Advances in Rheumatology最新文献

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Unveiling the clinical spectrum of relapsing polychondritis: insights into its pathogenesis, novel monogenic causes, and therapeutic strategies 揭示复发性多软骨炎的临床表现:对其发病机制、新的单基因病因和治疗策略的见解
IF 2.3 4区 医学
Advances in Rheumatology Pub Date : 2024-04-16 DOI: 10.1186/s42358-024-00365-z
Blanca E R G Bica, Alexandre Wagner S de Souza, Ivânio Alves Pereira
{"title":"Unveiling the clinical spectrum of relapsing polychondritis: insights into its pathogenesis, novel monogenic causes, and therapeutic strategies","authors":"Blanca E R G Bica, Alexandre Wagner S de Souza, Ivânio Alves Pereira","doi":"10.1186/s42358-024-00365-z","DOIUrl":"https://doi.org/10.1186/s42358-024-00365-z","url":null,"abstract":"Relapsing polychondritis is a rare multisystem disease involving cartilaginous and proteoglycan-rich structures. The diagnosis of this disease is mainly suggested by the presence of flares of inflammation of the cartilage, particularly in the ears, nose or respiratory tract, and more rarely, in the presence of other manifestations. The spectrum of clinical presentations may vary from intermittent episodes of painful and often disfiguring auricular and nasal chondritis to an occasional organ or even life-threatening manifestations such as lower airway collapse. There is a lack of awareness about this disease is mainly due to its rarity. In 2020, VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome, a novel autoinflammatory syndrome, was described. VEXAS syndrome is attributed to somatic mutations in methionine-41 of UBA1, the major E1 enzyme that initiates ubiquitylation. This new disease entity connects seemingly unrelated conditions: systemic inflammatory syndromes (relapsing chondritis, Sweet’s syndrome, and neutrophilic dermatosis) and hematologic disorders (myelodysplastic syndrome or multiple myeloma). Therefore, this article reviews the current literature on both disease entities.","PeriodicalId":48634,"journal":{"name":"Advances in Rheumatology","volume":"74 1","pages":""},"PeriodicalIF":2.3,"publicationDate":"2024-04-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140614377","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}
引用次数: 0
Hemophagocytic lymphohistiocytosis and macrophage activation syndrome: two rare sides of the same devastating coin 嗜血细胞淋巴组织细胞增多症和巨噬细胞活化综合征:同一枚毁灭性硬币的罕见两面
IF 2.3 4区 医学
Advances in Rheumatology Pub Date : 2024-04-16 DOI: 10.1186/s42358-024-00370-2
Flavio Sztajnbok, Adriana Rodrigues Fonseca, Leonardo Rodrigues Campos, Kátia Lino, Marta Cristine Félix Rodrigues, Rodrigo Moulin Silva, Rozana Gasparello de Almeida, Sandro Félix Perazzio, Margarida de Fátima Fernandes Carvalho
{"title":"Hemophagocytic lymphohistiocytosis and macrophage activation syndrome: two rare sides of the same devastating coin","authors":"Flavio Sztajnbok, Adriana Rodrigues Fonseca, Leonardo Rodrigues Campos, Kátia Lino, Marta Cristine Félix Rodrigues, Rodrigo Moulin Silva, Rozana Gasparello de Almeida, Sandro Félix Perazzio, Margarida de Fátima Fernandes Carvalho","doi":"10.1186/s42358-024-00370-2","DOIUrl":"https://doi.org/10.1186/s42358-024-00370-2","url":null,"abstract":"Hemophagocytic lymphohistiocytosis (HLH) is a rare genetic hyperinflammatory syndrome that occurs early in life. Macrophage activation syndrome (MAS) usually refers to a secondary form of HLH associated with autoimmunity, although there are other causes of secondary HLH, such as infections and malignancy. In this article, we reviewed the concepts, epidemiology, clinical and laboratory features, diagnosis, differential diagnosis, prognosis, and treatment of HLH and MAS. We also reviewed the presence of MAS in the most common autoimmune diseases that affect children. Both are severe diseases that require prompt diagnosis and treatment to avoid morbidity and mortality.","PeriodicalId":48634,"journal":{"name":"Advances in Rheumatology","volume":"19 1","pages":""},"PeriodicalIF":2.3,"publicationDate":"2024-04-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140614379","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}
引用次数: 0
Prevalence of spondyloarthritis in inflammatory bowel disease according ASAS and ultrassonography and its correlation with plasma calprotectin 根据 ASAS 和超声波检查得出的炎症性肠病脊柱关节炎患病率及其与血浆钙蛋白的相关性
