ARP Rheumatology最新文献

{"title":"Predictors of myositis in mixed connective tissue disease: A multicentre retrospective study.","authors":"Ana Teresa Melo, Manuel Silvério-António, Joana Martins-Martinho, Francisca Guimarães, Eduardo Dourado, Daniela Oliveira, Jorge Lopes, André Saraiva, Ana Gago, Margarida Correia, Ana Lúcia Fernandes, Sara Dinis, Rafaela Teixeira, Susana Pinto Silva, Carlos Costa, Tiago Beirão, Carolina Furtado, Pedro Abreu, Nikita Khmelinskii","doi":"10.63032/BAYU2491","DOIUrl":"10.63032/BAYU2491","url":null,"abstract":"<p><strong>Objectives: </strong>We aimed to identify clinical and serological predictors of myositis in mixed connective tissue disease (MCTD).</p><p><strong>Methods: </strong>We performed a nationwide, retrospective, multicentre study including adult-onset MCTD patients fulfilling at least one of the following diagnostic criteria: Sharp's, Kasukawa, Alarcón-Segovia, or Kahn's. Univariate analysis was performed using Chi-square, Fisher exact, Student's t or Mann-Whitney U tests, as appropriate. Multivariate analysis was performed using binary logistic regression.</p><p><strong>Results: </strong>Ninety-eight patients were included. Myositis was observed in 43.9% of patients, of whom 60.5% had myositis at disease onset. Proximal muscle weakness was described in 30 patients with muscle involvement (70%). Gastrointestinal involvement was identified in 28% and respiratory involvement in 29% of myositis patients. In the same subgroup of patients, 41.7% had a myopathic pattern on electromyography, and 47.1% had histological myositis features in the muscle biopsy. Fever (OR=6.96, p=0.022) was an independent predictor of myositis, regardless of sex, age at diagnosis, ancestry, and respiratory involvement. African ancestry (OR=8.39, p=0.019), leukopenia at the disease onset (OR 6.24, p=0.021), and younger age at diagnosis (OR=1.07/year, p=0.035) were identified as independent predictors of myositis at disease onset, regardless of sex and scleroderma pattern in capillaroscopy.</p><p><strong>Conclusions: </strong>Myositis is a common manifestation of MCTD, even at the disease onset. African ancestry, leukopenia at the disease onset, younger age at diagnosis, and fever should prompt a thorough evaluation for myositis.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":" ","pages":"174-181"},"PeriodicalIF":1.4,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142146443","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":"Classification criteria for large vessel vasculitis.","authors":"Cristina Ponte","doi":"10.63032/TIRL9893","DOIUrl":"10.63032/TIRL9893","url":null,"abstract":"","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":" ","pages":"170-173"},"PeriodicalIF":1.4,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142378391","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":"Rheumatoid arthritis and ANCA-associated vasculitis - an unusual overlap successfully treated with rituximab.","authors":"Margarida Santos Faria, Iolanda Godinho, Estela Nogueira, Joana Tavares, Daniel Carvalho, Cristina Ponte","doi":"10.63032/ZTDH8563","DOIUrl":"10.63032/ZTDH8563","url":null,"abstract":"","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":" ","pages":"248-249"},"PeriodicalIF":1.4,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142378392","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":"Antifibrotics in rheumatoid arthritis-associated interstitial lung disease - real-world data from a nationwide cohort.","authors":"Ana Catarina Duarte, Carlos Marques Gomes, Margarida Correia, Beatriz Mendes, Carolina Mazeda, Francisca Guimarães, Joana Abelha-Aleixo, Miguel Guerra, Roberto Pereira da Costa, Tiago Meirinhos, Maria José Santos","doi":"10.63032/POPM9413","DOIUrl":"10.63032/POPM9413","url":null,"abstract":"<p><strong>Introduction: </strong>Interstitial lung disease (ILD) is the most common pulmonary manifestation of rheumatoid arthritis (RA) and is associated with an increased mortality. Clinical trials have shown that antifibrotics (nintedanib and pirfenidone) can slow the progression of connective tissue disease-associated ILD. This study aims to evaluate the effectiveness and tolerability of antifibrotics in a national, real-world cohort of patients with RA-ILD.</p><p><strong>Material and methods: </strong>We conducted an observational multicenter study of RA-ILD patients treated with antifibrotics, who were prospectively followed in Reuma.pt. Demographic and clinical data, pulmonary function tests (PFTs) results and adverse events (AEs) were collected. A linear mixed model with random intercept was used to compare PFT results within 12 (±6) months before to 12 (±6) months after antifibrotic initiation. Drug persistence was evaluated using Kaplan-Meier curves.