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Gender differences in organ involvement and survival in systemic sclerosis – experience of a EUSTAR center 系统性硬化症器官受累和生存率的性别差异——欧洲癌症研究中心的经验
Q4 Medicine Pub Date : 2021-06-30 DOI: 10.37897/rjr.2021.2.4
L. Groșeanu, A. Petre, A. Balanescu, V. Bojinca, D. Opriș-Belinski, F. Berghea, I. Saulescu, D. Mazilu, Sanziana Daia Iliescu, A. Borangiu, C. Constantinescu, C. Cobilinschi, M. Negru, M. Abobului, R. Ionescu
Introduction. The low overall prevalence of systemic sclerosis (SSc) and the low proportion of male patients have resulted in a scarcity of studies assessing sex differences in SSc patients, and contradictory results have often been observed. Material and method. We performed a retrospective observational study using data extract from the EULAR scleroderma trials and research (EUSTAR) cohort 096 . We looked at sex influence on disease characteristics at baseline and then focused on patients with at least 2 years of follow-up to estimate the effects of sex on disease progression and survival. Results. 173 patients with SSc were available for the baseline analyses. In the longitudinal analysis after a mean follow-up of 3.5(±0.65) years, male sex was associated with a higher risk of scleroderma renal crisis (OR:9.45 (1.49 to 59.69); p = 0.004), digital contractures (OR:8.2 (3.1 to 21.9); p < 0.001), arrhythmias (OR: 3.37 (1.36 to 8.34); p = 0.006), pulmonary fibrosis (OR: 3.56, (1.51 to 8.41); p = 0.003), pulmonary hypertension (OR: 3.01 (1.19 to 7.59); p = 0.016), severe vascular involvement (OR:2.86, (1.22 to 6.73); p = 0.013) and low ventricular ejection fraction (OR: 2.84, (1.2 to 6.73); p = 0.014). Males had significantly reduced survival time after diagnosis (p = 0.004). The most frequent causes of death were scleroderma renal crisis in males and pulmonary hypertension in females. Conclusions. Although more common in women, SSc appears as strikingly more severe in men. Our results demonstrate a higher risk of severe organ involvement and poor prognosis in men. These results raise the point of including sex in the management and the decision-making process.
介绍系统性硬化症(SSc)的总体患病率较低,男性患者比例较低,导致很少有研究评估SSc患者的性别差异,而且经常观察到相互矛盾的结果。材料和方法。我们使用EULAR硬皮病试验和研究(EUSTAR)队列096的数据摘录进行了一项回顾性观察性研究。我们在基线时观察了性别对疾病特征的影响,然后将重点放在至少随访2年的患者身上,以评估性别对疾病进展和生存的影响。后果173名SSc患者可用于基线分析。在平均随访3.5(±0.65)年后的纵向分析中,男性与硬皮病肾危象的风险较高相关(OR:9.45(1.49-59.69);p=0.004),数字合同(OR:8.2(3.1至21.9);p<0.001)、心律失常(OR:3.37(1.36至8.34);p=0.006)、肺纤维化(OR:3.56,(1.51至8.41);p=0.003)、肺动脉高压(OR:3.01(1.19-7.59);p=0.016),严重血管受累(OR:2.86,(1.22-6.73);p=0.013)和低心室射血分数(OR:2.84(1.2-6.73);p=0.014)。男性在诊断后的生存时间显著缩短(p=0.004)。最常见的死亡原因是男性的硬皮病肾危象和女性的肺动脉高压。结论。尽管SSc在女性中更常见,但在男性中似乎更严重。我们的研究结果表明,男性发生严重器官受累和预后不良的风险更高。这些结果提出了将性别纳入管理和决策过程的观点。
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引用次数: 0
Ultrasound of the hand in systemic sclerosis 系统性硬化症患者手部超声检查
Q4 Medicine Pub Date : 2021-06-30 DOI: 10.37897/rjr.2021.2.2
H. Popov, M. Tămaș, C. Pamfil, S. Rednic
Systemic sclerosis is a chronic connective tissue disease characterized by multi-organ involvement but the main clinical changes occur in the hands, secondary to skin, joint and microvascular damage. Therefore the hand received a special attention for imaging and especially for ultrasound evaluation. In rheumatology US become an extension of the clinical examination and particularly in systemic sclerosis it has been proven to help with a better assessment of the skin, blood vessels, joints and tendons involvement. This evolution was allowed by permanent improvement of technology along with expanding the range of ultrasound applications which happened especially in past decade.
