Joana Victor Lage, Ana Teresa Guerra, Francisca Costa, Andreia Martins, Paula Correia, Catarina Luis
Kikuchi-Fujimoto disease (KFD) is a rare and benign condition mainly characterized by fever and lymphadenopathies. Although many studies have been carried out over time, its aetiology remains unclear, with infectious and autoimmune processes being hypothesized as the main causes. We report three cases of Kikuchi-Fujimoto disease. All patients were female and presented with fever and cervical lymphadenopathies. Extensive work up was performed, in order to rule out infectious, autoimmune and lymphoproliferative diseases. The diagnosis was established through lymph node excisional biopsy and histopathological examination. All patients were followed-up in a medical appointment, with one developing systemic lupus erythematosus (SLE).
{"title":"Kikuchi-Fujimoto - an enigmatic and rare disease: a report of 3 cases and brief review of the literature.","authors":"Joana Victor Lage, Ana Teresa Guerra, Francisca Costa, Andreia Martins, Paula Correia, Catarina Luis","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Kikuchi-Fujimoto disease (KFD) is a rare and benign condition mainly characterized by fever and lymphadenopathies. Although many studies have been carried out over time, its aetiology remains unclear, with infectious and autoimmune processes being hypothesized as the main causes. We report three cases of Kikuchi-Fujimoto disease. All patients were female and presented with fever and cervical lymphadenopathies. Extensive work up was performed, in order to rule out infectious, autoimmune and lymphoproliferative diseases. The diagnosis was established through lymph node excisional biopsy and histopathological examination. All patients were followed-up in a medical appointment, with one developing systemic lupus erythematosus (SLE).</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":" ","pages":"None"},"PeriodicalIF":1.4,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142146442","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}
Background: Intra-articular glucocorticoid injection (IAGI) is widely used for treatment of knee osteoarthritis (OA) flares. Response rates are generally around 70%. Several studies have tried to identify predictors of good response, but response to ultrasound (US)-guided injection has not yet been investigated. This study aimed to identify the predictors of response to IAGI performed under US guidance in patients with primary knee OA.
Materials and methods: A total of 116 patients (116 knees) presenting with unilateral or bilateral primary knee OA were enrolled for this prospective single-center study. All were aged >40 years and met the American College of Rheumatology (ACR) criteria for knee OA. Demographic, clinical, laboratory, and imaging data were collected, injection was performed using US guidance, and tolerance was assessed. The primary efficacy endpoint was ≥40% reduction in total WOMAC score (WOMAC40). Univariate and multivariate logistic regression analyses were conducted to identify the predictors of response.
Results: The mean age of the patients was 64.2 ± 9.4 years and mean BMI was 29.9 ± 3.8 kg/m2. Total WOMAC40 response rate was 61.2%. In multivariate analysis, the independent predictors of response were BMI.
背景:关节内注射糖皮质激素(IAGI)被广泛用于治疗膝关节骨性关节炎(OA)复发。反应率一般在 70% 左右。有几项研究试图确定良好反应的预测因素,但尚未对超声(US)引导注射的反应进行调查。本研究旨在确定原发性膝关节 OA 患者在 US 引导下进行 IAGI 反应的预测因素:这项前瞻性单中心研究共招募了 116 名单侧或双侧原发性膝关节 OA 患者(116 个膝关节)。所有患者的年龄均大于 40 岁,并符合美国风湿病学会(ACR)的膝关节 OA 标准。研究人员收集了人口统计学、临床、实验室和影像学数据,在 US 引导下进行了注射,并评估了耐受性。主要疗效终点是 WOMAC 总分(WOMAC40)降低≥40%。为确定反应的预测因素,进行了单变量和多变量逻辑回归分析:患者的平均年龄为 64.2 ± 9.4 岁,平均体重指数为 29.9 ± 3.8 kg/m2。WOMAC40总反应率为61.2%。在多变量分析中,预测反应的独立因素是体重指数(BMI)。
{"title":"Predictors of efficacy of ultrasound-guided intra-articular glucocorticoid injection in knee osteoarthritis: A prospective study.","authors":"Samy Slimani, Amel Aissoug, Souhila Aouidane, Nacif Eddine Ghodbane, Aicha Ladjouze-Rezig","doi":"10.63032/NJOL3215","DOIUrl":"10.63032/NJOL3215","url":null,"abstract":"<p><strong>Background: </strong>Intra-articular glucocorticoid injection (IAGI) is widely used for treatment of knee osteoarthritis (OA) flares. Response rates are generally around 70%. Several studies have tried to identify predictors of good response, but response to ultrasound (US)-guided injection has not yet been investigated. This study aimed to identify the predictors of response to IAGI performed under US guidance in patients with primary knee OA.</p><p><strong>Materials and methods: </strong>A total of 116 patients (116 knees) presenting with unilateral or bilateral primary knee OA were enrolled for this prospective single-center study. All were aged >40 years and met the American College of Rheumatology (ACR) criteria for knee OA. Demographic, clinical, laboratory, and imaging data were collected, injection was performed using US guidance, and tolerance was assessed. The primary efficacy endpoint was ≥40% reduction in total WOMAC score (WOMAC40). Univariate and multivariate logistic regression analyses were conducted to identify the predictors of response.</p><p><strong>Results: </strong>The mean age of the patients was 64.2 ± 9.4 years and mean BMI was 29.9 ± 3.8 kg/m2. Total WOMAC40 response rate was 61.2%. In multivariate analysis, the independent predictors of response were BMI.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":" ","pages":"101-105"},"PeriodicalIF":1.4,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139898243","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}
Susana P Silva, Gisela Eugénio, Miguel Pinto, Anabela Barcelos
Anti-HMGCR myopathy is an increasingly recognized immune-mediated necrotizing myopathy. However, there are currently no evidence-based treatments available, so case reports and clinical experience are used to guide current management. We report a case of a 49-year-old man, treated with atorvastatin, who presented to the emergency department with progressive proximal muscle weakness. Anti-HMGCR antibodies were detected, and muscle biopsy revealed necrotizing myopathy. Initially, therapy with high-dose glucocorticoids and methotrexate was started, but 12 weeks later, the patient developed clinical deterioration with dysphagia. Then, he was successfully treated with one cycle of rituximab along with physical therapy. The use of rituximab in immune-mediated necrotizing myopathy has been heterogeneously described in the literature but mostly in case reports. The European Neuromuscular Centre working group recommends the use of rituximab in refractory cases. However, some studies highlight the importance of early and aggressive treatment for this disease. Clinical prospective studies are necessary to make proper evidence-based recommendations.