IF 2.3 4区 医学
Advances in Rheumatology Pub Date : 2024-04-15 DOI: 10.1186/s42358-023-00348-6
Míriam Küster Huber, Valeria Valim, Érica Vieira Serrano, José Alexandre Mendonça, Rafael Burgomeister Lourenço, Thaisa Moraes Ribeiro Espírito Santo, Hilde Nordal, Maria de Fátima Bissoli, Maria Bernadete Renoldi de Oliveira Gavi
{"title":"Prevalence of spondyloarthritis in inflammatory bowel disease according ASAS and ultrassonography and its correlation with plasma calprotectin","authors":"Míriam Küster Huber, Valeria Valim, Érica Vieira Serrano, José Alexandre Mendonça, Rafael Burgomeister Lourenço, Thaisa Moraes Ribeiro Espírito Santo, Hilde Nordal, Maria de Fátima Bissoli, Maria Bernadete Renoldi de Oliveira Gavi","doi":"10.1186/s42358-023-00348-6","DOIUrl":"https://doi.org/10.1186/s42358-023-00348-6","url":null,"abstract":"Enteropathic spondyloarthritis is underdiagnosed and inflammatory biomarkers and ultrasonography (US) could be useful for screening inflammatory bowel disease (IBD) patients. The objective of this study was to evaluate the prevalence of spondyloarthritis (SpA) in IBD patients, according to the Assessment of SpondyloArthritis International Society (ASAS) criteria and the correlation of results of US of entheses and joints with plasma calprotectin levels. This was an observational cross-sectional study. Patients from the IBD outpatient clinic of a reference center were evaluated according to ASAS criteria classification, results of US of entheses and joints, and inflammatory biomarker measurements (erythrocyte sedimentation rates, C-reactive protein levels, fecal and plasma calprotectin levels). A p value lower than 0.05 was considered significant. A total of 30.5% of the studied sample (n = 118) of patients with IBD presented at least one inflammatory musculoskeletal manifestation. The overall prevalence of enteropathic SpA was 13.55%, with 10.16% axial SpA and 4.23% peripheral SpA according to the ASAS criteria. A total of 42.1% of patients had an MASEI score greater than 18, 35.2% had synovitis, and 14.7% had tenosynovitis on US, increasing the frequency of diagnosis of enteropathic SpA to 22.8%. Plasma calprotectin levels were similar to those in healthy controls, and correlated only with the fecal calprotectin level (p 0.041). A total of 13.5% of patients met the criteria in accordance with the ASAS criteria for enteropathic SpA, which increased to 22.8% with the addition of US. The prevalence of enthesitis, synovitis and tenosynovitis by US of symptomatic joints and entheses were 42%, 35% and 14.7% respectively. Plasma calprotectin was correlated with fecal calprotectin but not with inflammatory biomarkers or US or ASAS criteria.","PeriodicalId":48634,"journal":{"name":"Advances in Rheumatology","volume":"40 1","pages":""},"PeriodicalIF":2.3,"publicationDate":"2024-04-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140583870","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}
引用次数: 0
Drug survival and change of disease activity using a second janus kinase inhibitor in patients with difficult-to-treat rheumatoid arthritis who failed to a janus kinase inhibitor and subsequent biologics 使用第二种破伤风激酶抑制剂治疗破伤风激酶抑制剂和后续生物制剂治疗失败的难治性类风湿性关节炎患者的药物存活率和疾病活动性变化
IF 2.3 4区 医学
Advances in Rheumatology Pub Date : 2024-04-15 DOI: 10.1186/s42358-024-00368-w
Oh Chan Kwon, Wonho Choi, Soo Min Ahn, Ji Seon Oh, Seokchan Hong, Chang-Keun Lee, Bin Yoo, Min-Chan Park, Yong-Gil Kim
{"title":"Drug survival and change of disease activity using a second janus kinase inhibitor in patients with difficult-to-treat rheumatoid arthritis who failed to a janus kinase inhibitor and subsequent biologics","authors":"Oh Chan Kwon, Wonho Choi, Soo Min Ahn, Ji Seon Oh, Seokchan Hong, Chang-Keun Lee, Bin Yoo, Min-Chan Park, Yong-Gil Kim","doi":"10.1186/s42358-024-00368-w","DOIUrl":"https://doi.org/10.1186/s42358-024-00368-w","url":null,"abstract":"To assess the drug survival and change of disease activity using a second Janus kinase inhibitor (JAKi) after failure to a JAKi and subsequent biologic disease-modifying anti-rheumatic drugs (bDMARDs) in patients with difficult-to-treat rheumatoid arthritis (RA). This retrospective cohort study included 32 patients with difficult-to-treat