthritis (52%), followed by systemic lupus erythematosus (24%), juvenile dermatomyositis (5%), scleroderma (4%), and Takayasu arteritis (3%). Of children with PRD, a significant majority (97%) were found to be asymptomatic (10%) or presented with mild symptoms (87%) of the COVID-19 infection. No severe cases or deaths were observed. Regarding the use of glucocorticoids, immunosuppressants, or biologics for PRD treatment before COVID-19, no significant difference was found between asymptomatic/mild and moderate COVID-19 in children with PRD. Therefore, COVID-19 is not a threat to children with PRD in Japan.
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COVID-19 , Doenças Reumáticas , Reumatologia , Criança , Humanos , Masculino , Feminino , COVID-19/epidemiologia , Doenças Reumáticas/diagnóstico , Doenças Reumáticas/epidemiologia , Doenças Reumáticas/tratamento farmacológico , Japão/epidemiologia , Imunossupressores/uso terapêutico , Inquéritos e QuestionáriosRESUMO
OBJECTIVE: We conducted a nationwide epidemiological study to estimate the number of patients with Takayasu arteritis (TAK) and giant cell arteritis (GCA) in Japan and to describe the clinical characteristics of these patients. METHODS: The first survey was designed to estimate the number of patients with TAK and GCA who were treated at medical institutions in Japan in 2017. The second survey was designed to collect data on the clinical characteristics of the patients who were reported in the first survey. RESULTS: Of the 3495 institutions selected for the first survey, 1960 (56.1%) responded. The number of patients with clinically diagnosed TAK and GCA was estimated to be 5320 (95% confidence interval, 4810-5820) and 3200 (95% confidence interval, 2830-3570), respectively. Aortic regurgitation was reported in 35% of patients with TAK, and eye-related comorbidities were observed in 30.4% of patients with GCA. The common carotid and internal carotid arteries were the most frequently involved in patients with TAK (62.7%). Subclavian artery lesions and thoracic or abdominal aorta lesions were reported in 31% and 42.6% of patients with GCA, respectively. CONCLUSIONS: The number of patients with TAK and GCA was estimated simultaneously, and significant differences in clinical characteristics were observed between the two diseases.
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Arterite de Células Gigantes , Arterite de Takayasu , Humanos , Japão/epidemiologia , Arterite de Células Gigantes/diagnóstico , Artérias Carótidas/patologia , Arterite de Takayasu/patologia , ComorbidadeRESUMO
OBJECTIVES: To investigate an optimal composite score for disease activity in adult JIA from the viewpoint of the subsequent changes in physical function. METHODS: Patients with JIA under the following conditions were enrolled: 1) disease onset < 18 years; 2) registered in the database by Tokyo Women's Medical University for the first time between 2000 and 2020; and 3) ≥18 years old at the time of registration. Patients were stratified according to mean disease activity scores in SDAI, DAS28, and JADAS-27 during the first year from baseline. Trends of estimated mean change in Japanese-HAQ score (ΔJ-HAQ) from baseline to 2 years later was examined across the stratified groups of each index. RESULTS: We included 294 eligible individuals (median age at onset, 14.0 years; RF positive in 64.7%). A significant increasing trend of the estimated mean ΔJ-HAQ at 2 years after baseline was observed along with an increase in the mean disease activity during the first year measured using DAS28 (p = 0.01) and SDAI (p = 0.018), but not using JADAS-27. CONCLUSIONS: Disease activity measured using SDAI and DAS28, but not using JADAS27, was significantly associated with subsequent changes in physical function in adult patients with JIA.
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Antirreumáticos , Artrite Juvenil , Humanos , Adulto , Feminino , Adolescente , Artrite Juvenil/tratamento farmacológico , Índice de Gravidade de Doença , Sedimentação Sanguínea , Antirreumáticos/uso terapêuticoRESUMO
OBJECTIVES: We evaluated the real-world tolerability and effectiveness of tocilizumab in Japanese patients with Takayasu arteritis (TAK). METHODS: Patients with TAK who had not received tocilizumab in the previous 6 months were enrolled in ACTEMRA® (ACT)-Bridge, a phase 4, observational study, from 66 Japanese institutions (enrolment period, September 2017 to September 2020) and received weekly subcutaneous tocilizumab 162 mg (observation period, 52 weeks). RESULTS: Among 120 patients included (mean age, 38.4 years; mean disease duration, 7.7 years; treated for relapse, 50.8%; previous immunosuppressant use, 57.5%; glucocorticoid use at baseline, 97.5%), 49 (40.8%) reported adverse events. The most common adverse event of special interest was serious infection (7.5%). Relapse was observed in 24 (20.0%) patients (0.8%, 2.5%, and 16.7% reporting ≥3, 2, and 1 relapses, respectively). The reasons for diagnosing relapse included chest and back pain (45.8%), neck pain (25.0%), fatigue (16.7%), fever and headache (12.5% each), abnormal imaging findings (50.0%), and elevated inflammatory markers (16.7%). At the last observation, 83.0% of relapse-free patients recorded a concomitant glucocorticoid dose (prednisolone equivalent) <10 mg/day. CONCLUSIONS: This study demonstrated the effectiveness of tocilizumab in patients with TAK, with no new safety concerns. Tocilizumab plus glucocorticoids may be considered a treatment option for TAK.