</p><p><strong>Results: </strong>We included 40 RA-ILD patients, 27 (67.5%) initially treated with nintedanib and 13 (32.5%) with pirfenidone. Most of the patients were female (55%), and current or past smokers (52.5%). At antifibrotic initiation, mean age was 70.9 ± 7.1 years and median ILD duration 5.0 [IQR 2.3-7.5] years. A total of 20 patients were included in effectiveness analysis, with the use of antifibrotics interrupting the decline of forced vital capacity (FVC; decline 300 ± 500 mL in the year before antifibrotic initiation vs. improvement of 200 ± 400 mL in the year following antifibrotic initiation, p=0.336) and total lung capacity (TLC; decline 800 ± 300 mL in the year before antifibrotic initiation vs. improvement of 600 ± 900 mL in the year following antifibrotic initiation, p=0.147). However, diffusion capacity for carbon monoxide remained in decline (3% decline in the year before antifibrotic initiation vs. 2.9% decline in the year following antifibrotic initiation, p=0.75). AEs were reported in 16 (40%) patients and led to drug discontinuation in 12 (30%). Median duration of drug persistence was 150.3 weeks (95 %CI 11.0-289.6), with no difference between nintedanib and pirfenidone (p = 0.976).</p><p><strong>Conclusion: </strong>This study with real-world data corroborates the usefulness of antifibrotics in stabilizing lung function, based on FVC and TLC. However, AEs were frequently reported and were the main cause for drug discontinuation.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":" ","pages":"182-188"},"PeriodicalIF":1.4,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142378390","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":"Ferric carboxymaltose-induced hypophosphatemia - a case series.","authors":"Carla Campinho Ferreira, Paulo Pereira, Margarida Correia, Emanuel Costa, Diogo Esperança Almeida, José Redondo Costa, Ana Roxo Ribeiro, Joana Leite Silva","doi":"10.63032/DGZN9101","DOIUrl":"10.63032/DGZN9101","url":null,"abstract":"<p><p>Hypophosphatemia may cause serious complications. Depending on its severity and duration, signs and symptoms range from fatigue to life-threatening events, like severe rhabdomyolysis and mental status changes. Long-term consequences include osteomalacia. Hypophosphatemia may be secondary to the use of parental iron, mostly associated with ferric carboxymaltose (FCM), with an incidence of around 45% to 70%. We describe three cases of hypophosphatemia in patients with chronic iron deficiency anemia, requiring repeated FCM infusions. The patients' presentation to the Rheumatology department included musculoskeletal symptoms of severe hypophosphatemia and long-term hypophosphatemic osteomalacia, with fractures. We aim to raise awareness for ferric carboxymaltose-induced hypophosphatemia, an entity increasingly described in the literature that can be responsible for severe disability or potentially life-threatening adverse events.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":" ","pages":"226-230"},"PeriodicalIF":1.4,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142146437","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":"Systematic literature review to inform the Portuguese recommendations for the management of Raynaud's phenomenon and digital ulcers in systemic sclerosis and other connective tissue diseases.","authors":"Emanuel Costa, Filipe Cunha-Santos, Eduardo Dourado, Daniela Oliveira, Louise Falzon, Vasco Romão, Ana Catarina Duarte, Ana Cordeiro, Tânia Santiago, Alexandre Sepriano","doi":"10.63032/YHBL8967","DOIUrl":"10.63032/YHBL8967","url":null,"abstract":"<p><strong>Objective: </strong>To perform a systematic literature review (SLR) aimed at evaluating the efficacy and safety of pharmacological and non-pharmacological treatments for Raynaud's phenomenon (RP) and digital ulcers (DU) in patients with systemic sclerosis (SSc) and other connective tissue diseases (CTD), in order to inform the Portuguese recommendations for managing RP and DU in these patients.</p><p><strong>Methods: </strong>A SLR was conducted until May 2022 to identify studies assessing the efficacy and safety of pharmacological and non-pharmacological interventions for RP and DU in SSc and other CTD. Eligible study designs included randomized controlled trials (RCTs), controlled clinical trials, and their extensions for assessing efficacy and safety of interventions. Observational studies with a comparator were included for evaluating the efficacy and safety of non-pharmacological interventions and safety of pharmacological interventions. The risk of bias of each study was assessed using standard tools.</p><p><strong>Results: </strong>Out of 71 publications meeting the inclusion criteria, 59 evaluated pharmacological and 12 non-pharmacological interventions. We found moderate quality evidence supporting the efficacy of calcium channel blockers, phosphodiesterase-5 inhibitors, and intravenous prostacyclin analogues in reducing RP frequency, severity, and duration. Intravenous iloprost had a small to moderate effect size in improving DU healing. Phosphodiesterase-5 inhibitors were effective in reducing total DU count, new DU occurrence, and enhancing DU healing. Bosentan effectively prevented new DU in SSc patients. No new safety concerns were associated with these treatments. The studies on non-pharmacological interventions were, in general, of low quality, and had a small sample size. Warming measures decreased frequency and duration of RP attacks; laser therapy improved RP-related outcomes; local oxygen-ozone therapy improved RP outcomes as an add-on therapy; bone marrow mononuclear cell implantation improved DU-associated pain; periarterial sympathectomy and vascular bypass reduced DU number and finger amputation risk.