Clinical and experimental rheumatology最新文献

{"title":"The clinical characteristics of pharyngeal and laryngeal lesions in anti-MDA5-positive dermatomyositis patients.","authors":"Wenhan Huang, Feifeng Ren, Dan Deng, Lei Luo, Jun Zhou, Dongmei Huang, Mengxue Tian, Dandan Chen, Kechen Qian, Yanqiu Jiang, Lin Tang","doi":"10.55563/clinexprheumatol/t0478a","DOIUrl":"10.55563/clinexprheumatol/t0478a","url":null,"abstract":"<p><strong>Objectives: </strong>To investigate the clinical characteristics of pharyngeal and laryngeal lesions in patients with anti-melanoma differentiation-associated gene 5 antibodies-positive dermatomyositis (anti-MDA5-positive DM).</p><p><strong>Methods: </strong>Serological indicators of 131 anti-MDA5-positive DM patients were analysed. All 35 patients with pharyngeal and laryngeal symptoms underwent electronic laryngoscopy examinations.</p><p><strong>Results: </strong>Pharyngeal and laryngeal symptoms were observed in 26.7% of anti-MDA5-positive DM patients. Low levels of haemoglobin, albumin, prealbumin, high-density lipoprotein and rapidly progressive interstitial lung disease mainly appeared in patients with pharyngeal and laryngeal involvement compared to those without involvement. However, no significant difference in mortality was found between the two groups. The number of patients with pharyngeal and laryngeal involvement was significantly higher in anti-Ro-52 antibody-positive patients than in anti-Ro-52 antibody-negative patients. Patients with higher serum ferritin levels (1000 ng/ml ≤ serum ferritin ≤ 1500 ng/ml) were more likely to develop pharyngeal and laryngeal involvement compared to those with lower serum ferritin levels (serum ferritin < 500 ng/ml). Electronic laryngoscopy examinations effectively assisted rheumatologists in assessing the conditions of the pharyngeal mucosa, arytenoid area, epiglottis, and vocal cords. Some patients also presented with rare lesions such as pharyngeal posterior wall fistulas, epiglottic ulcers, and vocal cord white lesions.</p><p><strong>Conclusions: </strong>1. Pharyngeal and laryngeal lesions are not uncommon in anti-MDA5-positive DM, these patients have poorer nutritional status and more severe lung lesions; 2. Positive anti-Ro-52 antibodies and high serum ferritin levels are closely associated with pharyngeal and laryngeal involvement in anti-MDA5-positive DM; 3. Electronic laryngoscopy plays a crucial role in the diagnosis and evaluation of pharyngeal and laryngeal conditions.</p>","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":"276-281"},"PeriodicalIF":3.4,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142766651","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Unravelling the link: anti-TIF1γ dermatomyositis associated with plexiform neurofibroma.","authors":"Joshua A Peñafiel-Sam, Iban Aldecoa, José Milisenda, José A Gómez-Puerta","doi":"10.55563/clinexprheumatol/mmvy2o","DOIUrl":"10.55563/clinexprheumatol/mmvy2o","url":null,"abstract":"","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":"391-392"},"PeriodicalIF":3.4,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143188473","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The myositis landscape in clinical trials: 2025 and beyond.","authors":"Giovanni Zanframundo, Veronica Codullo, Lorenzo Cavagna","doi":"10.55563/clinexprheumatol/86an43","DOIUrl":"10.55563/clinexprheumatol/86an43","url":null,"abstract":"","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":"184-185"},"PeriodicalIF":3.4,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143457071","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Interleukin 16: a potential protective cytokine in idiopathic inflammatory myopathy.","authors":"Yueyuan Zhou, Limei Kang, Tao Liu, Geng Yin, Qibing Xie","doi":"10.55563/clinexprheumatol/ma69i6","DOIUrl":"10.55563/clinexprheumatol/ma69i6","url":null,"abstract":"","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":"388-389"},"PeriodicalIF":3.4,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141183671","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Challenges in international investigator-led rare disease clinical trials and the case for optimism in inclusion body myositis.","authors":"Merrilee Needham, Umesh A Badrising, Kelly Beer, Andrew J Heim, Althea Doverty, Annik Panicker, Olivier Benveniste, Mazen M Dimachkie","doi":"10.55563/clinexprheumatol/dyjcsn","DOIUrl":"https://doi.org/10.55563/clinexprheumatol/dyjcsn","url":null,"abstract":"<p><strong>Objectives: </strong>This paper aims to provide insight into the challenges and opportunities of conducting an investigator-led, international, multicentre clinical trial for Inclusion Body Myositis (IBM), a rare inflammatory myopathy.