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Eosinophilic Granulomatosis with Polyangiitis - description of a pediatric patient with severe disease. 嗜酸性肉芽肿病伴多血管炎-描述一名患有严重疾病的儿科患者。
4区 医学 Q3 RHEUMATOLOGY Pub Date : 2023-04-01
Ana Barbosa Rodrigues, Filipa Oliveira-Ramos, Rosário Ferreira, Cristina Camilo, Tiago Oliveira, Patrícia Costa-Reis

Introduction: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare vasculitis of small and medium sized blood vessels.

Case description: Thirteen-year-old male, with history of rhinitis and asthma, who presented to the emergency room with one week of asthenia, arthralgias and myalgias and two days of fever. A diffuse petechial rash, palpable purpura and polyarthritis were detected on examination. Leukocytosis (34990/μL) with eosinophilia (66%) and elevated C-reactive protein were identified. The patient was admitted and ceftriaxone and doxycycline were started. The clinical status deteriorated in the following days. The patient developed myopericarditis, bilateral pulmonary infiltrates and pleural effusion, requiring mechanical ventilation and aminergic support. Non-clonal eosinophils were detected on the bone marrow aspiration and the skin biopsy showed leukocytoclastic vasculitis with eosinophils. Antineutrophil cytoplasmic antibodies and genetic analysis for hypereosinophilic syndrome mutations were negative. After treatment with methylprednisolone for three days a fast clinical, laboratory and radiological improvement occurred. The patient started azatiophrine and reduced steroids progressively. No relapses occurred since diagnosis five years ago.

Discussion: Clinical suspicion and early treatment of EGPA are crucial to improve prognosis.

简介:嗜酸性肉芽肿病合并多血管炎(EGPA)是一种罕见的中小型血管炎。病例描述:13岁男性,有鼻炎和哮喘病史,因一周的虚弱、关节痛和肌痛以及两天的发烧而就诊于急诊室。检查发现弥漫性点疹,可触及紫癜和多发性关节炎。白细胞增多(34990/μL),嗜酸性粒细胞增多(66%),c反应蛋白升高。患者入院并开始使用头孢曲松和强力霉素。随后几天临床情况恶化。患者出现心包炎、双侧肺浸润和胸腔积液,需要机械通气和胺能支持。骨髓穿刺检测到非克隆性嗜酸性粒细胞,皮肤活检显示白细胞分裂性血管炎伴嗜酸性粒细胞。抗中性粒细胞胞浆抗体和嗜酸性粒细胞增多综合征基因分析均为阴性。甲泼尼龙治疗3天后,临床、实验室和放射学均有快速改善。患者开始使用氮化硫啡并逐渐减少类固醇。自五年前诊断以来,未发生复发。讨论:临床怀疑和早期治疗EGPA是改善预后的关键。
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引用次数: 0
Severe infections in Portuguese patients with rheumatoid arthritis under biologic treatment - a multicenter, nationwide study (SIPPRA-B Study). 葡萄牙类风湿性关节炎患者在生物治疗下的严重感染——一项多中心、全国性研究(sipra - b研究)
4区 医学 Q3 RHEUMATOLOGY Pub Date : 2023-04-01
Filipe Oliveira Pinheiro, Maria Seabra Rato, Pedro Madureira, Filipe Araújo, Maria João Salvador, Vanessa Fraga, Luisa Brites, Filipe Cunha Santos, Augusto Silva, Ana Rita Lopes, Margarida Cruz, João Paulo Vilas Boas, Maria Pontes Ferreira, Beatriz Samões, Tiago Beirão, Inês Santos, Daniel Carvalho, Lúcia Costa, Miguel Bernardes

Introduction: Despite years of experience with biological disease modifying anti-rheumatic drugs (bDMARD) in rheumatoid arthritis (RA), little is known about differences in infectious risk among bDMARDs. The aim of this study was to assess the incidence and type of infections in RA patients on bDMARDs and to determine possible predictors.

Methods: A retrospective multicenter cohort study that included patients registered in the Rheumatic Diseases Portuguese Registry (Reuma.pt) with RA, and exposed to at least one bDMARD until April 2021. RA patients under bDMARD and with at least one episode of severe infection (SI), defined as infection that requires hospitalization, use of parenteral antibiotics or that resulted in death, were compared to patients with no report of SI. Demographic and clinical data at baseline and at the time of each SI were collected to establish comparisons between different groups of bDMARDs. Comparisons between different bDMARDs were assessed and logistic regression was performed to identify predictors of SI.