RA who failed to a JAKi and subsequently to one or more bDMARDs and then switched to a second JAKi. To assess drug survival, electronic medical records of each patient were reviewed. Data on whether the second JAKi was discontinued, and the reasons for discontinuation were collected. The change of disease activity was assessed by analyzing changes in tender joint count (TJC), swollen joint count (SJC), patient’s global assessment of disease activity on a visual-analogue scale (VAS), erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), Disease Activity Score for 28 joints with ESR (DAS28-ESR), and DAS28-CRP from baseline to that at six months from initiation of the second JAKi. Overall, discontinuation of the second JAKi occurred in 20 (62.5%) patients. Primary failure, secondary failure, adverse events, and insurance coverage issues were the reasons for discontinuation in 9 (45.0%), 5 (25.0%), 2 (10.0%), and 4 (20.0%) patients, respectively. The estimated 2-year drug survival rate was 39.3%. In terms of change of disease activity, the second JAKi significantly improved TJC (p < 0.001), SJC (p < 0.001), VAS (p < 0.001), CRP (p = 0.026), DAS28-ESR (p < 0.001), and DAS28-CRP (p < 0.001) at 6-month compared with that at the baseline. Second JAKi could be a therapeutic option in patients with difficult-to-treat RA who have failed to a JAKi and subsequent bDMARDs.","PeriodicalId":48634,"journal":{"name":"Advances in Rheumatology","volume":"14 1","pages":""},"PeriodicalIF":2.3,"publicationDate":"2024-04-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140583894","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}
引用次数: 0
Ultrasonographic and power doppler parameters of nails fail to differentiate between onychodystrophy in patients with psoriasis vulgaris or psoriatic arthritis 指甲的超声波和功率多普勒参数无法区分寻常型银屑病和银屑病关节炎患者的甲软骨营养不良症
IF 2.3 4区 医学
Advances in Rheumatology Pub Date : 2024-04-11 DOI: 10.1186/s42358-024-00367-x
Anber Ancel Tanaka, Betina Werner, Annelise Correa Bueno Bragatto, Thelma Larocca Skare, Bárbara Stadler
{"title":"Ultrasonographic and power doppler parameters of nails fail to differentiate between onychodystrophy in patients with psoriasis vulgaris or psoriatic arthritis","authors":"Anber Ancel Tanaka, Betina Werner, Annelise Correa Bueno Bragatto, Thelma Larocca Skare, Bárbara Stadler","doi":"10.1186/s42358-024-00367-x","DOIUrl":"https://doi.org/10.1186/s42358-024-00367-x","url":null,"abstract":"Nail involvement is frequent in patients with psoriasis (Pso) and psoriatic arthritis (PsA) and there is a relationship between nail involvement and inflammation of the enthesis. The main objective of the present study is to describe the ultrasound findings and clinical characteristics of nails from patients with psoriasis and psoriatic arthritis with and without nail dystrophy. A cross-sectional study including consecutive patients with PsO and PsA was carried out. The study patients were divided into 4 groups, totaling 120 participants. Group 1: patients with psoriasis vulgaris and clinically normal nails; Group 2: patients with psoriasis vulgaris and onychodystrophy; Group 3: patients with psoriatic arthritis and clinically normal nails; Group 4: patients with psoriatic arthritis and onychodystrophy; All patients were submitted to dermatological and rheumatological clinical analysis. Ultrasound examinations was performed by a single examiner, blinded to all clinical data, with ultrasound high resolution, in B-mode or gray-scale (GS), Power Doppler (PD) and Spectral Doppler. A significant difference was found between the groups regarding the variable Psoriasis Area and Severity Index (PASI) (p = 0.008) and body surface area (BSA) (p = 0.005), with patients with psoriatic arthritis having lower PASI and BSA compared to patients with only cutaneous psoriasis. A positive relationship was found with the average ultrasound thickness of the nail bed and the Nail Psoriasis Severity Index (NAPSI) in correlation analysis (rho = 0.344). When we grouped patients with psoriasis and psoriatic arthritis, there was no significant difference between the cutaneous psoriasis groups and the psoriatic arthritis groups in terms of nail plate GS (p = 0.442), nail bed PD (p = 0.124). Greater nail bed thickness indicates early psoriatic nail disease, as confirmed in our study correlating NAPSI with nail bed thickness. Ultrasonography is a low-cost exam, promising in the evaluation, showing that the ultrasound grayscale is consistent with those who have dystrophic nails, but it can’t distinguish psoriasis from psoriatic arthritis, even in those with nail dystrophy.","PeriodicalId":48634,"journal":{"name":"Advances in Rheumatology","volume":"121 1","pages":""},"PeriodicalIF":2.3,"publicationDate":"2024-04-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140583872","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}
