{"title":"Comparison of the efficacy of Janus kinase inhibitors and adalimumab in rheumatoid arthritis: a meta-analysis.","authors":"Qiang Zhou, Sidong Zhu, Senjie Dai, Qingping Wu, Jiahao Zheng, Hao Zhu, Weifeng Yang","doi":"10.55563/clinexprheumatol/4g8g9q","DOIUrl":"https://doi.org/10.55563/clinexprheumatol/4g8g9q","url":null,"abstract":"<p><strong>Objectives: </strong>Rheumatoid arthritis (RA) is a prevalent autoimmune disorder. This study examines the comparative efficacy of Janus kinase inhibitors (JAKi) and adalimumab (ADA) in managing RA.</p><p><strong>Methods: </strong>As of May 2024, four electronic databases were systematically reviewed: PubMed, Web of Science, Embase, and the Cochrane Library. Data were analysed using Review Manager (RevMan) software. The risk ratio (RR) and its 95% confidence interval (CI) represented dichotomous outcomes. Evaluated outcome measures included ACR20, ACR50, ACR70, Clinical Disease Activity Index (CDAI), Simplified Disease Activity Index (SDAI), and Disease Activity Score 28-4 (C-reactive protein) (DAS28-4(CRP)).</p><p><strong>Results: </strong>The analysis encompassed 6 studies, totalling 4048 patients with RA. There was no statistically significant difference in efficacy between JAKi and ADA when assessing ACR20 (p=0.25) and DAS28-4(CRP) (p=0.57). However, JAKi demonstrated superior efficacy compared to ADA for ACR50 (RR=1.20; p=0.02), ACR70 (RR=1.24; p=0.03), CDAI (RR=1.17; p=0.01), and SDAI (RR=1.19; p=0.006) outcomes. Longitudinal analysis revealed that over a 52-week period, JAKi did not exhibit superior efficacy to ADA for ACR50 (RR=1.16; p=0.19) and ACR70 (RR=1.10; p=0.26). Specifically, the tofacitinib subgroup outperformed ADA (RR=1.49; p=0.003), while other JAKi treatments did not show a significant difference (RR=1.19; p=0.11) compared to ADA.</p><p><strong>Conclusions: </strong>JAKi generally offers better efficacy than ADA in the treatment of RA, though this advantage appears to be influenced by the duration of treatment.</p>","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":""},"PeriodicalIF":3.4,"publicationDate":"2025-02-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143457042","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}
Ömer Şevgin, Burak Buğday, Rabia Aydoğan Baykara, Gizem Günendi, Burcu Akkurt, Hilal Atasoy, Beyzanur Dikmen Hoşbaş, Mustafa Ferit Akkurt, Berna Karamancıoğlu
{"title":"Efficacy of different exercises in women with fibromyalgia syndrome: a randomised controlled trial.","authors":"Ömer Şevgin, Burak Buğday, Rabia Aydoğan Baykara, Gizem Günendi, Burcu Akkurt, Hilal Atasoy, Beyzanur Dikmen Hoşbaş, Mustafa Ferit Akkurt, Berna Karamancıoğlu","doi":"10.55563/clinexprheumatol/uol8so","DOIUrl":"https://doi.org/10.55563/clinexprheumatol/uol8so","url":null,"abstract":"<p><strong>Objectives: </strong>The objective of this study was to evaluate the impact of aerobic exercise, resistance exercise combined with aerobic exercise, and yoga exercises combined with aerobic exercise on pain and disease activity in patients with fibromyalgia syndrome (FM).</p><p><strong>Methods: </strong>The study population comprised 60 individuals with FM who met the inclusion criteria. The participants were randomly assigned to one of three groups. The first group underwent aerobic exercise (n=20), the second group combined aerobic exercise with yoga (n=20), and the third group engaged in aerobic and resistance exercise (n=20). All exercise interventions were conducted for a total of 12 weeks. Disease activity was evaluated using the Fibromyalgia Impact Questionnaire (FIQ), while pain status was assessed with the Melzack-Melzack Pain Questionnaire (MMPQ). All assessments were conducted before and following the completion of the exercise program. The clinical trial number of this study is NCT06006494.