Language：Japanese   Publisher：関東リウマチ研究会  

症例は10歳男児で、多関節痛、筋力低下、紅斑を主訴とした。入院時身体所見、皮膚所見、血液検査、画像所見より抗MDA5抗体陽性若年性皮膚筋炎と診断した。主体は皮膚・筋・関節病変で、間質性肺炎は軽微であった。メチルプレドニゾロン(mPSL)投与後、PSL、シクロフォスファミド間欠静注療法を行った。治療開始2週間後に関節症状の速やかな改善・CDASI低下・CMAS上昇、3ヵ月後に左上葉すりガラス影の消失を認めた。

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Language：Japanese   Publisher：(一社)日本リウマチ学会  

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Language：Japanese   Publisher：(一社)日本内科学会  

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Language：Japanese   Publisher：日本医科大学医学会  

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Language：English   Publishing type：Research paper (scientific journal)  

OBJECTIVE: Ultrasound is a promising tool to foster much-needed improvement of skin assessment in systemic sclerosis (SSc). Our aim was to develop evidence and expert opinion-based recommendations to promote the standardisation and harmonisation of technical execution and reporting of skin ultrasound studies in SSc. METHODS: A multidisciplinary task force of 16 members from five European countries and Japan was convened under the auspices of World Scleroderma Foundation. First, a systematic literature review (SLR) was performed. Then, each member proposed and formulated items to the overarching principles, recommendations and research agenda. Two rounds of mails exchange for consensus as well as an on-line meeting were performed to debate and refine the proposals. Two Delphi rounds of voting resulted in the final recommendations. Levels of evidence and strengths of recommendations were assigned, and task force members voted anonymously on the level of agreement with each of the items. RESULTS: Five overarching principles and seven recommendations were developed, based on an SLR and expert opinion, through consensus procedures. The overarching principles highlight the promising role of skin ultrasound in SSc assessment, the need for standardisation of technical aspects, sufficient training and adequate equipment. The recommendations provide standards for the execution and reporting of skin ultrasound in SSc. The research agenda includes the need for more research into unmet needs according to Outcome Measures in Rheumatology Algorithm requirements. CONCLUSION: These are the first recommendations providing guidance on the execution and reporting of skin ultrasound in SSc patients, aiming at improving the interpretability, reliability and generalisability of skin ultrasound, thus consolidating its role in research and practice.

DOI： 10.1136/rmdopen-2022-002371

PubMed

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Language：English   Publishing type：Research paper (scientific journal)  

OBJECTIVE: To summarize the published evidence in the literature on the role of ultrasound and elastography to assess skin involvement in systemic sclerosis (SSc). METHODS: A systematic literature review (SLR) was performed within the "Skin Ultrasound Working Group" of the World Scleroderma Foundation, according to the Cochrane Handbook. A search was conducted in Pubmed, Cochrane Library and Embase databases from 1/1/1979 to 31/5/2021, using the participants, intervention, comparator and outcomes (PICO) framework. Only full-text articles involving adults, reported in any language, assessing ultrasound to quantify skin pathology in SSc patients. Two reviewers performed the assessment of risk of bias, data extraction and synthesis, independently. RESULTS: Forty-six studies out of 3248 references evaluating skin ultrasound and elastography domains were included. B-mode ultrasound was used in 30 studies (65.2%), elastography in nine (19.6%), and both methods in seven (15.2%). The ultrasound outcome measure domains reported were thickness (57.8%) and echogenicity (17.2%); the elastography domain was stiffness (25%). Methods used for image acquisition and analysis were remarkably heterogeneous and frequently under-reported, precluding data synthesis across studies. The same applies to contextual factors and feasibility. Our data syntheses indicated evidence of good reliability and convergent validity for ultrasound thickness evaluation against mRSS and skin histological findings. Stiffness and echogenicity have limited evidence for validity against histological findings. Evidence for sensitivity to change, test-retest reliability, clinical trial discrimination or thresholds of meaning is limited or absent for reported ultrasound domains. CONCLUSION: Ultrasound is a valid and reliable tool for skin thickness measurement in SSc but there are significant knowledge gaps regarding skin echogenicity assessment by ultrasound and skin stiffness evaluation by elastography in terms of feasibility, validity and discrimination. Standardization of image acquisition and analysis is needed to foster progress.