系统性硬化症是一种以多器官受累为特征的慢性结缔组织疾病,但临床变化主要发生在手部,继发于皮肤、关节和微血管损伤。因此,手受到了特别的关注成像,特别是超声评估。在美国风湿病学成为临床检查的延伸,特别是在系统性硬化症中,它已被证明有助于更好地评估皮肤,血管,关节和肌腱的受累情况。这种演变是由于技术的不断改进以及超声应用范围的扩大,特别是在过去十年中。
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引用次数: 0
Scleroderma renal crisis as the sole presenting feature of systemic sclerosis in a postpartum woman 硬皮病肾危象是产后妇女系统性硬化症的唯一表现特征
Q4 Medicine Pub Date : 2021-06-30 DOI: 10.37897/rjr.2021.2.6
C. Cobilinschi, C. Cobilinschi, A. Constantinescu, R. Ionescu, D. Opriș-Belinski
Systemic sclerosis is a chronic autoimmune disorder characterized by multiorgan involvement, most notably of the skin through fibrosis and vasculopathy. One of its most feared complications requiring rapid intervention is scleroderma renal crisis, as it can be fatal in the absence of prompt treatment. A 34-year old woman presents with a history of acute renal failure and malignant hypertension occurring one month postpartum and no other scleroderma feature in the following 5 years. Eventually, skin, heart and lung involvement is observed, positive anti-ARN III polymerase antibodies and suggestive capillaroscopic findings. Immunosuppressive therapy with mycophenolate mofetil is initiated and later switched to off-label Rituximab, with significant improvement of disease manifestations. Regular patient monitoring for novel symptom occurrence and appropriate treatment adjustment is essential for optimal management of scleroderma.
系统性硬化症是一种慢性自身免疫性疾病,其特征是多器官受累,尤其是通过纤维化和血管病变引起的皮肤受累。需要快速干预的最令人担忧的并发症之一是硬皮病肾危象,因为如果不及时治疗,它可能会致命。一名34岁女性,产后一个月出现急性肾功能衰竭和恶性高血压病史,在接下来的5年中没有其他硬皮病特征。最终,观察到皮肤、心脏和肺部受累,抗ARN III聚合酶抗体阳性,并提示乳头镜检查结果。霉酚酸酯的免疫抑制治疗开始,后来改用非标签的利妥昔单抗,疾病表现显著改善。定期监测新症状的发生和适当的治疗调整对于硬皮病的最佳治疗至关重要。
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引用次数: 0
Naïve vs. switchers differences in treatment effectiveness in spondyloarthritis Naïve与switchers治疗脊椎关节炎疗效的差异
Q4 Medicine Pub Date : 2021-06-30 DOI: 10.37897/rjr.2021.2.5
Irina Andrada Craciun, Relu Liviu Craciun, S. Daia-Iliescu, R. Ionescu
Introduction. Treat-to-target concept in spondyloarthritis (SpA) is a highly disputed subject. Up to the present, there is no evaluation score that integrates the clinical and laboratory/imaging results for defining remission in SpA. This issue generates the need of identifying predictive factors of remission in SpA patients. Aim. The aim of this study is to assess the effectiveness of the biological treatment upon naïve patients versus switchers and to identify some difficulties in achieving remission. Material and method. 65 patients with SpA were enrolled in this retrospective study, using data from multiple-choice forms and medical reports from University Hospitals in Romania, between 2019-2021. Results. 65 patients with SpA, in treatment with one biological disease-modifying antirheumatic drug (bDMARD) were available for the baseline analysis. They were distributed into 3 groups, whether they changed the biological therapy: group 1 – naïve patients, group 2 – first-time switchers and group 3 – more than one-time switchers. BASDAI and ASDAS were calculated at baseline, 24 and 52 weeks for the 3 groups. The scores were higher for the switchers and the highest in the 3rd group. Retention time of the first bDMARD was compared between groups and between the biological therapies. Conclusions. Results showed that the best treatment response, as well as effectiveness, is reached by the naïve patients group. Greater retention time rate for the first bDMARD is associated with a greater chance of achieving remission.