</p><p><strong>Results: </strong>The measurements of the aerobic exercise and yoga group were significantly lower than those of the aerobic and resistance exercise group. A statistically significant difference was observed between the groups in terms of post-treatment MMPQ scores. The measurements of the aerobic exercise and yoga group were significantly lower than those of the aerobic exercise only and aerobic and resistance exercise groups. No statistically significant difference was observed between the post-treatment MMPQ scores of the aerobic and aerobic resistance exercise groups.</p><p><strong>Conclusions: </strong>The combination of aerobic exercise and yoga is more efficacious in the treatment of FM than aerobic exercise alone or a combination of resistance exercises and aerobic exercise.</p>","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":""},"PeriodicalIF":3.4,"publicationDate":"2025-02-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143406023","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":"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}
Darcisio Hortelan Antonio, Beatriz O L Carneiro, Taciana A P Fernandes, Adriana M Elias, Ana Julia Pantoja de Moraes, Ana Paula Vecchi, Andre Cavalcanti, Carlos N Rabelo, Cristina M Magalhaes, Flavio R Sztajnbok, Luciana M Carvalho, Luciana Paim Marques, Maria Teresa Terreri, Melissa M Fraga, Sheila K F de Oliveira, Silvana B Sacchetti, Simone Appenzeller, Teresa Robazzi, Virginia P L Ferriani, Claudio A Len, Clovis A A Silva, Claudia Saad-Magalhaes
{"title":"Longitudinal assessment of disease activity and muscle strength in juvenile dermatomyositis: a multicentre registry study.","authors":"Darcisio Hortelan Antonio, Beatriz O L Carneiro, Taciana A P Fernandes, Adriana M Elias, Ana Julia Pantoja de Moraes, Ana Paula Vecchi, Andre Cavalcanti, Carlos N Rabelo, Cristina M Magalhaes, Flavio R Sztajnbok, Luciana M Carvalho, Luciana Paim Marques, Maria Teresa Terreri, Melissa M Fraga, Sheila K F de Oliveira, Silvana B Sacchetti, Simone Appenzeller, Teresa Robazzi, Virginia P L Ferriani, Claudio A Len, Clovis A A Silva, Claudia Saad-Magalhaes","doi":"10.55563/clinexprheumatol/yrb7m2","DOIUrl":"10.55563/clinexprheumatol/yrb7m2","url":null,"abstract":"<p><strong>Objectives: </strong>To define disease activity measures, muscle strength and functional assessments in new-onset juvenile dermatomyositis (JDM) patients, at disease onset and follow up.</p><p><strong>Methods: </strong>A registry was set up in 18 hospitals, enrolling patients over 3-years (2015-2018). Clinical assessments were performed at baseline, and at 6, 12, 18 and 24 months after diagnosis. Disease Activity Score (DAS20), skin and musculoskeletal DAS sub-scales; Manual Muscle Test (MMT8); Childhood Myositis Assessment Scale (CMAS); Childhood Health Assessment Questionnaire disability index (CHAQ_DI 0-3) and 10 cm Visual Analog Scale (VAS) for overall wellbeing scores were compared by Poisson Model and Wald post-test for repeated measures.</p><p><strong>Results: </strong>Ninety-six cases, being 61 (64%) females, median age 10 years had JDM diagnosis and 12 (13%) onset calcinosis. Mean ±SD scores at diagnosis and 6 months intervals for DAS20 (0-20) were 7.8±5, 6.3 ±4.8, 5±4, 4.9 ±5 and 0.5 ±2.3; with significant difference from baseline (p<0.01). Skin DAS subscales were 2.8±3.3, 1.8±2.9, 1,1±2.2, 0.6±1.8, 0.4±1.5. MMT (0-80) 62.6±20.4, 70.2±13.5, 73.3±11, 75.7±7.9 and 74.8±7.8, with significant difference from baseline up to 6 months (p=0.016); CMAS (0-53) 29.5±11.4, 33.1±8.3, 34.2±5.8, 34±6 and 33.3±5.4. CHAQ-DI (0-3) 1±0.9, 0.6±0.7, 0.8±0.8, 1±0.8 and 1±0.3; parents VAS 4.1±2.5, 2±2.1; 1.3±2.8, 4.1±3.1, 1.7±2.2. There was no significant difference for CMAS, CHAQ-DI and parents VAS from baseline up to 24-month assessment.</p><p><strong>Conclusions: </strong>DAS20 scores improved gradually during follow up, MMT8 improved significantly during the first 6 months and CMAS, CHAQ-DI and parents VAS scores had no significant improvement with persistent functional impairment over 2-years.</p>","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":"379-386"},"PeriodicalIF":3.4,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142364611","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}
