記述言語：英語   掲載種別：研究論文（学術雑誌）  

Trisomy 21 (Down syndrome) is sometimes complicated by congenital heart disease; however, comorbid type I diabetes mellitus and diseases involving autoantibodies, such as Hashimoto disease and Graves disease, are not uncommon. Autoinflammatory diseases such as Kawasaki disease and systemic juvenile idiopathic arthritis are rare. We report a rare case of trisomy 21 with systemic juvenile idiopathic arthritis that responded well to the initial course of methylprednisolone pulse therapy but flared up and was complicated by macrophage activation syndrome (MAS). Subsequent methylprednisolone pulse therapy and cyclosporine resolved this condition. Cytokines were analyzed at several time points during the clinical course and revealed that interleukin-18, interleukin-6, and chemokine ligand 9 levels were elevated at MAS onset in the present patient, even though clinical symptoms had abated. Thus, early analysis of cytokine profiles should be performed to assess MAS risk and determine treatment intensity, even in T21 patients.

DOI： 10.1272/jnms.JNMS.2023_90-605

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Coronary peripheral circulatory disturbances in the remote stage of Kawasaki disease have been reported. In this study, of the 50 patients in the remote stage of Kawasaki disease who underwent coronary perfusion evaluation using adenosine-loaded 13N-ammonia positron emission tomography, 28 patients who did not have stenosis of ≥75% in the left coronary artery underwent an evaluation for myocardial flow reserve (MFR) of the left anterior descending artery (LAD) and left circumflex artery (LCx). Clinical findings were compared between patients with normal (≥2.0) and abnormal (<2.0) MFRs. In the group with an abnormal MFR in the LAD, the responsiveness of the coronary vascular resistance to adenosine stress decreased even in the LCx (3.50 ± 1.23 vs. 2.39 ± 0.25, p = 0.0100). In the group with an abnormal MFR in the LCx, the responsiveness of the coronary vascular resistance in the LAD also decreased (3.27 ± 1.39 vs. 2.03 ± 0.25, p = 0.0105), and the age of onset of Kawasaki disease tended to be younger in the group with abnormal MFR in the LAD and LCx. We found that the peripheral coronary circulation was extensively impaired in the remote stage of Kawasaki disease, suggesting that an early onset of Kawasaki disease may affect the peripheral coronary circulation in later years.

DOI： 10.3390/jcm11041134

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: Kawasaki disease (KD), a systemic vasculitis, is suspected to be related to abnormalities in innate immunity. Based on the important role of IL-1 signaling in innate immunity, we investigated the effects of an anti-IL-1β antibody using a Candida albicans water-soluble fraction (CAWS)-induced mouse model of KD. METHODS: CAWS (0.5 mg/mouse) was injected intraperitoneally into 5-week-old DBA/2 mice on five consecutive days. An anti-Murine IL-1β antibody (01BSUR) was administered at various doses (2.5, 5.0, and 10.0 mg/kg) and time points (2 days before, same day, and 2, 5, 7, and 14 days after CAWS administration). After 4 weeks, vasculitis in the aortic root was investigated histologically. Cytokines including IL-1β, -6, -10, and TNF-α were also measured. RESULTS: Groups administered 01BSUR at all doses showed a significant reduction in the area of vasculitis. In addition, 01BSUR inhibited vasculitis until 7 days after CAWS administration. In the analysis of various time points, the level of IL-6 was lower in all groups compared to the CAWS only group, but the levels of IL-1β, TNFα, and IL-10 were lower when 01BSUR was administered before CAWS. On the other hand, TNFα and IL-10 levels were restored when 01BSUR was administered after CAWS, suggesting that 01BSUR may have additional effects beyond blocking IL-1β signaling. CONCLUSIONS: The anti-IL-1β antibody significantly attenuated CAWS-induced vasculitis. The mechanism of inhibiting vasculitis is thought to include inhibition of the IL-1β pathway and additional effects beyond blocking IL-1β signaling.

DOI： 10.1272/jnms.JNMS.2019_86-206

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