引用次数: 0
Predictors of serious infections in rheumatoid arthritis-a prospective Brazilian cohort. 类风湿性关节炎严重感染的预测因素--巴西前瞻性队列。
IF 2 4区 医学
Advances in Rheumatology Pub Date : 2024-03-29 DOI: 10.1186/s42358-024-00363-1
Ana Luisa Bagno de Almeida, Maria Fernanda B Resende Guimarães, Maria Raquel da Costa Pinto, Leticia Rocha Pereira, Ana Paula Monteiro Gomides Reis, Karina Rossi Bonfiglioli, Paulo Louzada-Junior, Rina Dalva Neubarth Giorgi, Gláucio Ricardo Werner de Castro, Sebastião Cezar Radominski, Claiton Viegas Brenol, Alisson Pugliesi, Licia Maria Henrique da Mota, Geraldo da Rocha Castelar-Pinheiro
{"title":"Predictors of serious infections in rheumatoid arthritis-a prospective Brazilian cohort.","authors":"Ana Luisa Bagno de Almeida, Maria Fernanda B Resende Guimarães, Maria Raquel da Costa Pinto, Leticia Rocha Pereira, Ana Paula Monteiro Gomides Reis, Karina Rossi Bonfiglioli, Paulo Louzada-Junior, Rina Dalva Neubarth Giorgi, Gláucio Ricardo Werner de Castro, Sebastião Cezar Radominski, Claiton Viegas Brenol, Alisson Pugliesi, Licia Maria Henrique da Mota, Geraldo da Rocha Castelar-Pinheiro","doi":"10.1186/s42358-024-00363-1","DOIUrl":"10.1186/s42358-024-00363-1","url":null,"abstract":"<p><strong>Background: </strong>Infections increase mortality and morbidity and often limit immunosuppressive treatment in rheumatoid arthritis patients.</p><p><strong>Objective: </strong>To analyze the occurrence of serious infections and the associated factors in a cohort of rheumatoid arthritis patients under real-life conditions.</p><p><strong>Methods: </strong>We analyzed data from the REAL, a prospective observational study, that evaluated Brazilian RA patients, with clinical and laboratory data collected over a year. Univariate and multivariate analyses were performed from the adjustment of the logistic regression model Generalized Estimating Equations (GEE), with the primary outcome being the occurrence of serious infection, defined as need for hospitalization or use of intravenous antibiotics for its treatment.</p><p><strong>Results: </strong>841 patients were included with an average follow-up time of 11.2 months (SD 2.4). Eighty-nine serious infections occurred, corresponding to 13 infections per 100 patient-years. Pulmonary fibrosis, chronic kidney disease (CKD) and central nervous system disease increased the chances of serious infection by 3.2 times (95% CI: 1.5-6.9), 3.6 times (95% CI: 1.2-10.4) and 2.4 times (95% CI: 1.2-5.0), respectively. The use of corticosteroids in moderate doses increased the chances by 5.4 times (95% CI: 2.3-12.4), and for each increase of 1 unit in the health assessment questionnaire (HAQ), the chance increased 60% (95% CI: 20-120%).</p><p><strong>Conclusion: </strong>The use of corticosteroids at moderate doses increased the risk of serious infection in RA patients. Reduced functionality assessed by the HAQ and comorbidities were other important factors associated with serious infection in this cohort.</p>","PeriodicalId":48634,"journal":{"name":"Advances in Rheumatology","volume":"64 1","pages":"23"},"PeriodicalIF":2.0,"publicationDate":"2024-03-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140327289","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}
引用次数: 0
Effects of intra-articular injection of platelet-rich plasma on the inflammatory process and histopathological characteristics of cartilage and synovium in animals with osteoarthritis: a systematic review with meta-analysis. 关节内注射富血小板血浆对骨关节炎动物软骨和滑膜炎症过程及组织病理学特征的影响:系统综述与荟萃分析。
IF 2 4区 医学
Advances in Rheumatology Pub Date : 2024-03-29 DOI: 10.1186/s42358-024-00364-0