DOI： 10.1016/j.semarthrit.2022.151954

PubMed

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Language：English   Publishing type：Research paper (scientific journal)  

Progressive multifocal leukoencephalopathy (PML) is a rare opportunistic infection of the central nervous system caused by reactivation of JC virus (JCV). Typical PML shows confluent, bilateral but asymmetric, subcortical lesions in the supratentorial white matter on magnetic resonance imaging (MRI). We report here a 50-year-old woman with systemic lupus erythematosus complicated with lymphoma who developed PML with atypical brain MRI findings limited to the infratentorial area at presentation. She presented with numbness on the right side of the face, including her tongue, clumsiness of the right hand, and gait disturbance, after completion of remission induction therapy for lymphoma, including rituximab. Brain MRI demonstrated a solitary lesion limited to the cerebellum and brainstem, but a definitive diagnosis could not be made from cerebrospinal fluid study or tentative histologic evaluation of brain biopsy specimens. Despite methylprednisolone pulse therapy, her neurological deficits progressively worsened. One month later, in-depth analysis of her cerebrospinal fluid and brain biopsy specimens confirmed the presence of JCV. Eventually, the localised unilateral crescent-shaped cerebellar lesions on MRI expanded to the contralateral cerebellum, middle cerebellar hemisphere, pons, and midbrain and finally developed multifocal invasion into the white matter of the cerebral hemispheres. Our case suggests that PML could first present with a solitary infratentorial lesion in immunocompromised patients.

DOI： 10.1080/24725625.2021.1899763

PubMed

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Publishing type：Research paper (scientific journal)   Publisher：SAGE Publications  

Ultrasound has been widely explored in systemic sclerosis in the clinical and research settings. Ultrasound allows a non-invasive and ionising radiation-free ‘window’ into this complex disease and is well-suited to repeated examinations. Ultrasound provides novel insights into the pathogenesis and measurement of disease in systemic sclerosis, including early (preclinical) internal organ involvement. The purpose of this review is to describe the role of ultrasound to foster clinical and research advancements in systemic sclerosis relating to (1) musculoskeletal, (2) digital ulcer, (3) lung disease and (4) skin disease. We also highlight unmet needs which much be addressed for ultrasound to assume a central role in systemic sclerosis clinical care and research.

DOI： 10.1177/2397198320970394

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Other Link： http://journals.sagepub.com/doi/full-xml/10.1177/2397198320970394

Language：Japanese   Publisher：日本医科大学医学会  

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Language：Japanese   Publisher：日本臨床免疫学会  

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Language：Japanese   Publisher：(一社)日本臨床免疫学会  

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Language：Japanese   Publisher：日本リウマチ学会-関東支部  

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Language：Japanese   Publisher：日本医科大学医学会  

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Language：English  

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Language：Japanese   Publisher：(一社)日本リウマチ学会  

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Language：Japanese   Publisher：(一社)日本リウマチ学会  

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Language：Japanese   Publisher：(一社)日本リウマチ学会  

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Language：Japanese   Publisher：関東リウマチ研究会  

70歳女性。7ヵ月前にANCA関連血管炎性中耳炎(OMAAV)を発症し、ステロイド(PSL)による治療を開始した。治療開始後は難聴の著明な改善を認めたが、PSLの漸減により右眼の視野障害、眼痛が出現した。右球後視神経炎の診断でステロイドパルス、PSL治療を受け症状は改善し、POCYを追加されるも視神経炎が再燃したたため当科入院となった。入院後はPSL、IVCYを行い、症状は一時改善したが、入院4ヵ月後に視神経炎とOMAAVが再燃したため、リツキシマブ投与を4コース行ったところ、症状は改善した。現在、メソトレキセートを追加し、再燃なく経過している。

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Language：Japanese   Publisher：関東リウマチ研究会  

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Language：Japanese   Publisher：関東リウマチ研究会  

症例は26歳女性で、2年前に移動性の単関節炎、1年前に左難聴が出現した。3ヵ月前に有痛性の口内炎が多発、両膝関節痛が増悪し歩行困難となった。その後、褐色尿、鼻出血、四肢の紫斑、眼球結膜充血、咽頭痛が出現し、呼吸困難と喀血がみられたため当院転院となった。病歴でみられた症状と得られた所見を多発血管炎性肉芽腫症(GPA)の診断基準に当てはめると、主要症状、組織所見、検査所見すべてを満たしたため、診断はGPAに半月体形成性糸球体腎炎とび漫性肺胞出血を伴う肺腎症候群と考えられた。まずステロイドパルス療法を行い、血漿交換も併用し、その後VVECMOを装着した。徐々に肺胞出血が改善し、炎症所見やANCAのtiterも低下し、ECMO導入から12日間で離脱した。人工呼吸器からも離脱し、酸素投与を終了した。腎機能障害の著明な改善がみられたため透析を終了し、67日目に退院した。