介绍。治疗目标的概念在脊椎关节炎(SpA)是一个高度争议的主题。到目前为止,还没有一个综合临床和实验室/影像学结果的评估评分来定义SpA的缓解。这一问题产生了识别SpA患者缓解的预测因素的需求。的目标。本研究的目的是评估naïve患者与转换者生物治疗的有效性,并确定实现缓解的一些困难。材料和方法。这项回顾性研究纳入了65名SpA患者,使用了2019-2021年间罗马尼亚大学医院的多项选择表格和医疗报告的数据。结果:65例SpA患者接受一种生物疾病缓解抗风湿药物(bDMARD)治疗,可用于基线分析。根据是否改变生物疗法,将患者分为3组:1组(naïve患者)、2组(首次转换者)和3组(超过一次转换者)。3组分别在基线、24周和52周计算BASDAI和ASDAS。转换组的得分更高,第三组的得分最高。比较两组间及两种生物疗法间首次bDMARD的滞留时间。结论。结果显示,naïve患者组的治疗效果最好。首次bDMARD的保留时间越长,获得缓解的机会越大。
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引用次数: 0
COVID-19 in patients with chronic inflammatory rheumatic diseases: Do these patients have a higher risk of COVID-19 due to their underlying medication? 慢性炎性风湿性疾病患者中的新冠肺炎:这些患者是否因其潜在药物而患新冠肺炎的风险更高?
Q4 Medicine Pub Date : 2021-06-30 DOI: 10.37897/rjr.2021.2.1
Claudia Dragomir, A. Burlui, Geanina-Florica Popescu, Ioan-Teodor Dragoi, A. Cardoneanu, L. Macovei, E. Rezus
Since the first case was reported to the World Health Organization (in late December 2019 in Wuhan, Hubei Province, China), by mid-June 2021, there have been 175,541,600 confirmed cases of COVID-19 globally, including 3,798,361 reported deaths. Many of those infected with SARS-CoV-2 have an asymptomatic form of the disease or mild flu-like symptoms. In another category of patients, the disease may be more aggressive, with a severe form that can lead to acute respiratory distress syndrome (ARDS), respiratory failure, and even death with a negative impact on patients. The COVID-19 pandemic has arisen serious concerns in the rheumatology community regarding the management of immunosuppressed patients diagnosed with inflammatory rheumatic diseases. It has been stated that severe forms of COVID-19 occur as a result of exacerbated inflammation status and cytokine production. Nevertheless, it remains unclear whether the use of biological agents subjects the patient to a higher risk, or rather protects them against severe forms of the disease. According to the American College of Rheumatology, rheumatic diseases were not identified as a risk factor that predicted poor outcome in patients with COVID-19. However, various studies have certified that corticosteroid treatment in patients with chronic rheumatic diseases at a dose >10 mg/day is associated with an increased risk of infection as well as the possibility of developing more severe COVID-19 symptoms.
自向世界卫生组织报告第一例病例(2019年12月底在中国湖北省武汉市)以来,截至2021年6月中旬,全球新冠肺炎确诊病例已达175541600例,其中死亡3798361例。许多感染严重急性呼吸系统综合征冠状病毒2型的人都有无症状的疾病或轻微的流感样症状。在另一类患者中,这种疾病可能更具攻击性,其严重形式可能导致急性呼吸窘迫综合征(ARDS)、呼吸衰竭,甚至死亡,对患者产生负面影响。新冠肺炎大流行引起了风湿病界对诊断为炎性风湿性疾病的免疫抑制患者的管理的严重关注。有人指出,严重形式的新冠肺炎是由于炎症状态恶化和细胞因子产生而发生的。然而,目前尚不清楚生物制剂的使用是否会使患者面临更高的风险,或者更确切地说,是保护他们免受严重疾病的影响。根据美国风湿病学会的数据,风湿性疾病未被确定为预测新冠肺炎患者预后不良的危险因素。然而,各种研究证明,慢性风湿性疾病患者的皮质类固醇治疗剂量>10 mg/天与感染风险增加以及出现更严重新冠肺炎症状的可能性有关。
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引用次数: 0
Pregnancy, systemic lupus erythematosus and a short communication on labor complications as new onset of the disease 妊娠、系统性红斑狼疮和分娩并发症作为新发疾病的简短交流
Q4 Medicine Pub Date : 2021-06-30 DOI: 10.37897/rjr.2021.2.3
A. Simionescu, S. Daia-Iliescu
Systemic lupus erythematosus (SLE) occur frequently in women of fertile age. In the pathogenesis of SLE, estrogen plays an important role, hormonal changes such as pregnancy and the postpartum increase the risk of disease flares. Also, pregnancy in SLE patients carries a higher fetal risk compared with healthy women. Pregnancy outcome may be optimized by careful planning of the pregnancy and close follow-up of the mother and of the fetus. SLE is associated with high maternal and fetal risk especially when non-diagnosed before planning a pregnancy. Herein we present two cases of SLE manifested by preeclampsia and acute renal insufficiency during labor and postpartum period, with a difficult diagnosis after a few months of a worsening clinical situation.