Homero Garcia-Motta, Cristiano Carvalho, Evelyn Maria Guilherme, Marcos Paulo Braz de Oliveira, Karina Nogueira Zambone Pinto Rossi
{"title":"Effects of intra-articular injection of platelet-rich plasma on the inflammatory process and histopathological characteristics of cartilage and synovium in animals with osteoarthritis: a systematic review with meta-analysis.","authors":"Homero Garcia-Motta, Cristiano Carvalho, Evelyn Maria Guilherme, Marcos Paulo Braz de Oliveira, Karina Nogueira Zambone Pinto Rossi","doi":"10.1186/s42358-024-00364-0","DOIUrl":"10.1186/s42358-024-00364-0","url":null,"abstract":"<p><strong>Background: </strong>Osteoarthritis (OA) affects the entire joint, causing structural changes in articular cartilage, subchondral bone, ligaments, capsule, synovial membrane, and periarticular muscles that afflicts millions of people globally, leading to persistent pain and diminished quality of life. The intra-articular use of platelet-rich plasma (PRP) is gaining recognition as a secure therapeutic approach due to its potential regenerative capabilities. However, there is controversial clinical data regarding efficacy of PRP for OA treatment. In this context, gathering scientific evidence on the effects of PRP in treating OA in animal models could provide valuable insights into understanding its impact on aspects like cartilage health, synovial tissue integrity, and the inflammatory process in affected joints. Thus, the objective of this study was to assess the effects of PRP injections on inflammation and histopathological aspects of cartilage and synovium in animal models of OA through a comprehensive systematic review with meta-analysis.</p><p><strong>Methods: </strong>A electronic search was conducted on Medline, Embase, Web of Science, The Cochrane Library, LILACS, and SciELO databases for relevant articles published until June 2022. A random-effects meta-analysis was employed to synthesize evidence on the histological characteristics of cartilage and synovium, as well as the inflammatory process. The GRADE approach was utilized to categorize the quality of evidence, and methodological quality was assessed using SYRCLE's RoB tool.</p><p><strong>Results: </strong>Twenty-one studies were included in the review, with twelve of them incorporated into the meta-analysis. PRP treatment demonstrated superior outcomes compared to the control group in terms of cartilage histology (very low quality; p = 0.0002), synovium histology (very low quality; p < 0.0001), and reductions in proinflammatory markers, including IL-1 (low quality; p = 0.002), IL-6 (very low quality; p < 0.00001), and TNF-α (very low; p < 0.00001). However, PRP treatment did not yield a significant impact on PDGF-A levels (very low quality; p = 0.81).</p><p><strong>Conclusion: </strong>PRP appears capable of reducing proinflammatory markers (IL-1, IL-6, TNF-α) and mitigating cartilage and synovium damage in animals with OA. However, the levels of evidence of these findings are low to very low. Therefore, more rigorous studies with larger samples are needed to improve the quality of evidence.</p><p><strong>Prospero registration: </strong>CRD42022250314.</p>","PeriodicalId":48634,"journal":{"name":"Advances in Rheumatology","volume":"64 1","pages":"24"},"PeriodicalIF":2.0,"publicationDate":"2024-03-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140327288","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}
引用次数: 0
What should rheumatologists know about Gaucher disease and Fabry disease? Connecting the dots for an overview. 风湿病学家对戈谢病和法布里病应了解多少?连接点概述。
IF 2 4区 医学
Advances in Rheumatology Pub Date : 2024-03-22 DOI: 10.1186/s42358-024-00362-2
Rafael Alves Cordeiro, Nilton Salles Rosa Neto, Henrique Ayres Mayrink Giardini
