Interleukin (IL)-18 is a pleiotropic, pro-inflammatory cytokine involved in the regulation of innate and adaptive immune responses. IL-18 has attracted increasing attention as a key mediator in ...autoinflammatory diseases associated with the development of macrophage activation syndrome (MAS) including systemic juvenile idiopathic arthritis and adult-onset Still’s disease. In these diseases, dysregulation of inflammasome activity and overproduction of IL-18 might be associated with the development of MAS by inducing natural killer cell dysfunction. Serum IL-18 levels are high in patients with these diseases and therefore are useful for the diagnosis and monitoring of disease activity. In contrast, a recent study revealed the overproduction of IL-18 was present in cases of autoinflammation without susceptibility to MAS such as pyogenic sterile arthritis, pyoderma gangrenosum, and acne (PAPA) syndrome. The pathogenic and causative roles of IL-18 remain unclear in these autoinflammatory diseases. Further investigations are necessary to clarify the role of IL-18 and its importance as a therapeutic target in the pathogenesis of autoinflammatory diseases.
To understand the awareness of transitional care in patients with JIA and their families.
A questionnaire survey on transitional care was conducted among patients with JIA during their transitional ...period who were attending the pediatric rheumatology of our university and the members of parents' association of JIA (the Asunaro-kai).
57.1% of patients and 35.9% of their parents did not know the word 'transitional care'. Approximately half of them did not have the opportunity to discuss transition or transfer to adult rheumatology. 61.2% of patients and 78.6% of their parents were worried about transition or transfer to adult rheumatology, and their biggest concern was about building trust with a new doctor. Approximately half of them wished to transfer to adult rheumatology after establishing a period of consultation with both pediatric and adult rheumatology. With regard to the timing of transfer, the majority of them wanted to consult with their doctors regardless of their age. The information they wanted to know was the prognosis of the disease itself, the medical system after adulthood, and data on pregnancy and childbirth.
The development of transitional care requires that pediatricians and adult rheumatologists work together to listen to the needs of patients and their families.
The pathology of rheumatic diseases and rheumatic diseases is systemic inflammatory disorders caused by autoinflammation and autoimmune responses, and a significant progress of inflammation science ...and rheumatology made diagnostic technique, a therapeutic drug, and therapy develop remarkably. On the contrary, in Japan, the regional disparities in the medical system are still large, and there are a lot of problems that should be solved. At first, regarding the solutions to such problems in pediatric rheumatic diseases, we need to investigate the actual situations and find the problems in specific regions; it is necessary to build medical care network taking regional characteristics in consideration in order to improve the medical quality in Japan. In addition, based on a long-term prospect, it is crucial to classify and manage each of problems surrounding patients, such as those on transitional care for chronic diseases, the cooperation with the parent association in Japan, and medical cost.
The aim of this study was to update the Japan College of Rheumatology (JCR) clinical practice guidelines (CPG) for the management of rheumatoid arthritis (RA; JCR CPG for RA) according to recent ...changes in the medical environment in Japan. This article is a digest version of the guidance.
We used the Grading of Recommendations, Assessment, Development, and Evaluation method to update the 2014 JCR CPG for RA. A consensus was formed by CPG panel members.
We identified 36 important clinical questions regarding drug treatment and developed corresponding recommendations for RA. The recommendations included the following RA medications: non-steroidal anti-inflammatory drugs, corticosteroids, conventional synthetic disease-modifying antirheumatic drugs, biological disease-modifying antirheumatic drugs, anti-receptor activator for nuclear factor-κB ligand antibodies, and Janus kinase inhibitors, as well as the tapering and discontinuation of these medications. Recommendations regarding the efficacy and safety of treatments in the elderly and patients with comorbidities were also developed. Finally, we used these recommendations to create an original algorithm for drug treatment for RA based on the Treat-to-Target approach.
The 2020 JCR CPG for RA provides a useful tool for rheumatologists, health care professionals, and patients with RA, enabling shared decision-making in a variety of clinical situations.
This case–control study investigated immune thrombocytopenic purpura (ITP) risk following live, inactivated, and simultaneous vaccination, with a focus on infants aged < 2 years. We matched case ...patients with ITP to one or two control patients with other diseases by institution, hospital visit timing, sex, and age. We calculated McNemar’s pairwise odds ratios (ORs 95% confidence interval) with 114 case–control pairs. The case group had 27 (44%) males and 22 (35%) infants, and the control group included 49 (43%) males and 42 (37%) infants. For all age groups, the McNemar’s OR for ITP occurrence was 1.80 (0.54–6.84,
p
= 0.64) for all vaccines. Among infants, these were 1.50 (0.17–18.0,
p
= 0.50) for all vaccines, 2.00 (0.29–22.1,
p
= 0.67) for live vaccines, and 1.00 (0.01–78.5,
p
= 0.50) for inactivated vaccines. Sex-adjusted common ORs for simultaneous vaccination were 1.52 (0.45–5.21,
p
= 0.71) for all vaccines, 1.83 (0.44–7.59,
p
= 0.40) for inactivated vaccines only, and 1.36 (0.29–6.30,
p
= 0.69) for mixed live and inactivated vaccines. In infants, these were 1.95 (0.44–8.72,
p
= 0.38), 1.41 (0.29–6.94,
p
= 0.67) and 2.85 (0.43–18.9,
p
= 0.28), respectively. These limited data suggest no significant ITP risk following vaccinations or simultaneous vaccination in any age group, including infants.