系统性红斑狼疮(SLE)常见于育龄妇女。在SLE的发病机制中,雌激素起着重要的作用,孕期和产后等激素的变化增加了疾病发作的风险。此外,SLE患者的妊娠与健康妇女相比具有更高的胎儿风险。通过周密的妊娠计划和对母体和胎儿的密切随访,妊娠结局可能会得到优化。SLE与母体和胎儿的高风险相关,特别是在计划怀孕前未确诊的情况下。在此,我们报告了两例SLE,表现为先兆子痫和分娩和产后急性肾功能不全,在几个月的临床情况恶化后难以诊断。
{"title":"Pregnancy, systemic lupus erythematosus and a short communication on labor complications as new onset of the disease","authors":"A. Simionescu, S. Daia-Iliescu","doi":"10.37897/rjr.2021.2.3","DOIUrl":"https://doi.org/10.37897/rjr.2021.2.3","url":null,"abstract":"Systemic lupus erythematosus (SLE) occur frequently in women of fertile age. In the pathogenesis of SLE, estrogen plays an important role, hormonal changes such as pregnancy and the postpartum increase the risk of disease flares. Also, pregnancy in SLE patients carries a higher fetal risk compared with healthy women. Pregnancy outcome may be optimized by careful planning of the pregnancy and close follow-up of the mother and of the fetus. SLE is associated with high maternal and fetal risk especially when non-diagnosed before planning a pregnancy. Herein we present two cases of SLE manifested by preeclampsia and acute renal insufficiency during labor and postpartum period, with a difficult diagnosis after a few months of a worsening clinical situation.","PeriodicalId":33518,"journal":{"name":"Revista Romana de Reumatologie","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2021-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41848509","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 2
Pulmonary abnormalities on high-resolution computed tomography in patients with long standing rheumatoid arthritis 长期类风湿关节炎患者的高分辨率计算机断层扫描肺部异常
Q4 Medicine Pub Date : 2021-03-31 DOI: 10.37897/rjr.2021.1.3
I. Andronache, C. Suța, Sabina Ciocodei, I. Bulbuc, C. Mihailov, O. Arghir, M. Șuța, Constanta Romania Medimar Imagistic Services
Background. Rheumatoid arthritis (RA) is a systemic inflammatory disease, associated with a number of extra-articular organ manifestations. Pulmonary involvement is a frequent and severe extraarticular manifestations of rheumatoid arthritis. RA can affect lung parenchyma, airways and pleura. Objectives. To identify RA-related lung disease on chest computed tomography (CT). Material and methods. We performed high-resolution computed tomography (HRCT) on a total of 92 patients with longstanding RA. Results. The subjects were predominantely female (79.3%), the age at entry was 63.77 ±11.56 years, and 42.9% had a history of smoking. Disease duration was 15.00±11.55 years. Pulmonary CT abnormalities were found in 71 of the 92 patients (77.2%). The most common HRCT anomalies were: linear attenuation (reticulation) (52.11%), bronchiectasis andbronchial wall thickening (45%), nodular attenuation (39.43%) and pleural involvement (pleural effusion or thickening) (39.43%). Conclusions. We conclude that RA-related lung disease was commonly detected on chest CT imaging in longstanding RA patients.
背景。类风湿性关节炎(RA)是一种全身性炎症性疾病,与许多关节外器官表现相关。肺受累是类风湿性关节炎常见且严重的关节外表现。类风湿性关节炎可累及肺实质、气道和胸膜。目标。目的:在胸部计算机断层扫描(CT)上鉴别ra相关的肺部疾病。材料和方法。我们对92例长期RA患者进行了高分辨率计算机断层扫描(HRCT)。结果。研究对象以女性为主(79.3%),入组年龄为63.77±11.56岁,有吸烟史的占42.9%。病程15.00±11.55年。92例患者中有71例(77.2%)出现肺部CT异常。最常见的HRCT异常为线状衰减(网状)(52.11%)、支气管扩张和支气管壁增厚(45%)、结节性衰减(39.43%)和胸膜受累(胸腔积液或增厚)(39.43%)。结论。我们得出结论,RA相关的肺部疾病在长期RA患者的胸部CT成像中通常被检测到。
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引用次数: 0
Antiphospholipid syndrome and cancer 抗磷脂综合征与癌症
Q4 Medicine Pub Date : 2021-03-31 DOI: 10.37897/rjr.2021.1.2
R. Ionescu, B. Pharmacy
Antiphospholipid antibodies syndrome (APS) is an acquired prothrombotic status, that is due to the occurrence of a certain type of autoantibodies directed against negatively charged phospholipid structures – hence the name. Neoplasia is also characterized by a systemic procoagulant status, through various mechanisms. Obviously, both conditions may occur in the same patient, raising the question of a possible causal relation. Regardless of the potential liaison between the two events, clinicians caring for neoplastic patients as well as for APS patients, should be aware of the presence of both conditions in the same patient, as this has important therapeutic consequences.
