Clinical and experimental rheumatology最新文献

{"title":"Environmental triggers for idiopathic inflammatory myopathies: unravelling the known and unknown.","authors":"Chiara Cardelli, Francesca Trentin, Federico Fattorini, Michele Diomedi, Elenia Laurino, Simone Barsotti, Marta Mosca, Linda Carli","doi":"10.55563/clinexprheumatol/0x1gob","DOIUrl":"https://doi.org/10.55563/clinexprheumatol/0x1gob","url":null,"abstract":"<p><p>Idiopathic inflammatory myopathies (IIM) are a heterogeneous group of rare connective tissues diseases that usually share the common feature of immune-mediated muscle or lung injury. Whereas their pathogenesis is widely recognised as multifactorial, the specific triggers initiating their onset remain largely elusive. Factors such as infections, inhalants, or geoclimatic variables are implicated, yet due to the limitations inherent in studies involving small and non-homogeneous cohorts, findings often appear fragmented and inconclusive. This review endeavours to present the most updated evidence regarding the influence of environmental factors in determining the onset of IIM, with the aim of offering insight to optimise the routinary management of affected patients.</p>","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":""},"PeriodicalIF":3.4,"publicationDate":"2025-02-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143188466","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":"Different phenotypic manifestations between Brazilian and Japanese anti-MDA5 antibody-positive dermatomyositis: an international tricentric longitudinal study.","authors":"Marlise S M S Faria, Akira Yoshida, Naoki Mugii, Pleiades T Inaoka, Takashi Matsushita, Takahisa Gono, Masataka Kuwana, Samuel K Shinjo","doi":"10.55563/clinexprheumatol/9s7djz","DOIUrl":"https://doi.org/10.55563/clinexprheumatol/9s7djz","url":null,"abstract":"<p><strong>Objectives: </strong>Anti-MDA5 autoantibodies are strongly associated with interstitial lung disease (ILD) and rapidly progressive ILD (RP-ILD) in Asian patients with dermatomyositis (DM) or amyopathic DM (ADM). However, this association has not yet been established in Brazilian patients with anti-MDA5(+) DM/ADM. This study aimed to investigate the phenotypic differences between Brazilian and Japanese patients with anti-MDA5(+) DM/ADM, with a particular focus on ILD.</p><p><strong>Methods: </strong>This was an international, tricentric, retrospective cohort study conducted in one Brazilian and two Japanese tertiary centres. Patients diagnosed with anti-MDA5(+) DM/ADM at the three centres were enrolled. Clinical characteristics and outcomes were collected using a pre-standardised protocol and compared between Brazilian and Japanese patients.</p><p><strong>Results: </strong>Thirty-four Brazilian and 65 Japanese patients were analysed. Brazilian patients were younger at the time of diagnosis than Japanese patients. The prevalence of muscle weakness, myalgia, dysphagia, heliotrope rash, V-sign, calcinosis, Raynaud's phenomenon, and digital ulcers was higher in Brazilian patients, whereas mechanic's hands were more prevalent in Japanese patients. The prevalence of ILD was significantly lower in Brazilian patients than in Japanese patients (50.0% vs. 98.5%, P<0.001). RP-ILD was observed in 34 (52.3%) Japanese patients and in only one (3.3%) Brazilian patient (P<0.001). Outcomes including overall survival and the frequency of relapses and complications, such as severe infection and malignancy, were comparable between the two populations.</p><p><strong>Conclusions: </strong>Brazilian patients with anti-MDA5(+) DM/ADM had a higher prevalence of skin and muscle involvement, whereas the prevalence of ILD and RP-ILD was significantly lower than in Japanese patients.</p>","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":""},"PeriodicalIF":3.4,"publicationDate":"2025-02-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143188463","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":"Correlations between IgG4-related disease and allergic reactions: implications for future therapeutic strategies.","authors":"Difei Lian, Qiyuan Hao, Yanying Liu","doi":"10.55563/clinexprheumatol/2g7num","DOIUrl":"https://doi.org/10.55563/clinexprheumatol/2g7num","url":null,"abstract":"<p><p>IgG4-related disease (IgG4-RD) is a chronic multi-organ immune fibroinflammatory disorder. It can affect almost any organ, with the primary treatment being corticosteroids, sometimes supplemented with conventional immunosuppressants or biological agents, such as rituximab therapy. The occurrence of this disease is associated with aberrant adaptive immune responses, but its specific pathological mechanisms remain unclear. Patients with IgG4-RD often have allergic diseases such as asthma, rhinitis, and urticaria.Allergic reactions and IgG4-RD may share similar pathological mechanisms, including activation of Th2 immune responses, excessive secretion of IgG4 and IgE, and increased blood/tissue eosinophils. The aim of this article is to review the allergy-like characteristics of IgG4-RD and emphasise the potential of allergy-targeted therapies in the treatment of IgG4-RD patients.