Background: Regular assessment of bone mineral density (BMD) by dual-energy X-ray absorptiometry (DXA) is essential for detecting glucocorticoid-induced osteoporosis in juvenile-onset autoimmune ...diseases. Z-score is used to standardize osteoporosis assessment in children and is evaluated with only one of three devices in Japan. The purpose of this study was to determine how many Japanese medical facilities for pediatric rheumatic diseases were unable to use Z-scores to evaluate osteoporosis. Methods: Electronic questionnaires were distributed between 2017 and 2019 to hospitals belonging to the Pediatric Rheumatology Association of Japan and to university hospitals and public children's hospitals that provide medical care for pediatric rheumatic diseases. The questionnaire inquired about the location of DXA measurement, manufacturer (Hologic, GE healthcare, Hitachi), and measurement site, and the answers were collected using Google Forms. Statcel 4 was used for analysis. Results: Overall, 120 facilities responded to the survey, of which 117 had DXA. In the remaining three facilities, DXA was not installed in two and was out of order in one. Bone loss in childhood was evaluated using a Z-score calculated from age-based reference values; however, 30% of hospitals without HOLOGIC DXA could not evaluate osteoporosis by Z-score in Japanese childhood. The characteristics of the hospitals enrolled in this study did not bias the selection of Hologic DXA. Conclusions: Neighboring institutions should consider sharing access to Hologic DXA equipment, to ensure use of uniform reference values. GE BMD reference values should be established for Japanese children.
To update and revise the diagnostic criteria for mixed connective tissue disease (MCTD) issued by the Japan Research Committee of the Ministry of Health, Labor, and Welfare (MHLW), a round table ...discussion by experts from rheumatology, dermatology, and pediatric medicine was conducted in multiple occasions.
The definition of MCTD, and items included in the diagnostic criteria were generated by consensus method and evaluation using clinical data of typical and borderline cases of MCTD, by applying to the diagnostic criteria for MCTD proposed in 1996 and 2004 by the Research Committee of MHLW.
To the end, all committee members reached consensus. Then, the criteria were assessed in an independent validation cohort and tested against preexisting criteria. The revised criteria facilitate an understanding of the overall picture of this disease by describing the concept of MCTD, common manifestations, immunological manifestation and characteristic organ involvement. Conditions with characteristic organ involvement include pulmonary arterial hypertension, aseptic meningitis and trigeminal neuropathy. Even if the overlapping manifestations are absent, MCTD can be diagnosed based on the presence of the characteristic organ involvement. Furthermore, the criteria were validated for applicability in actual clinical cases, and public comments were solicited from the Japan College of Rheumatology and other associated societies.
After being reviewed through public comments, the revised diagnostic criteria have been finalized.
Early intervention to manage high blood pressure (BP) in young adulthood is a promising approach for the prevention of future cardiovascular diseases. We aimed to examine the ability of childhood ...health information to predict the incidence of young adults with high BP. This cohort study included baseline clinical data of Japanese individuals aged 12-13 years. A total of 1129 participants were followed up for an average of 8.6 years. We examined the association of childhood variables consisting of body weight, body mass index, systolic BP, white blood cell count, red blood cell count, hemoglobin, hematocrit, platelet count, uric acid, total cholesterol, high-density lipoprotein cholesterol, and low-density lipoprotein cholesterol with the development of high BP defined as ≥120/80 mmHg at 18-22 years old. At follow-up, the prevalence of high BP was 42.2% in men and 7.7% in women. Young men with high BP had childhood baseline characteristics that included higher body weight, body mass index, systolic BP, red blood cell count, hemoglobin, hematocrit, and uric acid than normotensive men. Young women with high BP had higher body weight, systolic BP, and uric acid at baseline. Multivariable logistic regression analysis revealed that a model including body weight, systolic BP, hematocrit, and uric acid had the highest predictive power (AUC 0.65 95% CI, 0.62-0.69) for men, and a model including body weight, systolic BP, and uric acid had the highest predictive power (AUC 0.70 95% CI, 0.58-0.81) for women. Comprehensive childhood health information contributes to the prediction of high BP in young adults.
Objective To evaluate infliximab (IFX) in patients with Kawasaki disease (KD) that was unresponsive to additional intravenous immunoglobulin (IVIG) therapy and subsequent rescue with supplementary ...plasma exchange (PE) in patients unresponsive to treatment. Study design We studied 76 patients with KD who received IVIG therapy twice and were unresponsive to additional IVIG. Reults Seventy were treated with IFX alone (92.1%). Six patients who were unresponsive IFX (7.9%) were further treated by PE. This resulted in disappearance of fever and other clinical symptoms, and improvement of laboratory data. There was no severe life-threatening adverse events.Twelve of the 76 cases had developed coronary artery dilatation, and 3 had coronary artery aneurysm within 1 month of disease onset. At the end of follow-up, in all cases, coronary artery lesions were suppressed or reversed. Conclusions Treatment of intractable KD with sequential IVIG, IFX, and PE treatments in a step-wise protocol was effective.
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