抗磷脂抗体综合征(APS)是一种获得性凝血酶原状态,是由于出现了针对带负电荷磷脂结构的某种类型的自身抗体——因此得名。通过各种机制,肿瘤还表现为系统性促凝状态。显然,这两种情况都可能发生在同一名患者身上,这就提出了可能的因果关系问题。不管这两个事件之间的潜在联系如何,照顾肿瘤患者和APS患者的临床医生都应该意识到同一患者同时存在这两种情况,因为这会产生重要的治疗后果。
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引用次数: 0
Two case reports and a literature review on eosinophilic fasciitis 嗜酸性筋膜炎2例报告及文献复习
Q4 Medicine Pub Date : 2021-01-01 DOI: 10.37897/rjr.2021.1.6
D. Mazilu, I. Saulescu, R. Ionescu
Eosinophilic fasciitis is a rare disorder of unknown etiology and poorly understood pathogenesis. It may be triggered by excessive exercise, physical factors such as radiation therapy, exposure to certain medications, infections, the initiation of hemodialysis and some other medical conditions. Skin modifications appear with collagenous thickening of the subcutaneous fascia with typical aspect of “peau d’orange” and the “groove sign”. Arthritis, myalgia, myositis, neuropathies may occur. The majority of patients have peripheral blood eosinophilia. The diagnosis is confirmed with a full thickness incisional biopsy of skin and subcutaneous tissue down to the muscle surface and fascia. Systemic glucocorticoids are the mainstay of treatment, but some patients require additional immunosuppressive therapy. The long-term prognosis of this disease is unknown and in some cases the disease may releapse. We describe two patients with eosinophilic fasciitis, their evolution and complications, associating other pathologies: morphea and antiphospholipid syndrome, making the diagnosis and the treatment more challenging.
嗜酸性筋膜炎是一种罕见的疾病,病因不明,发病机制也不清楚。它可能由过度运动、放射治疗等物理因素、接触某些药物、感染、开始血液透析和其他一些医疗条件引发。皮肤改变表现为皮下筋膜胶原增厚,典型表现为“橘皮征”和“沟征”。可能发生关节炎、肌痛、肌炎、神经病变。多数患者外周血嗜酸性粒细胞增多。诊断是通过对皮肤和皮下组织进行全层切口活检,直至肌肉表面和筋膜。全身性糖皮质激素是主要的治疗方法,但有些患者需要额外的免疫抑制治疗。这种疾病的长期预后尚不清楚,在某些情况下,疾病可能会复发。我们描述了两例嗜酸性筋膜炎患者,他们的演变和并发症,相关的其他病理:嗜血病和抗磷脂综合征,使诊断和治疗更具挑战性。
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引用次数: 1
Uveitis as an extraarticular manifestation in spondyloarthritis 葡萄膜炎是脊椎关节炎的一种关节外表现
Q4 Medicine Pub Date : 2020-12-31 DOI: 10.37897/RJR.2020.4.1
R. Ionescu, B. Pharmacy
Spondylatrhritis is a multifaceted disease, primary involving the axial skeleton. But sometimes, the patient present either with articular involvement plus other organ invelovement, or with only extraarticular involvement. As the most frequent, uveitis is the main extraarticular manifestation (EAM) of spondylarthritis. This EAM is very important because it can be the first manifestation of SpA, so it may bring the patient to the doctor, be it an ophthalmologist or a rheumatologist. Diagnosing uveitis in the context of SpA, is of paramount importance also because anterior uveitis is an emergency, because it can lead to blindness. So, knowing the clinical appearance and the treatment of uveitis in the context of SpA is crucial for providing the best care for SpA patients.
脊柱炎是一种多面性疾病,主要累及中轴骨骼。但有时,患者表现为关节受累和其他器官受累,或仅关节外受累。葡萄膜炎是脊柱炎最常见的关节外表现(EAM)。这种EAM非常重要,因为它可能是SpA的第一个表现,所以它可能会把病人带到医生那里,无论是眼科医生还是风湿病医生。在SpA的背景下诊断葡萄膜炎是至关重要的,因为前葡萄膜炎是一种紧急情况,因为它可以导致失明。因此,了解SpA背景下葡萄膜炎的临床表现和治疗对于为SpA患者提供最佳护理至关重要。
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引用次数: 1
期刊
Revista Romana de Reumatologie
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