Results: We included 3394 patients, 2833 (83.5%) female, with a mean age at RA diagnosis of 45.5±13.7 years. SI was diagnosed in 142 of the 3394 patients evaluated (4.2%), totaling 151 episodes of SI. At baseline, patients with SI had a significantly higher proportion of prior orthopedic surgery, asthma, interstitial lung disease, chronic kidney disease and corticosteroid use, higher mean age and longer median disease duration at first bDMARD. Nine patients died (6.0%). Ninety-two SI (60.9%) occurred with the first bDMARD, the majority leading to discontinuation of the bDMARD within 6 months (n=75, 49.7%), while 65 (43.0%) restarted the same bDMARD and 11 (7.3%) switched to another bDMARD (6 of them to a different mechanism of action). In the multivariate analysis, we found that chronic kidney disease, asthma, infliximab, corticosteroid use, interstitial lung disease, previous orthopedic surgery, higher Health Assessment Questionnaire and DAS284V-ESR are independent predictors of SI.

Conclusion: This study described the incidence and types of SI among Portuguese RA patients on biologics, identifying several predictors of SI, both globally and with different bDMARDs. Physicians should be aware of the real-word infectious risk in RA patients on bDMARDs when making treatment decisions.

尽管生物疾病修饰抗风湿药物(bDMARD)治疗类风湿性关节炎(RA)已有多年的经验,但对bDMARD之间感染风险的差异知之甚少。本研究的目的是评估bdmard类风湿性关节炎患者感染的发生率和类型,并确定可能的预测因素。方法:一项回顾性多中心队列研究,纳入在葡萄牙风湿病登记处(Reuma.pt)登记的RA患者,并在2021年4月之前暴露于至少一种bDMARD。接受bDMARD治疗且至少有一次严重感染(SI)发作的RA患者与未报告SI的患者进行比较,严重感染定义为需要住院治疗、使用肠外抗生素或导致死亡的感染。收集基线和每次SI时的人口学和临床数据,以建立不同bdmard组之间的比较。评估不同bdmard之间的比较,并进行逻辑回归以确定SI的预测因子。结果:我们纳入3394例患者,其中2833例(83.5%)为女性,RA诊断时的平均年龄为45.5±13.7岁。3394例患者中有142例(4.2%)被诊断为SI,共151次SI发作。在基线时,SI患者既往骨科手术、哮喘、间质性肺疾病、慢性肾脏疾病和皮质类固醇使用的比例明显较高,首次bDMARD时的平均年龄较高,中位疾病持续时间较长。死亡9例(6.0%)。92例SI(60.9%)发生在第一次bDMARD中,大多数导致在6个月内停止bDMARD (n=75, 49.7%), 65例(43.0%)重新开始使用相同的bDMARD, 11例(7.3%)切换到另一个bDMARD(其中6例使用不同的作用机制)。在多因素分析中,我们发现慢性肾脏疾病、哮喘、英夫利昔单抗、皮质类固醇使用、间质性肺疾病、既往骨科手术、较高的健康评估问卷和DAS284V-ESR是SI的独立预测因素。结论:本研究描述了葡萄牙使用生物制剂的RA患者SI的发生率和类型,确定了全球和不同bdmard患者SI的几个预测因素。在做出治疗决定时,医生应该意识到服用bdmard的RA患者的真实感染风险。
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引用次数: 0
Pyogenic extensor tenosynovitis of the ankle with associated synovial cyst caused by Streptococcus pneumoniae - a case report. 由肺炎链球菌引起的化脓性踝关节伸肌腱滑膜炎伴滑膜囊肿1例。
4区 医学 Q3 RHEUMATOLOGY Pub Date : 2023-04-01
Sara Paiva Dinis, Francisca Guimarães, Hugo Parente, Catarina Dantas Soares, Maria Pontes Ferreira, Soraia Azevedo, Daniela Santos Faria, Daniela Peixoto, Carmo Afonso, Filipa Teixeira, José Tavares-Costa
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引用次数: 0
IgG4-related disease and isolated retroperitoneal fibrosis: A narrative review. igg4相关疾病和孤立性腹膜后纤维化:叙述性综述
4区 医学 Q3 RHEUMATOLOGY Pub Date : 2023-04-01
Almurtada Razok, Maria Emilia Romero Noboa, Faria Sami, Kirtan Nandlalbhai Patolia, Saman Tanveer

Retroperitoneal fibrosis (RPF) can occur due to many etiologies and is categorized into idiopathic and secondary. Etiologies of secondary RPF include medications, autoimmune disease, malignancy, and IgG4-related disease (IgG4-RD). Although IgG4-RD usually involves multiple systems synchronically including the pancreas, aorta, and kidneys, it can present with isolated RPF without involvement of other organ systems. Caution must be exercised in these instances as the diagnosis should be confirmed based on specific clinical, radiographic, and histopathologic criteria. Such confirmation can affect the work-up and therapeutic approach as treatment with corticosteroids can lead to remission, both clinically and radiographically.

