Language：English   Publisher：Modern Rheumatology  

Objectives: This study aimed to describe the treatment selection for systemic lupus erythematosus (SLE) using data from the Kyushu Collagen Disease Network for SLE (KCDN-SLE) registry, a multicentre prospective registry in Japan. Methods: This study used data from patients registered between August 2022 and November 2023. Clinical characteristics, purpose of agent initiation, other candidate agents, and short-term efficacy and safety were evaluated. Results: We analysed 69 previously treated patients with SLE (mean age 43.7 years; 62 females, 7 males). Hydroxychloroquine, biological agents, and immunosuppressive agents were initiated during the maintenance phase in 12, 41, and 16 patients, respectively. In patients with active organ involvement, hydroxychloroquine and biological agents were widely used for initiation. In those who already achieved treatment goals, biological agents alone were predominantly selected. The SLE Disease Activity Index 2000 score and prednisolone dose declined significantly over a 6-month follow-up period. Among 48 patients with active disease, 22 achieved a lupus low disease activity state, but this had no evident association with the initiation of a biological agent. In total, 14 adverse events, predominantly infections, were observed. Conclusions: Biological agents were used preferentially, and the therapeutic agents were appropriately effective and mostly achieved the purpose of agent initiation.

DOI： 10.1093/mr/roae058

Web of Science

Scopus

PubMed

Language：English   Publisher：Tuberculosis  

Mycobacteria often cause chronic infection. To establish persistence in the host, mycobacteria need to evade host immune responses. However, the molecular mechanisms underlying the evasion strategy are not fully understood. Here, we demonstrate that mycobacterial cell wall lipids trigger an inhibitory receptor to suppress host immune responses. Mycolic acids are major cell wall components and are essential for survival of mycobacteria. By screening inhibitory receptors that react with mycobacterial lipids, we found that mycolic acids from various mycobacterial species bind to mouse Clec12A, and more potently to human Clec12A. Clec12A is a conserved inhibitory C-type lectin receptor containing immunoreceptor tyrosine-based inhibitory motif (ITIM). Innate immune responses, such as MCP-1 production, and PPD-specific recall T cell responses were augmented in Clec12A-deficient mice after infection. In contrast, human Clec12A transgenic mice were susceptible to infection with M. tuberculosis. These results suggest that mycobacteria dampen host immune responses by hijacking an inhibitory host receptor through their specific and essential lipids, mycolic acids. The blockade of this interaction might provide a therapeutic option for the treatment or prevention of mycobacterial infection.

DOI： 10.1016/j.tube.2022.102294

Scopus

PubMed

Language：English   Publisher：Oxford University Press  

症例1は30歳男性で、四肢の皮疹、発熱、腹痛、関節痛を呈して当院に来院した。5日前にCOVID-19ワクチン接種を受けており、接種前に服薬や感染徴候はなかった。身体所見では四肢と体幹に触知可能な紫斑を認め、臨床検査にて白血球数増加、CRP高値、顕微的血尿がみられた。抗核抗体は陽性、MPO-ANCA抗体とPR3-ANCA抗体は陰性であり、左前腕部の皮膚生検にて好中球浸潤を伴う表皮びらん、赤血球細胞漏出、血管周囲の核破片、フィブリン沈着を認めたことからIgA血管炎と診断した。静注プレドニゾロン(PSL)60mg/日を3日間、さらに経口PSL 40mg/日を開始した。その結果、1週後には症状は改善しPSLを漸減した。症例2は22歳男性で、下肢の皮疹と関節痛を訴えて来院した。6日前にCOVID-19ワクチン接種を受けており、身体所見では下肢に触知可能な紫斑を認め、臨床検査にて白血球数増加、CRP高値がみられ、皮膚生検と免疫蛍光染色の結果からIgA血管炎と診断した。経口PSL 30mg/日を開始したところ1週後には症状は改善しPSLを漸減した。

Language：Japanese   Publisher：JIBI TO RINSHO KAI  

<p>From 2014 to 2021, we examined the clinical features of 10 cases diagnosed with otitis media with ANCA-associated vasculitis (OMAAV) at our hospital. The first visit dates were 2014, 2015, and 2017 (1 case each), 2019 and 2020 (3 cases each), and 2021 (1 case). The period from the first visit to the start of treatment ranged from seven days to five months, and treatment was able to be started within 26 days from the first in all but 1 case (first visit in 2014). According to the Ministry of Health, Labor, and Welfare standards, ANCA-associated vasculitis was diagnosed in four cases with definitive granulomatosis with polyangiitis (GPA) and six with suspected GPA. Hypertrophic pachymeningitis was observed in one case. Facial nerve palsy was observed in two cases, ultimately improving in both cases. All patients remained alive during the observation period. The diagnostic criteria proposed in 2015 enabled the early diagnosis of OMAAV and facilitated early treatment in collaboration with collagen physicians.</p>

DOI： 10.11334/jibi.68.4_235

CiNii Research

Language：Japanese   Publisher：耳鼻と臨床会  

2014-2021年の期間において、当院でANCA関連血管炎性中耳炎(OMAAV)と診断した10例について臨床的特徴を検討した。初診日は2014年、2015年、2017年が各1例、2019年と2020年が各3例、2021年が1例で、初診診療科は耳鼻咽喉科が9例、内科が1例であった。初診から治療開始までの期間は7日から5ヵ月で、2014年初診の1例を除き26日以内に治療を開始できた。厚労省基準によるANCA関連血管炎の診断は多発血管炎性肉芽腫症(GPA)確実例が4例、GPA疑いが6例であった。肥厚性硬膜炎は1例、顔面神経麻痺は2例に合併していた。顔面神経麻痺は2例とも改善し、生命予後は全例生存中であった。診断基準2015が提唱されたことで早期診断が可能になり、内科医と連携した早期治療が容易となった。(著者抄録)

Language：Japanese  

Language：Japanese  

Language：Japanese  

Language：Japanese  

Language：Japanese  

Language：Japanese  

Language：Japanese  

Language：Japanese