</p><p><strong>Methods: </strong>An international, multicentre, randomised, controlled trial of a repurposed drug (sirolimus) was initiated based on promising results from a mono-centric pilot study. The progress of the trial was analysed to identify key challenges encountered and solutions developed.</p><p><strong>Results: </strong>This large, collaborative study has presented a mosaic of challenges and opportunities, many ubiquitous with investigator-led trials. Key challenges have included securing adequate funding, coordinating manufacture of placebo, negotiating international contracts, managing limited study budgets and delays linked to the COVID-19 pandemic. Alongside these challenges, the study team have found opportunities for creative and effective solutions, including the flexibility of building study databases, optimising digital data capture and harnessing patient involvement.</p><p><strong>Conclusions: </strong>Instrumental to the progress of the trial has been the collaboration between site teams, patient partnership and adaptability.</p>","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":"43 2","pages":"309-315"},"PeriodicalIF":3.4,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143522661","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The diagnostic utility of intima-media thickness measurement compared with the halo sign in temporal artery ultrasonography: a single-centre retrospective study.","authors":"Kazuma Yoshida, Ryuichi M Sada, Akiharu Yoshioka, Hiroyuki Akebo, Hirofumi Miyake, Kazuhiro Hatta","doi":"10.55563/clinexprheumatol/nazkih","DOIUrl":"https://doi.org/10.55563/clinexprheumatol/nazkih","url":null,"abstract":"<p><strong>Objectives: </strong>We aimed to examine the diagnostic utility of temporal artery ultrasonography (TAUS) based on measurement of intima-media thickness (IMT) compared with the halo sign in diagnosing cranial giant cell arteritis (GCA).</p><p><strong>Methods: </strong>We retrospectively analysed consecutive patients with clinically suspected GCA who had undergone TAUS between January 2011 and December 2021 in Tenri hospital, Japan. A cut-off value of 0.5 mm was used for the IMT of the temporal arteries. We examined the diagnostic value of TAUS based on each of the halo sign and increased IMT in diagnosing cranial GCA.</p><p><strong>Results: </strong>In total, 203 patients were included. Temporal artery biopsy (TAB) was performed in 59 patients, with 32 being biopsy-positive. Fifty-three patients were diagnosed with cranial GCA. The sensitivity and specificity of TAUS based on the increased IMT and halo sign were as follows: sensitivity, 62.3% and 32.1%; specificity, 90.0% and 100% compared with the clinical diagnosis; and sensitivity, 81.2% and 46.9%; specificity, 76.9% and 96.2% compared with the TAB. When the relationship between the IMT and halo sign was evaluated, patients with cranial GCA who presented with the halo sign had the highest IMT compared with those without the halo sign or those without cranial GCA.</p><p><strong>Conclusions: </strong>A TAUS diagnosis relying only on the halo sign is specific but can underestimate cranial GCA. Therefore, evaluation of the IMT in addition to the halo sign can improve the diagnostic accuracy of TAUS when diagnosing cranial GCA.