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Glucocorticoides , Arterite de Takayasu , Humanos , Adulto , Glucocorticoides/efeitos adversos , Arterite de Takayasu/tratamento farmacológico , Japão , Anticorpos Monoclonais Humanizados/efeitos adversos , Recidiva , Resultado do TratamentoRESUMO
OBJECTIVES: Although epidemiological surveys of paediatric rheumatic diseases in Japan have been conducted, they were single surveys with no continuity. This is the first report of the Pediatric Rheumatology Association of Japan registry database, which was established to continuously collect data for paediatric rheumatic diseases. METHODS: Pediatric Rheumatology International Collaborate Unit Registry version 2 (PRICUREv2) is a registry database established by the Pediatric Rheumatology Association of Japan. The registry data were analysed for the age of onset, time to diagnosis, sex differences, seasonality, and other factors. RESULTS: Our data showed the same trend regarding rates of paediatric rheumatic diseases reported in Japan and other countries. The age of onset was lower in juvenile idiopathic arthritis (JIA) and juvenile dermatomyositis and higher in systemic lupus erythematosus and Sjögren's syndrome. The time to diagnosis was relatively short in JIA and systemic lupus erythematosus but longer in juvenile dermatomyositis and Sjögren's syndrome. Rheumatoid factor-positive polyarticular JIA showed a seasonality cluster with regard to onset. CONCLUSION: PRICUREv2 aided the retrieval and evaluation of current epidemiological information on patients with paediatric rheumatic diseases. It is expected that the data collection will be continued and will be useful for expanding research in Japan.
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Artrite Juvenil , Dermatomiosite , Lúpus Eritematoso Sistêmico , Doenças Reumáticas , Reumatologia , Síndrome de Sjogren , Criança , Humanos , Masculino , Feminino , Doenças Reumáticas/epidemiologia , Dermatomiosite/diagnóstico , Dermatomiosite/epidemiologia , Síndrome de Sjogren/diagnóstico , Síndrome de Sjogren/epidemiologia , Japão/epidemiologia , Artrite Juvenil/epidemiologia , Sistema de Registros , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/epidemiologiaRESUMO
Introduction: This study established an independent evaluation index for patients with childhood-onset chronic diseases in Japan. Methods: From November to December 2020, three Delphi rounds were conducted. Thirty-nine participants completed at least one survey. We asked them about targets of social independence for 10 types of activities (education/labor/finance/acquisition of necessities/housing/transportation/leisure/social relationship/intimate relationships/sexuality). The Delphi technique was to be repeated until a consensus of over 80% of participants was reached. Results: The targets chosen for measuring independence in patients with childhood-onset chronic diseases were as follows: "Graduation from high school," "Labor for livelihood (including temporary turnover)," "Financially independent (including temporary turnover, excluding students)," "Buy or rent a house and buy the daily necessities and get the public services you need to live," "Do housework alone," "Plan alone and use transportation to get around," "Participate in play/recreation/leisure activities on own initiative," "Engage in relationships with other people outside of a limited environment (home, school, office, hospital, etc.)," "Create and maintain intimate or romantic relationships between individuals (couples, lovers, sexual partners)," and "Use or know how to use contraceptives and how to prevent sexually transmitted diseases." Conclusions: We established an independent evaluation index for patients with childhood-onset chronic diseases in Japan through a three-round Delphi process. The assessment of social independence using our independent evaluation index may help plan for and provide appropriate support and assistance to these patients.
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Medical advances in childhood-onset chronic diseases (CCDs) have significantly improved the prognosis of these diseases; however, they have also resulted in an increase in the number of cases requiring continued medical care in adulthood. The transition from the pediatric to adult healthcare system has recently received worldwide attention. In Japan, in the last decade, there has been a growing awareness of the transition to adult health care in each specialized chronic disease area with a childhood onset. This review focuses on transitional care in pediatric rheumatology, a CCD. Non-pediatric rheumatology departments, such as rheumatology and orthopedics, are potential counterparts for accepting pediatric rheumatology patients; however, several challenges must be met for a seamless transition to adult care. The characteristics of pediatric rheumatic diseases, which are rarer than non-pediatric rheumatic diseases, and the status and problems in transition will be outlined. The mission of pediatricians is not only to follow up diseases but also to support patients' independence. All medical staff and multidisciplinary professionals must cooperate toward this new goal.
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Since the Japan Pediatric Society published its "Recommendations on Transitional Care for Patients with Childhood-Onset Chronic Diseases" in 2014, there has been an increased interest in the health care transition of adolescents with childhood-onset chronic diseases in Japan. However, the actual status of healthcare transition was not studied yet. The purpose of this study was to explore the prevalence of transitional support for adolescent patients with childhood-onset chronic disease and the factors hindering their transition. We conducted an anonymous questionnaire survey in August 2020, targeting physicians and nurses involved in health care transition at 494 pediatric facilities in Japan. Survey items included demographic data, health care systems related to transition to adult departments, health care transition programs based on Six Core Elements (establishing transition policy, tracking and monitoring transition progress, assessing patient readiness for transition, developing the transition plan with a medical summary, transferring the patient, completing the transfer/following up with the patient and family), barriers to transition (34-item, 4-point Likert scale), and expectations in supporting transition (multiple-choice responses), which consisted of five items (78 questions); all questions were structured. Descriptive statistics were used for analysis. Of the 225 responses collected (45.5% response rate), 88.0% were from pediatricians. More than 80% of respondents transferred patients of 20 years or older, but only about 15% had took a structured transition process of four or more based on the Six Core Elements. The top transition barriers were "intellectual disability/rare disease" and "dependence on pediatrics" as patient/family factors, and "lack of collaboration with adult healthcare (relationship, manpower/system, knowledge/understanding)" as medical/infrastructure factors. The study provides future considerations, including the promotion of structured health care transition programs, development of transitional support tailored to the characteristics of rare diseases and disorders, and establishment of a support system with adult departments.
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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.