</p>","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":""},"PeriodicalIF":3.4,"publicationDate":"2025-02-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143188354","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":"C-reactive protein thresholds for discriminating active disease in axial spondyloarthritis: should we lower them?","authors":"Sara Alonso-Castro, Pablo González Del Pozo, Paula Alvarez, Norma Calleja, Stefanie Burger, Rubén Queiro","doi":"10.55563/clinexprheumatol/lelu7p","DOIUrl":"https://doi.org/10.55563/clinexprheumatol/lelu7p","url":null,"abstract":"<p><strong>Objectives: </strong>Although C-reactive protein (CRP) is the main inflammatory biomarker used for axial spondyloarthritis (axSpA) assessment, its sensitivity for detecting active disease is low. We aimed to analyse CRP thresholds capable of discriminating across disease activity states in axSpA.</p><p><strong>Methods: </strong>Two hundred consecutive patients with axSpA were recruited (with/without biologics). Discriminative CRP thresholds were determined by the Youden index, while their sensitivity/specificity was evaluated by the area under the ROC curve (AUROC). Sensitivity analyses were performed based on exposure to biologic drugs.</p><p><strong>Result: </strong>One hundred and twenty-two men and 78 women were included, mean age 43.5±11.6 years, mean age at diagnosis 34±10.7 years, average disease duration 8.6±6.5 years. Median CRP 0.20 mg/dl (IQR 0.10-0.40). A CRP ≥0.25 mg/dl discriminated the population with high/very high disease activity [AUROC 0.71; OR 3.9, p<0.001]. This threshold rose to CRP ≥0.35 mg/dl [AUROC 0.72; OR 10.4, p<0.001] among patients without biological therapy, remaining at ≥0.25 mg/dl [AUROC 0.70; OR 4.02, p<0.001] in those exposed to these therapies. The standard inflammatory CRP value (≥0.5 mg/dl) was highly specific (0.90) but poorly sensitive (0.35) in detecting high/very high disease activity states, making it less discriminatory than previous thresholds.</p><p><strong>Conclusions: </strong>Our results suggest adopting lower than standard CRP cut-off values for a better assessment of both the global activity of the disease and a better interpretation of the composite activity indices used in axSpA.</p>","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":""},"PeriodicalIF":3.4,"publicationDate":"2025-02-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143188355","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":"Disease models of myositis: overview of cell culture and rodent systems.","authors":"Michael Hauke, Mari Kamiya, Conrad Chris Weihl, Jens Schmidt","doi":"10.55563/clinexprheumatol/4yp71i","DOIUrl":"https://doi.org/10.55563/clinexprheumatol/4yp71i","url":null,"abstract":"<p><p>Inflammatory myopathies (in short: myositis) display a heterogenic group of rare inflammatory diseases of the skeletal muscle and other organs such as lung, heart and skin. Patients typically display muscular weakness, wasting and a variable response to treatment. The pathogenesis involves invasion of muscle fibres by mononuclear cells and deposition of autoantibodies. In vitro and in vivo models are crucial to understand the so far unresolved complex network of pathomechanisms and how to design future treatment strategies. So far, no model can represent all features of the human disease, but each facilitates analysis of distinct mechanisms of the disease. A range of different in vitro and in vivo models have been developed in recent years to functionally study myositis pathology. This review provides an overview of muscle cell culture systems and transgenic as well as inducible animal models that each represent distinct features of myositis.