Michael Hauke, Mari Kamiya, Conrad Chris Weihl, Jens Schmidt
{"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":"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":"345-353"},"PeriodicalIF":3.4,"publicationDate":"2025-02-01","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}
Chiara Cardelli, Francesca Trentin, Federico Fattorini, Michele Diomedi, Elenia Laurino, Simone Barsotti, Marta Mosca, Linda Carli
{"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":"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":"186-192"},"PeriodicalIF":3.4,"publicationDate":"2025-02-01","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}
Sharfaraz Salam, Jasper M Morrow, Sachit Shah, Michael G Hanna, Mazen M Dimachkie, Pedro M Machado
{"title":"Imaging evaluation of the upper limbs in inclusion body myositis: an unmet need.","authors":"Sharfaraz Salam, Jasper M Morrow, Sachit Shah, Michael G Hanna, Mazen M Dimachkie, Pedro M Machado","doi":"10.55563/clinexprheumatol/q7ozm2","DOIUrl":"10.55563/clinexprheumatol/q7ozm2","url":null,"abstract":"<p><p>Inclusion body myositis (IBM) is an acquired myopathy belonging to the spectrum of idiopathic inflammatory myopathies. It commonly presents in individuals aged above 50 years of age. Characteristic clinical features of IBM include weakness of the quadriceps and finger flexors. There are currently no effective drug treatments for IBM. However as more clinical drug trials are being conducted it is important that more precise outcome measures are developed to track disease progression and assess treatment effects. Imaging techniques such as magnetic resonance imaging (MRI) and ultrasound have been increasingly used to study intramuscular changes within the thigh and calf muscles. In particular quantitative MRI assessments of the lower limb have started to be employed as endpoints for clinical trials in IBM patients. However, in comparison to the lower limb, there is a relative lack in robust imaging biomarkers for the upper limb muscles. It is prudent that this paucity is addressed as the majority of IBM patients have forearm involvement and, in many individuals, upper limb weakness is their main source of disability. Imaging focussed studies thus far indicate preferential flexor digitorium profundus involvement. In this review, we discuss the imaging modalities that have been used to evaluate intramuscular changes and the possible techniques which could be developed further as upper limb biomarkers for IBM.</p>","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":"372-378"},"PeriodicalIF":3.4,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143457050","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}
Weijin Zhang, Guohai Huang, Shaoyu Zheng, Jianqun Lin, Shijian Hu, Jinghua Zhuang, Zexuan Zhou, Guangzhou Du, Kedi Zheng, Shaoqi Chen, Qichuan Zhang, Angelina Mikish, Anna-Maria Hoffmann-Vold, Masataka Kuwana, Marco Matucci-Cerinic, Daniel E Furst, Yukai Wang
{"title":"Risk prediction modelling in idiopathic inflammatory myositis-associated interstitial lung disease based on seven factors including serum KL-6 and lung ultrasound B-lines.","authors":"Weijin Zhang, Guohai Huang, Shaoyu Zheng, Jianqun Lin, Shijian Hu, Jinghua Zhuang, Zexuan Zhou, Guangzhou Du, Kedi Zheng, Shaoqi Chen, Qichuan Zhang, Angelina Mikish, Anna-Maria Hoffmann-Vold, Masataka Kuwana, Marco Matucci-Cerinic, Daniel E Furst, Yukai Wang","doi":"10.55563/clinexprheumatol/ylf0oe","DOIUrl":"10.55563/clinexprheumatol/ylf0oe","url":null,"abstract":"<p><strong>Objectives: </strong>To develop a user-friendly nomogram-based predictive model for interstitial lung disease (ILD) in patients with idiopathic inflammatory myositis (IIM).</p><p><strong>Methods: </strong>A retrospective study was conducted at Shantou Central Hospital, encompassing 205 IIM patients diagnosed between January 2013 and December 2022. We used the LASSO regression method in the discovery set to select features for model construction, followed by efficacy verification through AUC of ROC. Afterwards, KL-6 values and LUS B-lines number were added into this model to evaluate whether these 2 factors added to the model efficiency. Finally, a web version was constructed to make it more available.