腹膜后纤维化(RPF)可由多种病因引起,分为特发性和继发性。继发性RPF的病因包括药物治疗、自身免疫性疾病、恶性肿瘤和igg4相关疾病(IgG4-RD)。虽然IgG4-RD通常同时累及包括胰腺、主动脉和肾脏在内的多个系统,但它可以表现为孤立性RPF而不累及其他器官系统。在这些情况下必须谨慎行事,因为诊断应根据特定的临床、放射学和组织病理学标准来确定。这种确认可以影响检查和治疗方法,因为皮质类固醇治疗可以导致临床和放射学上的缓解。
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引用次数: 0
Effectiveness of biosimilar infliximab CT-P13 compared to originator infliximab in biological-naïve patients with rheumatoid arthritis and axial spondyloarthritis: data from the Portuguese Register. 英夫利昔单抗CT-P13生物仿制药与原药英夫利昔单抗在biological-naïve类风湿性关节炎和轴性脊柱性关节炎患者中的有效性:来自葡萄牙注册的数据
4区 医学 Q3 RHEUMATOLOGY Pub Date : 2023-04-01
José Marona, Alexandre Sepriano, Sofia Ramiro, Diogo Almeida, Luísa Brites, Maura Couto, Inês Cunha, Bruno Miguel Fernandes, Jorge Garcia, Ana Teresa Melo, Teresa Nóvoa, Margarida Oliveira, Patrícia Pinto, Maria José Santos, Cândida Silva, João Eurico Fonseca, Filipe César Araújo

Objectives: To compare the effectiveness of the infliximab biosimilar CT-P13 with originator infliximab over 24 months of follow-up in biological-naïve patients with rheumatoid arthritis (RA) and axial spondyloarthritis (axSpA).

Methods: Biological-naïve patients from the Rheumatic Diseases Portuguese Register (Reuma.pt), with a clinical diagnosis of RA or axSpA, who were starting either the infliximab biosimilar CT-P13 or the originator infliximab after 2014 (date of market entry of CT-P13 in Portugal), were included. Patients on biosimilar and originator were compared regarding different response outcomes at 3 and 6 months, adjusting for age, sex and baseline C-reactive protein (CRP). The main outcome was the change in DAS28-erytrocyte sedimentation rate (ESR) for RA and the ASDAS-CRP for axSpA. Additionally, the effect of infliximab biosimilar vs originator on different response outcomes over 24 months of follow-up was tested with longitudinal generalized estimating equations (GEE) models.

Results: In total, 140 patients were included, 66 (47%) of which with RA. The distribution of patients starting the infliximab biosimilar and the originator was the same between the two diseases (approximately 60% and 40%, respectively). From the 66 patients with RA, 82% were females, mean age was 56 years (SD 11) and mean DAS28-ESR 4.9 (1.3) at baseline. As for the patients with axSpA, 53% were males, mean age was 46 years (13) and mean ASDAS-CRP 3.7 (0.9) at baseline. There were no differences in efficacy between RA patients treated with the infliximab biosimilar and the originator, either at 3 months (∆DAS28-ESR: -0.6 (95% CI -1.3; 0.1) vs -1.2 (-2.0; -0.4)), or at 6 months (∆DAS28-ESR: -0.7 (-1.5; 0.0) vs -1.5 (-2.4; -0.7)). This was also true for patients with axSpA (∆ASDAS-CRP at 3 months: -1.6 (-2.0; -1.1) vs -1.4 (-1.8; -0.9) and at 6 months: -1.5 (-2.0; -1.1) vs -1.1 (-1.5; -0.7)). Results were similar with the longitudinal models over 24 months.

Conclusion: There are no differences in effectiveness between the infliximab biosimilar CT-P13 and the infliximab originator in the treatment of biological-naïve patients with active RA and axSpA in clinical practice.