{"title":"What should rheumatologists know about Gaucher disease and Fabry disease? Connecting the dots for an overview.","authors":"Rafael Alves Cordeiro, Nilton Salles Rosa Neto, Henrique Ayres Mayrink Giardini","doi":"10.1186/s42358-024-00362-2","DOIUrl":"10.1186/s42358-024-00362-2","url":null,"abstract":"<p><p>Gaucher and Fabry diseases are lysosomal storage disorders in which deficient enzyme activity leads to pathological accumulation of sphingolipids. These diseases have a broad phenotypic presentation. Musculoskeletal symptoms and pain complaints are frequently reported by patients. Thus, rheumatologists can be contacted by these patients, contributing to the correct diagnosis, earlier indication of appropriate treatment and improvement of their prognosis. This review describes important concepts about Gaucher and Fabry diseases that rheumatologists should understand to improve patients' quality of life and change the natural history of these diseases.</p>","PeriodicalId":48634,"journal":{"name":"Advances in Rheumatology","volume":"64 1","pages":"22"},"PeriodicalIF":2.0,"publicationDate":"2024-03-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140194865","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}
引用次数: 0
Effectiveness and safety of biological and target synthetic drugs treatment for psoriatic arthritis: a systematic review with network meta-analysis. 生物和靶向合成药物治疗银屑病关节炎的有效性和安全性:系统综述与网络荟萃分析。
IF 2 4区 医学
Advances in Rheumatology Pub Date : 2024-03-21 DOI: 10.1186/s42358-024-00361-3
Thais Montezuma, Livia Fernandes Probst, Matheus Oliveira Almeida
{"title":"Effectiveness and safety of biological and target synthetic drugs treatment for psoriatic arthritis: a systematic review with network meta-analysis.","authors":"Thais Montezuma, Livia Fernandes Probst, Matheus Oliveira Almeida","doi":"10.1186/s42358-024-00361-3","DOIUrl":"10.1186/s42358-024-00361-3","url":null,"abstract":"<p><strong>Background: </strong>Psoriatic arthritis (PA) is a chronic inflammatory systemic arthritis that can result in loss of functional capacity and joint deformation. This systematic review assessed the effectiveness and safety of biological and target synthetic drugs for treating PA.</p><p><strong>Methods: </strong>We searched for randomized clinical trials (RCTs) that evaluated the use of Adalimumab, Etanercept, Infliximab, Golimumab, Secukinumab, Certolizumab Pegol and Tofacitinib in the main general databases and clinical trial registers databases. The primary outcomes were ACR 50, PsARC, and serious adverse events. Two independent reviewers performed study selection and data extraction. Network meta-analyses were conducted using a random effects model and frequentist approach. The CINeMA software was used to assess the certainty of evidence.</p><p><strong>Results: </strong>We included 33 RCTs (n = 11,034). The results from the network meta-analysis for the ACR 50 at 6-months follow-up showed that all drugs were superior to placebo, with Secukinumab (high certainty of evidence), Infliximab (very low certainty of evidence) and Adalimumab (high certainty of evidence) ranking the highest. Regarding the PsARC (at 6-months follow-up), all drugs, except for Golimumab (very low certainty of evidence), were superior to placebo, with Etanercept (low certainty of evidence), Infliximab (low certainty of evidence) and Certolizumab Pegol (low certainty of evidence) being the most effective drugs. There were no significant differences in the risk of serious adverse events between the drugs and placebo. Golimumab (very low certainty of evidence), Secukinumab (low certainty of evidence), and Adalimumab (very low certainty of evidence) ranked the highest for safety.</p><p><strong>Conclusions: </strong>In conclusion, based on the balance between efficacy and safety, Secukinumab and Adalimumab may be the preferred options among the evaluated drugs for treating patients with PsA. However, caution is necessary when interpreting the safety findings, as they are supported by evidence of low to very low certainty. Consequently, the balance between benefits and potential risks may change as new safety evaluation studies become available.</p><p><strong>Protocol registration: </strong>PROSPERO: CRD42022315577.</p>","PeriodicalId":48634,"journal":{"name":"Advances in Rheumatology","volume":"64 1","pages":"21"},"PeriodicalIF":2.0,"publicationDate":"2024-03-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140185985","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}
引用次数: 0
Clinical course of nontuberculous mycobacterial pulmonary disease in patients with rheumatoid arthritis. 类风湿性关节炎患者非结核分枝杆菌肺病的临床过程。
IF 2 4区 医学
Advances in Rheumatology Pub Date : 2024-03-15 DOI: 10.1186/s42358-024-00357-z
Nakwon Kwak, Jinyoung Moon, Joong-Yub Kim, Jun Won Park, Jae-Joon Yim
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