</p><p><strong>Conclusion: </strong>The available evidence supports the efficacy and safety of pharmacological interventions, namely nifedipine, sildenafil, iloprost, and bosentan in treating RP and DU in patients with SSc and other CTD. Scarce and low-quality evidence does support the use of some non-pharmacological interventions but with only a modest effect size. This SLR underscores the limited availability of high-quality evidence for determining the optimal treatment.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":" ","pages":"128-144"},"PeriodicalIF":1.4,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139672840","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":"Lessons learnt from the recent recommendations for the non-pharmacological management of systemic sclerosis.","authors":"Tânia Santiago, Ruben Fernandes, Ricardo Ferreira, Ioannis Parodis, Carina Bostrom","doi":"10.63032/JBRG6950","DOIUrl":"10.63032/JBRG6950","url":null,"abstract":"<p><p>In inflammatory rheumatic diseases, including, systemic sclerosis (SSc) there is growing evidence that treatment strategies should not only target disease control in terms of clinical features and laboratory tests but consider distinct interventions to mitigate all domains of perceived disease impact. The results of a multicentric work based on data from the Rheumatic Diseases Portuguese Registry (Reuma.pt)/Scleroderma indicated that the optimization of outcomes for patients with SSc would in all probability require assessment of the needs of individual patients and consider adjunctive interventions in clinical practice to mitigate all significantly affected domains of disease impact. Recently, in June 2023, a task force under the auspices of EULAR, comprising rheumatologists, health professionals and patient advocates published four overarching principles and twelve recommendations for the non-pharmacological management of people living with SSc and systemic lupus erythematosus (SLE).</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":" ","pages":"81-82"},"PeriodicalIF":1.4,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139898241","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":"Gastrointestinal bleeding - an atypical presentation of granulomatosis with polyangiitis.","authors":"Ana Catarina Moniz, Marta Teixeira Almeida, Bruno Pereira, Cristina Ponte, Ana Valido","doi":"10.63032/GYJZ4840","DOIUrl":"10.63032/GYJZ4840","url":null,"abstract":"","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":" ","pages":"243-245"},"PeriodicalIF":1.4,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142146438","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":"Gut microbiota in axial spondyloarthritis : genetics, medications and future treatments.","authors":"Nehal Yemula, Riyad Sheikh","doi":"10.63032/WUII1201","DOIUrl":"10.63032/WUII1201","url":null,"abstract":"<p><p>Axial spondyloarthritis, also referred to as ankylosing spondylitis, is a chronic inflammatory condition that predominantly affects the axial spine but may also present with peripheral arthritis. It falls within the umbrella of disorders known as spondyloarthropathies. In addition to axial spondyloarthritis, this group includes psoriatic arthritis, enteropathic arthritis, reactive arthritis, and undifferentiated spondyloarthropathy, with axial spondyloarthritis being one of the most common. The overall mechanisms underlying the development of axial spondyloarthritis are complex and multifactorial. There is a significant and well-recognized association between axial spondyloarthritis and the HLA-B27 gene, but there have also been non-HLA genes identified in the disease process, as well as certain inflammatory cytokines that play a role in the inflammatory process, such as tumor necrosis factor (TNF). More recently, there has been research and new evidence linking changes in the gut microbiota to the disease process of axial spondyloarthritis. Research into the role of the gut microbiota and gut dysbiosis is a large, ever-growing field. It has been associated with a multitude of conditions, including axial spondyloarthritis. This mini-review highlights the symbiotic relationship of the gut microbiota with the pathogenesis, therapeutic agents and future treatments of axial spondyloarthritis.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":" ","pages":"216-225"},"PeriodicalIF":1.4,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142146439","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":"Drugs and ANCA - an atypical association.","authors":"Tiago Beirão, Beatriz Samões, Catarina Rua, Romana Vieira, Joana Abelha-Aleixo, Patricia Pinto, Flavio Costa, Diogo Fonseca, Margarida Mota, Catarina Amorim Meireles, Taciana Videira","doi":"10.63032/PXUT9006","DOIUrl":"10.63032/PXUT9006","url":null,"abstract":"<p><p>Eosinophilic vasculitis is a rare condition due to vascular damage and infiltration of eosinophils into tissues. Antineutrophil cytoplasmic antibody (ANCA), like perinuclear ANCA (p-ANCA), is common in the context of systemic vasculitis, but its association with illicit drug use is unusual. This case report showcases a distinctive scenario featuring a positive pANCA, prompting a meticulous examination of the interplay between vasculitic manifestations and drug abuse.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":" ","pages":"246-247"},"PeriodicalIF":1.4,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142146436","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}