目的:比较英夫利昔单抗生物仿制药CT-P13与原药英夫利昔单抗在biological-naïve类风湿性关节炎(RA)和轴性脊柱炎(axSpA)患者24个月的随访疗效。方法:Biological-naïve来自葡萄牙风湿病登记处(Reuma.pt)的临床诊断为RA或axSpA的患者,他们在2014年(CT-P13在葡萄牙上市的日期)之后开始使用英夫利昔单抗生物仿制药CT-P13或原药英夫利昔单抗。在调整年龄、性别和基线c反应蛋白(CRP)后,比较生物仿制药和原研药患者在3个月和6个月时的不同反应结果。主要结果是RA患者的das28 -红细胞沉降率(ESR)和axSpA患者的ASDAS-CRP的变化。此外,采用纵向广义估计方程(GEE)模型测试了英夫利昔单抗生物仿制药与原药对24个月随访期间不同缓解结果的影响。结果:共纳入140例患者,其中66例(47%)为RA。两种疾病中开始使用英夫利昔单抗生物仿制药和原药的患者分布相同(分别约为60%和40%)。66例RA患者中,82%为女性,平均年龄56岁(SD 11),基线时平均DAS28-ESR为4.9(1.3)。axSpA患者中53%为男性,平均年龄46岁(13岁),基线时ASDAS-CRP平均值为3.7(0.9)。使用英夫利昔单抗生物仿制药治疗的RA患者与原药治疗的RA患者的疗效没有差异,无论是在3个月时(∆DAS28-ESR: -0.6 (95% CI -1.3;0.1) vs -1.2 (-2.0;-0.4),或6个月时(∆DAS28-ESR: -0.7 (-1.5;0.0) vs -1.5 (-2.4;-0.7))。axSpA(∆ASDAS-CRP)患者在3个月时也是如此:-1.6 (-2.0;-1.1) vs -1.4 (-1.8;-0.9), 6个月时-1.5 (-2.0;-1.1) vs -1.1 (-1.5;-0.7))。结果与24个月的纵向模型相似。结论:英夫利昔单抗生物仿制药CT-P13与英夫利昔单抗原药治疗biological-naïve活动性RA和axSpA患者临床疗效无差异。
{"title":"Effectiveness of biosimilar infliximab CT-P13 compared to originator infliximab in biological-naïve patients with rheumatoid arthritis and axial spondyloarthritis: data from the Portuguese Register.","authors":"José Marona,&nbsp;Alexandre Sepriano,&nbsp;Sofia Ramiro,&nbsp;Diogo Almeida,&nbsp;Luísa Brites,&nbsp;Maura Couto,&nbsp;Inês Cunha,&nbsp;Bruno Miguel Fernandes,&nbsp;Jorge Garcia,&nbsp;Ana Teresa Melo,&nbsp;Teresa Nóvoa,&nbsp;Margarida Oliveira,&nbsp;Patrícia Pinto,&nbsp;Maria José Santos,&nbsp;Cândida Silva,&nbsp;João Eurico Fonseca,&nbsp;Filipe César Araújo","doi":"","DOIUrl":"","url":null,"abstract":"<p><strong>Objectives: </strong>To compare the effectiveness of the infliximab biosimilar CT-P13 with originator infliximab over 24 months of follow-up in biological-naïve patients with rheumatoid arthritis (RA) and axial spondyloarthritis (axSpA).</p><p><strong>Methods: </strong>Biological-naïve patients from the Rheumatic Diseases Portuguese Register (Reuma.pt), with a clinical diagnosis of RA or axSpA, who were starting either the infliximab biosimilar CT-P13 or the originator infliximab after 2014 (date of market entry of CT-P13 in Portugal), were included. Patients on biosimilar and originator were compared regarding different response outcomes at 3 and 6 months, adjusting for age, sex and baseline C-reactive protein (CRP). The main outcome was the change in DAS28-erytrocyte sedimentation rate (ESR) for RA and the ASDAS-CRP for axSpA. Additionally, the effect of infliximab biosimilar vs originator on different response outcomes over 24 months of follow-up was tested with longitudinal generalized estimating equations (GEE) models.</p><p><strong>Results: </strong>In total, 140 patients were included, 66 (47%) of which with RA. The distribution of patients starting the infliximab biosimilar and the originator was the same between the two diseases (approximately 60% and 40%, respectively). From the 66 patients with RA, 82% were females, mean age was 56 years (SD 11) and mean DAS28-ESR 4.9 (1.3) at baseline. As for the patients with axSpA, 53% were males, mean age was 46 years (13) and mean ASDAS-CRP 3.7 (0.9) at baseline. There were no differences in efficacy between RA patients treated with the infliximab biosimilar and the originator, either at 3 months (∆DAS28-ESR: -0.6 (95% CI -1.3; 0.1) vs -1.2 (-2.0; -0.4)), or at 6 months (∆DAS28-ESR: -0.7 (-1.5; 0.0) vs -1.5 (-2.4; -0.7)). This was also true for patients with axSpA (∆ASDAS-CRP at 3 months: -1.6 (-2.0; -1.1) vs -1.4 (-1.8; -0.9) and at 6 months: -1.5 (-2.0; -1.1) vs -1.1 (-1.5; -0.7)). Results were similar with the longitudinal models over 24 months.</p><p><strong>Conclusion: </strong>There are no differences in effectiveness between the infliximab biosimilar CT-P13 and the infliximab originator in the treatment of biological-naïve patients with active RA and axSpA in clinical practice.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":"2 2","pages":"132-140"},"PeriodicalIF":0.0,"publicationDate":"2023-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9809887","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
Hypokalemic paralysis due to renal tubular acidosis: uncommon initial manifestation of primary Sjögren´s syndrome. 肾小管酸中毒所致低钾血症性麻痹:原发性Sjögren综合征的罕见初始表现。
4区 医学 Q3 RHEUMATOLOGY Pub Date : 2023-04-01
Margarida Correia, Carla Campinho Ferreia, Emanuel Costa, Diogo Esperança Almeida, Joana Leite Silva, Ana Roxo Ribeiro, Sofia Marques, Marcos Cerqueira