</p>","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":""},"PeriodicalIF":3.4,"publicationDate":"2025-01-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143188472","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"How to prescribe rituximab wisely for antineutrophil cytoplasmic antibody-associated vasculitides.","authors":"Loïc Guillevin","doi":"10.55563/clinexprheumatol/b1ec25","DOIUrl":"https://doi.org/10.55563/clinexprheumatol/b1ec25","url":null,"abstract":"","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":""},"PeriodicalIF":3.4,"publicationDate":"2025-01-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143188467","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Severe lung damage in anti-RNPC-3 antibody-positive patients: expanding the spectrum beyond systemic sclerosis.","authors":"Yasuhiko Yamano, Yoshinao Muro, Haruka Koizumi, Masashi Akiyama, Tomoki Kimura, Yasuhiro Kondoh","doi":"10.55563/clinexprheumatol/5o45p1","DOIUrl":"https://doi.org/10.55563/clinexprheumatol/5o45p1","url":null,"abstract":"","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":""},"PeriodicalIF":3.4,"publicationDate":"2025-01-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143188470","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Systemic lupus erythematosus-related macrophage activation syndrome inducing severe cytopenia successfully treated with anifrolumab: a case report.","authors":"Emanuele Chiara, Michela Gasparotto, Danilo Malandrino, Edoardo Biancalana, Maria Letizia Uban, Elena Silvestri, Giacomo Emmi","doi":"10.55563/clinexprheumatol/uumehz","DOIUrl":"https://doi.org/10.55563/clinexprheumatol/uumehz","url":null,"abstract":"","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":""},"PeriodicalIF":3.4,"publicationDate":"2025-01-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143188471","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Clinical outcomes in cancer patients with immune checkpoint inhibitor-induced arthritis treated with methotrexate: a retrospective longitudinal monocentric pilot study.","authors":"Elvis Hysa, Andrea Casabella, Nicola Iandolino, Emanuele Gotelli, Carlo Genova, Enrica Teresa Tanda, Carmen Pizzorni, Vanessa Smith, Alberto Sulli, Maurizio Cutolo, Sabrina Paolino","doi":"10.55563/clinexprheumatol/e49am5","DOIUrl":"https://doi.org/10.55563/clinexprheumatol/e49am5","url":null,"abstract":"<p><strong>Objectives: </strong>Immune-mediated adverse events (irAEs) from immune checkpoint inhibitors (ICIs) often require high-dose glucocorticoids (GCs), which can promote cancer progression and counteract ICI benefits. This study evaluated the articular and oncologic clinical outcomes of ICI-induced arthritis treated with methotrexate (MTX) as a GC-sparing agent.</p><p><strong>Methods: </strong>Adult patients with ICI-induced arthritis in 2023 were included. Arthritis was assessed using the disease activity score on 28 joints by C-reactive protein (DAS28-CRP), with follow-ups every 3 months. All patients received subcutaneous MTX, and oncologic outcomes were evaluated using RECIST 1.1 criteria after one year.</p><p><strong>Results: </strong>Fourteen patients (median age 74.5 years) with melanoma (64.3%), colorectal cancer (14.3%), lung cancer (14.3%), or Hodgkin's lymphoma (7.1%) were treated with PD1 antagonists (92.9%) or combined with CTLA4 blockers (7.1%). Arthritis presentations included oligo-arthritis (36%), mono-arthritis (29%), polyarthritis (21%), and polymyalgia rheumatica-like syndrome (14.3%), with a mean onset of 4.7±3.7 months post-ICI. MTX was started for all at a mean dose of 9.5±1.5 mg weekly, beginning at the first rheumatology visit in 78.5% of patients. Over a mean follow-up of 12.8±4.6 months, DAS28-CRP scores improved significantly, and prednisone dosage was in all reduced (3.6 mg at V4 vs. 8.4 mg at V0, p=0.003). No major MTX-related toxicities were noted. Cancer responses at follow-up were complete (50%), partial (21.4%), stable disease (7.1%), and progression (21.5%).</p><p><strong>Conclusions: </strong>The use of MTX in ICI-induced arthritis showed promising results in reducing GC dosages and managing the inflammatory articular activity, with no major toxicities observed over one year. These findings suggest that MTX may be a viable GC-sparing option in this context, but larger, controlled studies are needed to confirm these observations and better understand the impact on both articular and oncologic outcomes.</p>","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":""},"PeriodicalIF":3.4,"publicationDate":"2025-01-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143188353","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}