</p><p><strong>Results: </strong>Among the 205 IIM patients, 115 (56.1%) patients were diagnosed with ILD, and 90 (43.9%) did not. The predictive model, derived from the training set, comprised four independent risk factors, including age, presence of respiratory symptoms, anti-melanoma differentiation-associated gene 5 (MDA-5) antibody positivity, and anti-aminoacyl transfer RNA synthetase (anti-ARS) antibodies positivity. Notably, anti-TIF1-γ antibody positivity emerged as a protective factor. The AUC of the ROC based on these 5 factors was 0.876 in the training set and 0.861 in the validation set. The AUC of the ROC based on the 5 factors plus KL-6 was 0.922, 5 factors plus B-line number was 0.949 and 5 factors plus both KL-6 and B-line number was 0.951. Accordingly, a nomogram and a web version were developed.</p><p><strong>Conclusions: </strong>This predictive model demonstrates robust capability to assess ILD risk in IIM patients, particularly when augmented with serum KL-6 level or/and LUS B-line number.</p>","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":" ","pages":"260-268"},"PeriodicalIF":3.4,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142364613","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}
Aleksandra H Opinc-Rosiak, Ewelina M Kulesza, Agnieszka J Mikosińska, Olga E Brzezińska, Joanna S Makowska
{"title":"Infections as a predominant cause of death in adult patients with idiopathic inflammatory myopathies.","authors":"Aleksandra H Opinc-Rosiak, Ewelina M Kulesza, Agnieszka J Mikosińska, Olga E Brzezińska, Joanna S Makowska","doi":"10.55563/clinexprheumatol/kcdmct","DOIUrl":"https://doi.org/10.55563/clinexprheumatol/kcdmct","url":null,"abstract":"<p><strong>Objectives: </strong>To evaluate causes of death in the single-centre Polish cohort of patients with idiopathic inflammatory myopathies (IIM) and to identify risk factors associated with fatal outcomes.</p><p><strong>Methods: </strong>Electronic medical database was retrospectively analysed, data on the clinical symptoms, serological profiles, administered treatment and outcomes in IIM patients were collected. Two subgroups were distinguished - the deceased and the survived group. Statistical analysis was performed to identify differences between the subgroups and risk factors contributing to fatal outcomes.</p><p><strong>Results: </strong>79 patients with IIM were identified, most frequently with antisynthetase syndrome, dermatomyositis and polymyositis. Among them 9 patients (11.39%) deceased. The mean age at the time of diagnosis was 57.10 ± 14,59years old. Median diagnostic delay reached 5 months. The majority of the deaths (77.78%) occurred within the first 18 months after IIM diagnosis. As compared to the survivors, patients with fatal outcomes were more frequently affected by cardiac involvement (p=0.026), suffered from concomitant autoimmune disorders (p=0.028) were treated with mycophenolate mofetil (p=0.005) and cyclophosphamide (p=0.006). 89.89% of all deaths in our cohort were caused by various infections, predominantly of the respiratory tract. Although 10.13% of the patients had a history of malignancy, none of the deaths was caused by malignancy.</p><p><strong>Conclusions: </strong>Patients with IIM associated with cardiac involvement and concomitant autoimmune diseases may be at higher risk of fatal outcomes. Infections were the predominant cause of death in our cohort of patients. The majority of fatal outcomes occurred at the relatively early stage of the disease.</p>","PeriodicalId":10274,"journal":{"name":"Clinical and experimental rheumatology","volume":"43 2","pages":"290-299"},"PeriodicalIF":3.4,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143522665","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}