Primary Sjögren´s Syndrome is an immune-mediated disease characterized by exocrine glands dysfunction due to lymphoplasmacytic infiltration with sicca symptoms being one of its main features. The disease may, however, present as distal renal tubular acidosis due to renal involvement, which can range from asymptomatic to life-threatening. We describe the case of a 33-year-old woman with hypokalemic paralysis and metabolic acidosis secondary to distal renal tubular acidosis, leading to the diagnosis of primary Sjögren´s Syndrome. Although rare, recognizing primary Sjögren´s Syndrome as a possible cause of distal renal tubular acidosis may elicit an earlier diagnosis and treatment, improving the patient´s prognosis.

原发性Sjögren综合征是一种免疫介导的疾病,以淋巴浆细胞浸润引起的外分泌腺功能障碍为特征,干燥症状是其主要特征之一。然而,由于肾脏受累,该病可能表现为远端肾小管酸中毒,其范围从无症状到危及生命。我们描述了一个33岁的女性低钾性麻痹和代谢性酸中毒继发于远端肾小管酸中毒,导致原发性Sjögren综合征的诊断。虽然罕见,但认识到原发性Sjögren’s综合征是远端肾小管酸中毒的可能原因可能会引起早期诊断和治疗,改善患者的预后。
{"title":"Hypokalemic paralysis due to renal tubular acidosis: uncommon initial manifestation of primary Sjögren´s syndrome.","authors":"Margarida Correia,&nbsp;Carla Campinho Ferreia,&nbsp;Emanuel Costa,&nbsp;Diogo Esperança Almeida,&nbsp;Joana Leite Silva,&nbsp;Ana Roxo Ribeiro,&nbsp;Sofia Marques,&nbsp;Marcos Cerqueira","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Primary Sjögren´s Syndrome is an immune-mediated disease characterized by exocrine glands dysfunction due to lymphoplasmacytic infiltration with sicca symptoms being one of its main features. The disease may, however, present as distal renal tubular acidosis due to renal involvement, which can range from asymptomatic to life-threatening. We describe the case of a 33-year-old woman with hypokalemic paralysis and metabolic acidosis secondary to distal renal tubular acidosis, leading to the diagnosis of primary Sjögren´s Syndrome. Although rare, recognizing primary Sjögren´s Syndrome as a possible cause of distal renal tubular acidosis may elicit an earlier diagnosis and treatment, improving the patient´s prognosis.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":"2 2","pages":"166-169"},"PeriodicalIF":0.0,"publicationDate":"2023-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9811905","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
Cumulative dose and length of methotrexate treatment were not shown to be predictors of hepatic fibrosis by elastography - a monocentric cohort study. 一项单中心队列研究显示,甲氨蝶呤的累积剂量和治疗时间并不能预测肝纤维化的弹性成像。
4区 医学 Q3 RHEUMATOLOGY Pub Date : 2023-02-22
Filipe Oliveira Pinheiro, Rui Gaspar, Bruno Miguel Fernandes, Armando Peixoto, Guilherme Macedo, Iva Brito

Objective: Methotrexate is used in several inflammatory diseases, such as rheumatoid arthritis (RA), spondyloarthritis (SpA) or inflammatory bowel disease (IBD). There has been some controversy regarding methotrexate liver toxicity, especially since the use of newer techniques. We aim to evaluate the prevalence of liver injury in methotrexate-treated patients with inflammatory diseases.

Methods: We performed a cross-sectional study where consecutive patients diagnosed with RA, SpA or IBD, treated with methotrexate, were submitted to liver elastography. The cutoff for fibrosis was ≥7.1 kPa. Comparisons between groups were evaluated using chi-square, t test and Mann-Whitney U test. Correlations were made between continuous variables using Spearman correlation. Logistic regression was performed to determine predictors of fibrosis.

Results: A total of 101 patients were included, 60 (59.4%) females, aged 46.2±12.6 years. Eleven patients (10.9%) had fibrosis, with a median score of 4.8 (4.1-5.9) kPa. Patients with fibrosis had higher rates of daily alcohol consumption (63.6% vs 31.1%, p=0.045). Methotrexate exposure time (OR 1.001, 95% CI 0.999-1.003, p=0.549) and cumulative dose (OR 1.000, 95% CI 1000-1000, p=0.629) were shown not to be predictors of fibrosis, unlike alcohol (OR 3.875, 95% CI 1.049-14.319, p=0.042). In multivariate logistic regression analysis, methotrexate cumulative and exposure times were not predictors of significant fibrosis, even when adjusted for alcohol consumption.