</p>","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":""},"PeriodicalIF":3.4,"publicationDate":"2025-02-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143188464","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 characteristics and clinical risk model of the lower gastrointestinal involvement in systemic sclerosis.","authors":"Shihan Xu, Haochen Huang, Jiaxin Zhou, Mengtao Li, Xiaofeng Zeng, Hong Yang, Dong Xu","doi":"10.55563/clinexprheumatol/nhp9h9","DOIUrl":"https://doi.org/10.55563/clinexprheumatol/nhp9h9","url":null,"abstract":"<p><strong>Objectives: </strong>Systemic sclerosis (SSc) is a systemic autoimmune disease that could involve multiple organs. The lower gastrointestinal involvement (LGIT) in systemic sclerosis (SSc-LGIT) is a serious manifestation and has a poor prognosis. This study aims to explore the related risk factors of SSc-LGIT that require hospitalisation and build a clinical risk model.</p><p><strong>Methods: </strong>SSc patients with LGIT admitted to Peking Union Medical College Hospital (PUMCH) were enrolled between December 2003 and December 2023. The controls were selected in SSc patients without LGIT in the same period after matching age and gender at a ratio of 1:3. Clinical data of both groups was collected to build the SSc-LGIT clinical prediction model by machine learning using R software.</p><p><strong>Results: </strong>A total of 42 SSc patients with LGIT and 126 matched SSc patients without LGIT were enrolled. Compared to the control group, SSc-LGIT patients had lower level of body mass index (BMI), haemoglobin (HB), albumin (ALB). Cardiomyopathy and puffy finger were more common, but arthritis/arthralgia was less. Higher hs-CRP and a higher rate of anti-Ro-52 antibody positivity were found in these patients. A multivariate analysis revealed BMI, cardiomyopathy, HB, ALB, hs-CRP as independent related factors for SSc-LGIT, and a clinical risk model containing these five items was built.</p><p><strong>Conclusions: </strong>A clinical risk model of SSc-LGIT was established and it has demonstrated capability in predicting the risk of severe lower gastrointestinal involvement in systemic sclerosis.</p>","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":""},"PeriodicalIF":3.4,"publicationDate":"2025-02-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143188351","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":"Predictive and diagnostic biomarkers for cytomegalovirus infection in patients with rheumatic musculoskeletal diseases treated by high-dose glucocorticoid therapy: multicentre, prospective cohort study.","authors":"Hirohiko Sueki, Kazuteru Noguchi, Bunki Natsumoto, Keishi Fujio, Yutaro Hayashi, Yuko Kaneko, Takahisa Gono, Kuninobu Wakabayashi, Haruka Ito, Takahiko Yoshimoto, Akatsuki Kokaze, Takemi Otsuki, Yurika Shimizu, Tatsuo Ito, Koh Okamoto, Shu Okugawa, Kyoji Moriya, Kiyoshi Matsui","doi":"10.55563/clinexprheumatol/th30zv","DOIUrl":"https://doi.org/10.55563/clinexprheumatol/th30zv","url":null,"abstract":"<p><strong>Objectives: </strong>The incidence of cytomegalovirus (CMV) infection or disease in patients with rheumatic musculoskeletal diseases is reported to be 2%. Over half received pulsed methylprednisolone, and some experienced a fatal outcome. In this study, we aimed to explore predictive and diagnostic biomarkers for CMV infection or disease in such patients and compare them with biomarkers reported for immune reconstitution inflammatory syndrome (IRIS) in people with HIV.</p><p><strong>Methods: </strong>In this multicentre prospective cohort study, we collected blood and saliva samples from 38 patients with rheumatic musculoskeletal disease before initiating high-dose glucocorticoid therapy, at the start of glucocorticoid tapering, at the onset of CMV infection, and 4 weeks later. Peripheral blood cell counts, flow cytometry for CD4, CD8, and Tregs, ELISA for cytokine/chemokine panels, and measurements of herpesvirus-derived DNA in saliva were performed.</p><p><strong>Results: </strong>Lower white blood cells, CD4+ cells, IL-6, and interferon-γ levels and higher interferon-inducible protein (IP)-10 and granulysin levels at baseline could be predictive biomarkers for CMV infection. Furthermore, lower platelet counts and higher IL-10, IP-10, granulysin, TNF-a, IL-1ra, and IL-15 levels at the onset of CMV infection were found as diagnostic biomarkers for CMV infection. EBV, human herpes virus (HHV)-6, and HHV-7 DNA levels in the saliva were significantly increased after high-dose glucocorticoids, regardless of CMV infection.</p><p><strong>Conclusions: </strong>We identified predictive and diagnostic biomarkers for CMV infection after high-dose glucocorticoid therapy for rheumatic musculoskeletal diseases. While similarities with IRIS biomarkers in patients living with HIV were observed, complete agreement could not be confirmed.</p>","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":"143188469","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}