{"title":"Clinical and persistent remission in anti-HMGCR immune-mediated necrotizing myopathy to a single cycle of rituximab - a case-based review.","authors":"Susana P Silva, Gisela Eugénio, Miguel Pinto, Anabela Barcelos","doi":"10.63032/ZVNO7794","DOIUrl":"10.63032/ZVNO7794","url":null,"abstract":"<p><p>Anti-HMGCR myopathy is an increasingly recognized immune-mediated necrotizing myopathy. However, there are currently no evidence-based treatments available, so case reports and clinical experience are used to guide current management. We report a case of a 49-year-old man, treated with atorvastatin, who presented to the emergency department with progressive proximal muscle weakness. Anti-HMGCR antibodies were detected, and muscle biopsy revealed necrotizing myopathy. Initially, therapy with high-dose glucocorticoids and methotrexate was started, but 12 weeks later, the patient developed clinical deterioration with dysphagia. Then, he was successfully treated with one cycle of rituximab along with physical therapy. The use of rituximab in immune-mediated necrotizing myopathy has been heterogeneously described in the literature but mostly in case reports. The European Neuromuscular Centre working group recommends the use of rituximab in refractory cases. However, some studies highlight the importance of early and aggressive treatment for this disease. Clinical prospective studies are necessary to make proper evidence-based recommendations.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":" ","pages":"231-236"},"PeriodicalIF":1.4,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142146435","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}
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
Objectives: We aimed to identify clinical and serological predictors of myositis in mixed connective tissue disease (MCTD).
Methods: 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.
Results: 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.
Conclusions: 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.
{"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}
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
Introduction: 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.
Material and methods: 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.
Results: 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).
Conclusion: 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.
{"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}
Emanuel Costa, Filipe Cunha-Santos, Eduardo Dourado, Daniela Oliveira, Louise Falzon, Vasco Romão, Ana Catarina Duarte, Ana Cordeiro, Tânia Santiago, Alexandre Sepriano
Objective: 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.
Methods: 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.
Results: 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.
Conclusion: 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.
目的进行一项系统性文献综述(SLR),旨在评估系统性硬化症(SSc)和其他结缔组织疾病(CTD)患者雷诺现象(RP)和数字溃疡(DU)的药物和非药物治疗的有效性和安全性,从而为葡萄牙提出的治疗这些患者雷诺现象和数字溃疡的建议提供参考:在 2022 年 5 月之前进行了一次 SLR,以确定评估药物和非药物干预对 SSc 和其他 CTD 患者 RP 和 DU 的疗效和安全性的研究。符合条件的研究设计包括用于评估干预措施疗效和安全性的随机对照试验(RCT)、对照临床试验及其扩展研究。还包括有比较对象的观察性研究,用于评估非药物干预措施的疗效和安全性以及药物干预措施的安全性。使用标准工具对每项研究的偏倚风险进行了评估:在符合纳入标准的 71 篇出版物中,59 篇对药物干预措施进行了评估,12 篇对非药物干预措施进行了评估。我们发现中等质量的证据支持钙通道阻滞剂、磷酸二酯酶-5抑制剂和静脉注射前列环素类似物在减少RP发生频率、严重程度和持续时间方面的疗效。静脉注射伊洛前列素在改善 DU 愈合方面具有小到中等程度的效果。磷酸二酯酶-5抑制剂可有效减少DU总数、新DU发生率,并促进DU愈合。波生坦可有效预防 SSc 患者出现新的 DU。这些治疗方法没有引起新的安全问题。非药物干预研究的质量普遍较低,样本量也较小。保暖措施减少了RP发作的频率和持续时间;激光疗法改善了RP相关的预后;局部氧-臭氧疗法作为一种附加疗法改善了RP预后;骨髓单核细胞植入改善了DU相关的疼痛;动脉周围交感神经切除术和血管旁路术减少了DU的数量和手指截肢的风险:现有证据支持药物干预(即硝苯地平、西地那非、伊洛前列素和波生坦)治疗 SSc 和其他 CTD 患者 RP 和 DU 的有效性和安全性。稀少且低质量的证据确实支持使用一些非药物干预措施,但其效果并不显著。该 SLR 强调,用于确定最佳治疗方法的高质量证据非常有限。
{"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}
Carla Campinho Ferreira, Paulo Pereira, Margarida Correia, Emanuel Costa, Diogo Esperança Almeida, José Redondo Costa, Ana Roxo Ribeiro, Joana Leite Silva
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.
{"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}
Tânia Santiago, Ruben Fernandes, Ricardo Ferreira, Ioannis Parodis, Carina Bostrom
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).
{"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}