Conclusions: In this study, we found that fibrosis detected on hepatic elastography was not associated with methotrexate, unlike alcohol. Therefore, it is of paramount importance to redefine risk factors for liver toxicity in patients with inflammatory diseases under treatment with methotrexate.

目的:甲氨蝶呤用于治疗多种炎症性疾病,如类风湿性关节炎(RA)、脊柱关节炎(SpA)或炎症性肠病(IBD)。关于甲氨蝶呤的肝脏毒性一直存在争议,尤其是在使用较新技术之后。我们旨在评估甲氨蝶呤治疗的炎症性疾病患者肝损伤的发生率:我们进行了一项横断面研究,对连续诊断为 RA、SpA 或 IBD 并接受甲氨蝶呤治疗的患者进行了肝脏弹性成像检查。肝纤维化的临界值为≥7.1 kPa。组间比较采用卡方检验、t检验和曼惠尼U检验。连续变量之间的相关性采用斯皮尔曼相关法。采用逻辑回归法确定纤维化的预测因素:共纳入 101 例患者,其中女性 60 例(59.4%),年龄(46.2±12.6)岁。11名患者(10.9%)出现纤维化,中位分值为4.8(4.1-5.9)kPa。纤维化患者每天饮酒的比例更高(63.6% vs 31.1%,P=0.045)。甲氨蝶呤暴露时间(OR 1.001,95% CI 0.999-1.003,p=0.549)和累积剂量(OR 1.000,95% CI 1000-1000,p=0.629)与酒精(OR 3.875,95% CI 1.049-14.319,p=0.042)不同,不是纤维化的预测因素。在多变量逻辑回归分析中,即使对饮酒量进行调整,甲氨蝶呤累积量和暴露时间也不能预测明显的纤维化:在这项研究中,我们发现肝弹性成像检测到的肝纤维化与甲氨蝶呤无关,而与酒精不同。因此,重新定义正在接受甲氨蝶呤治疗的炎症性疾病患者的肝脏毒性风险因素至关重要。
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引用次数: 0
Outcome of Uveitis in Juvenile Idiopathic Arthritis and Spondyloarthritis Patients - A 5-Year Follow-Up Study. 青少年特发性关节炎和脊椎关节炎患者葡萄膜炎的预后-一项5年随访研究。
4区 医学 Q3 RHEUMATOLOGY Pub Date : 2023-01-01
Filipe Oliveira Pinheiro, Mariana Leuzinger-Dias, Bruno Miguel Fernandes, Diogo Fonseca, Joana Vilaça, Luís Figueira, Iva Brito

Objective: Uveitis is a frequent complication of juvenile idiopathic arthritis (JIA) and spondyloarthritis (SpA). The aim of this study is to evaluate the prevalence and risk factors for complications associated with uveitis in patients with JIA and SpA.

Methods: A longitudinal, monocentric cohort study that included patients diagnosed with JIA and SpA who developed uveitis. Demographic, laboratory, and clinical data were collected including complications of uveitis, HLA-B27, antinuclear antibodies, erythrocyte sedimentation rate, C-reactive protein, visual acuity and DMARD treatment. Comparison between groups (complicated versus uncomplicated uveitis) was evaluated using chi-square, t test and Mann-Whitney U test. Logistic regression was performed to determine predictors of complications.

Results: A total of 270 patients were evaluated, of which 37 patients (13.7%) had uveitis and were included in this study. Twenty patients were female (54.1%), aged 11.9±8.7 years at diagnosis of SpA/JIA and 15.3±9.9 years at diagnosis of uveitis. Twenty-seven patients (73.0%) had a diagnosis of JIA (23 with oligoarticular disease) and in 12 patients (32.4%) uveitis was the first manifestation. Fifteen (40.5%) patients exhibited complications during follow-up period. Eleven patients (29.7%) underwent ophthalmologic surgery. Complications were significantly higher in patients with JIA (51.9% vs 10.0% in SpA, p=0.03), as was the need for surgery (40.7% vs 0%, p=0.02). Complications in JIA were significantly more frequent in patients who had uveitis as the initial presentation (50.0% vs 7.7%, p=0.03); no significant differences were found between the groups in the other variables studied. Univariate logistic regression analysis showed that uveitis as the first manifestation of JIA (OR 12.0, confidence interval 95% 1.21-118.89, p=0.03) is a significant predictor of complications.

Conclusion: We found higher rates of complications and need for ophthalmologic surgery in patients with JIA-associated uveitis. The initial presentation of JIA as uveitis is significantly associated with the occurrence of uveitis complications, so it is essential that there is a collaboration between ophthalmologist and rheumatologist in the diagnosis and treatment of these patients.