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：BIOMED CENTRAL LTD  

Background: Some patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) also have positivity of rheumatoid factor (RF). However, the clinical significance of this occurrence remains unknown in AAV patients. The aim of this study was to clarify an association between the presence of RF and clinical features in patients with AAV.
Results: Forty-seven patients diagnosed with AAV who were not complicated with RA were enrolled in this study. We compared clinical manifestations of AAV between an RF-positive subset (n = 29) and an RF-negative subset (n= 18). The Birmingham Vasculitis Activity Score (BVAS) was higher (P = 0.026) in the RF-positive subset than in the RF-negative subset. The levels of CRP and ESR were higher in the RF-positive patients (P = 0.020 and P = 0.007, respectively) compared to the RF-negative subset. IgM-RF titers were significantly correlated with the BVAS (r = 0.50, P = 0.0004). In addition, the IgM-RF titers had significant correlations with the levels of CRP (r= 0.41, P = 0.004), ESR (r = 0.39, P = 0.016), IgM (r = 0.36, P = 0.016) and IgG (r = 0.37, P = 0.015). The frequency of commencement of dialysis therapy, usage of mechanical ventilation and mortality were higher in the RF-positive subset than in the RF-negative subset.
Conclusions: In patients with AAV, RF titers were significantly correlated with disease activity and the levels of inflammatory markers. The presence of RF could be a poor prognostic factor in patients with AAV.

DOI： 10.1186/s12865-017-0234-8

Web of Science

PubMed

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Language：English   Publisher：WILEY  

Web of Science

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Language：English   Publisher：WILEY  

Web of Science

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Language：Japanese   Publisher：日本内科学会-関東地方会  

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Language：Japanese   Publisher：(一社)日本リウマチ学会  

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Language：Japanese   Publisher：(一社)日本リウマチ学会  

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Language：Japanese   Publisher：(一社)日本リウマチ学会  

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Language：Japanese   Publisher：(一社)日本リウマチ学会  

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Language：Japanese   Publisher：(一社)日本リウマチ学会  

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Language：Japanese   Publisher：(一社)日本リウマチ学会  

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Language：Japanese   Publisher：(一社)日本リウマチ学会  

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Language：Japanese   Publisher：(一社)日本リウマチ学会  

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Language：Japanese   Publisher：(一社)日本リウマチ学会  

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Language：Japanese   Publisher：日本内科学会-関東地方会  

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Language：Japanese   Publisher：日本リウマチ学会-関東支部  

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Language：Japanese   Publisher：日本リウマチ学会-関東支部  

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Language：Japanese  

CiNii Books

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Language：Japanese  

J-GLOBAL

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Language：Japanese  

J-GLOBAL

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Language：Japanese  

J-GLOBAL

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Language：Japanese  

J-GLOBAL

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Language：Japanese   Publisher：日本脊椎関節炎学会  

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Language：Japanese   Publisher：日本脊椎関節炎学会  

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Language：Japanese  

J-GLOBAL

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Language：Japanese   Presentation type：Oral presentation (general)  

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Language：Japanese   Presentation type：Poster presentation  

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Language：Japanese  

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Language：Japanese   Presentation type：Oral presentation (general)  

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Language：Japanese   Presentation type：Oral presentation (general)  

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Language：Japanese   Presentation type：Poster presentation  

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Language：Japanese   Presentation type：Oral presentation (general)  

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Language：Japanese   Presentation type：Poster presentation  

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Language：Japanese   Presentation type：Poster presentation  

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Language：English   Presentation type：Poster presentation  

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Language：Japanese   Presentation type：Oral presentation (general)  

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Presentation type：Symposium, workshop panel (nominated)  

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Language：Japanese   Presentation type：Oral presentation (general)  

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Presentation type：Oral presentation (general)  

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Presentation type：Poster presentation  

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Presentation type：Poster presentation  

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Presentation type：Oral presentation (general)  

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Presentation type：Poster presentation  

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