目的:葡萄膜炎是青少年特发性关节炎(JIA)和脊椎关节炎(SpA)的常见并发症。本研究的目的是评估JIA和SpA患者与葡萄膜炎相关并发症的患病率和危险因素。方法:一项纵向、单中心队列研究,包括JIA和SpA诊断为葡萄膜炎的患者。收集人口统计学、实验室和临床数据,包括葡萄膜炎并发症、HLA-B27、抗核抗体、红细胞沉降率、c反应蛋白、视力和DMARD治疗。采用卡方检验、t检验和Mann-Whitney U检验评价两组间(复杂与非复杂葡萄膜炎)的比较。采用逻辑回归确定并发症的预测因素。结果:共评估270例患者,其中37例(13.7%)有葡萄膜炎纳入本研究。女性20例(54.1%),诊断为SpA/JIA时年龄11.9±8.7岁,诊断为葡萄膜炎时年龄15.3±9.9岁。27例(73.0%)被诊断为JIA(23例合并少关节病),12例(32.4%)以葡萄膜炎为首发表现。随访期间出现并发症15例(40.5%)。11例患者(29.7%)行眼科手术。JIA患者的并发症明显更高(51.9% vs 10.0%, SpA, p=0.03),手术需求也更高(40.7% vs 0%, p=0.02)。以葡萄膜炎为首发症状的JIA患者的并发症明显更频繁(50.0% vs 7.7%, p=0.03);在研究的其他变量中,两组之间没有发现显著差异。单因素logistic回归分析显示,葡萄膜炎作为JIA的首发表现(OR 12.0,置信区间95% 1.21-118.89,p=0.03)是并发症的重要预测因子。结论:jia相关性葡萄膜炎患者并发症发生率较高,需要眼科手术治疗。JIA最初表现为葡萄膜炎与葡萄膜炎并发症的发生显著相关,因此眼科医生和风湿病专家在诊断和治疗这些患者时的合作至关重要。
{"title":"Outcome of Uveitis in Juvenile Idiopathic Arthritis and Spondyloarthritis Patients - A 5-Year Follow-Up Study.","authors":"Filipe Oliveira Pinheiro,&nbsp;Mariana Leuzinger-Dias,&nbsp;Bruno Miguel Fernandes,&nbsp;Diogo Fonseca,&nbsp;Joana Vilaça,&nbsp;Luís Figueira,&nbsp;Iva Brito","doi":"","DOIUrl":"","url":null,"abstract":"<p><strong>Objective: </strong>Uveitis is a frequent complication of juvenile idiopathic arthritis (JIA) and spondyloarthritis (SpA). The aim of this study is to evaluate the prevalence and risk factors for complications associated with uveitis in patients with JIA and SpA.</p><p><strong>Methods: </strong>A longitudinal, monocentric cohort study that included patients diagnosed with JIA and SpA who developed uveitis. Demographic, laboratory, and clinical data were collected including complications of uveitis, HLA-B27, antinuclear antibodies, erythrocyte sedimentation rate, C-reactive protein, visual acuity and DMARD treatment. Comparison between groups (complicated versus uncomplicated uveitis) was evaluated using chi-square, t test and Mann-Whitney U test. Logistic regression was performed to determine predictors of complications.</p><p><strong>Results: </strong>A total of 270 patients were evaluated, of which 37 patients (13.7%) had uveitis and were included in this study. Twenty patients were female (54.1%), aged 11.9±8.7 years at diagnosis of SpA/JIA and 15.3±9.9 years at diagnosis of uveitis. Twenty-seven patients (73.0%) had a diagnosis of JIA (23 with oligoarticular disease) and in 12 patients (32.4%) uveitis was the first manifestation. Fifteen (40.5%) patients exhibited complications during follow-up period. Eleven patients (29.7%) underwent ophthalmologic surgery. Complications were significantly higher in patients with JIA (51.9% vs 10.0% in SpA, p=0.03), as was the need for surgery (40.7% vs 0%, p=0.02). Complications in JIA were significantly more frequent in patients who had uveitis as the initial presentation (50.0% vs 7.7%, p=0.03); no significant differences were found between the groups in the other variables studied. Univariate logistic regression analysis showed that uveitis as the first manifestation of JIA (OR 12.0, confidence interval 95% 1.21-118.89, p=0.03) is a significant predictor of complications.</p><p><strong>Conclusion: </strong>We found higher rates of complications and need for ophthalmologic surgery in patients with JIA-associated uveitis. The initial presentation of JIA as uveitis is significantly associated with the occurrence of uveitis complications, so it is essential that there is a collaboration between ophthalmologist and rheumatologist in the diagnosis and treatment of these patients.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":"2 1","pages":"17-21"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9350832","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
Arthritis or maybe not? Pachydermodactyly: the great mimicker of juvenile idiopathic arthritis. 是不是关节炎?厚皮性:幼年特发性关节炎的伟大模仿者。
4区 医学 Q3 RHEUMATOLOGY Pub Date : 2023-01-01
Ana Bento da Silva, Maria Helena Lourenço, Maria João Gonçalves, Jaime Cunha Branco, Manuela Costa, Ana Filipa Mourão

Arthritis in the paediatric population is the hallmark of many rheumatic inflammatory diseases, as well as other cutaneous, infectious, or neoplastic conditions. It can be quite devastating, whereby prompt recognition and treatment of these disorders are essential. However, arthritis can sometimes be mistaken for other cutaneous or genetic conditions leading to misdiagnosis and overtreatment. Pachydermodactyly is a rare and benign form of digital fibromatosis, usually manifested by swelling of the proximal interphalangeal joints of both hands, mimicking arthritis. The authors report a case of a 12-year-old boy with a one-year history of painless swelling of the proximal interphalangeal joints of both hands that was referred to the Paediatric Rheumatology department due to the suspicion of juvenile idiopathic arthritis. The diagnostic work-up was unremarkable, and the patient remained asymptomatic over an 18-month follow-up period. A diagnosis of pachydermodactyly was assumed and no treatment was introduced, given the benign nature of the disorder and absence of symptoms. Therefore, it was possible to safely discharge the patient from the Paediatric Rheumatology clinic.

在儿童人群中,关节炎是许多风湿性炎症性疾病以及其他皮肤、感染性或肿瘤性疾病的标志。它可能具有相当的破坏性,因此及时识别和治疗这些疾病是至关重要的。然而,关节炎有时会被误认为是其他皮肤或遗传疾病,导致误诊和过度治疗。厚皮畸形是一种罕见的良性手指纤维瘤病,通常表现为双手近端指间关节肿胀,类似关节炎。作者报告了一个12岁的男孩,有一年的双手近端指间关节无痛肿胀史,由于怀疑青少年特发性关节炎而被转介到儿科风湿病科。诊断检查无显著差异,患者在18个月的随访期间无症状。假定诊断为厚皮畸形,鉴于疾病的良性性质和无症状,没有进行治疗。因此,可以安全地将患者从儿科风湿病诊所出院。
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引用次数: 0
Tuberculous Osteomyelitis of The Pubic Symphysis - a Case Report of a Rare Entity Mimicking Spondyloarthritis. 耻骨联合结核性骨髓炎-一罕见的模拟脊柱炎的病例报告。
4区 医学 Q3 RHEUMATOLOGY Pub Date : 2023-01-01
Filipe Oliveira Pinheiro, Pedro Madureira, Maria Seabra Rato, Lúcia Costa

Tuberculosis (TB) osteomyelitis of the pubic symphysis is an extremely rare diagnosis. Axial spondyloarthritis (SpA) is characterized by inflammatory back pain and enthesitis, and involvement of pubic symphysis is very unusual at presentation. A 36-year-old female patient with a history of inflammatory back and pubic pain was referred to Rheumatology. She had a pelvic magnetic resonance imaging (MRI) suggestive of osteitis pubis. She was started on etoricoxib 90mg/day as axial spondyloarthritis was suspected, with no improvement. Pelvic MRI was repeated and showed osteomyelitis of the iliopubic branches. An ultrasound-guided biopsy was performed, and culture was positive for Mycobacterium tuberculosis. Further imaging studies revealed small cavitations and several centrilobular micronodules with a tree-in-bud pattern in the upper lung lobes and in the upper segment of the lower left lobe. She was started on anti-tuberculous treatment for 1 year and had a good clinical and radiological response. TB osteomyelitis of the pubic symphysis is a rare entity and has seldom been reported. However, this is the first case, to our knowledge, where the clinical picture mimicked an itself unusual presentation of SpA.

耻骨联合的结核性骨髓炎是一种极为罕见的诊断。轴性脊柱炎(SpA)的特征是炎症性背痛和全身炎,在表现时累及耻骨联合是非常罕见的。一位36岁女性患者,有背部炎症和耻骨疼痛史,被转介至风湿病科。她有盆腔磁共振成像(MRI)提示耻骨炎。她开始服用依托妥昔布90mg/天,因为怀疑是轴性脊柱炎,没有改善。骨盆MRI复查显示髂耻骨支骨髓炎。超声引导下活检,结核分枝杆菌培养阳性。进一步的影像学检查显示肺上叶和左下叶上段有小空洞和几个小叶中心微结节,呈树状芽状。患者开始抗结核治疗1年,临床和放射学反应良好。耻骨联合结核骨髓炎是一种罕见的实体,很少被报道。然而,据我们所知,这是第一例临床图片模仿自身不寻常的SpA表现。
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引用次数: 0
期刊
ARP Rheumatology
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