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Overlapping clinical features of systemic juvenile idiopathic arthritis and SARS-CoV-2-related multisystem inflammatory syndrome in children. 小儿系统性特发性关节炎与sars - cov -2相关多系统炎症综合征的重叠临床特征
Q3 RHEUMATOLOGY Pub Date : 2023-01-01 DOI: 10.5114/reum/161185
Oksana Boyarchuk, Tetiana Kovalchuk

Introduction: Differential diagnosis of the systemic juvenile idiopathic arthritis (sJIA) is often complicated, because of the variability in clinical presentation and the absence of specific signs.

Material and methods: The PubMed/Medline and Scopus databases from the years 2013-2022 were analysed for full articles in English and the following key words were used: "juvenile idiopathic arthritis" and "MIS-C"; "juvenile idiopathic arthritis" and "Kawasaki disease". As an example of the problem the case description of a 3-year-old patient is presented.

Results: In the first step 167 publications were identified; however, after exclusion of duplicated articles and those not relevant to the topic, only 13 were included in the analysis. We analysed studies that describe overlapping clinical features of sJIA and Kawasaki disease (KD) or multisystem inflammatory syndrome in children (MIS-C). The main issues we discussed were the search for the specific features that would distinguish one disease from another. Fever refractory to intravenous immunoglobulin treatment was the most frequent indicator among the features of clinical courses. Among other clinical signs prolonged, recurrent fever, rash, an incomplete KD phenotype, Caucasian race, splenomegaly, and complicated macrophage activation syndrome also supported sJIA diagnosis. Among laboratory tests, high ferritin and serum interleukin-18 levels were found to be the most useful in differentiation. The present case demonstrates that prolonged, unexplained, recurrent fever with a specific pattern should be the reason to suspect sJIA.

Conclusions: Overlapping features of sJIA and SARS-CoV-2-related MIS-C complicates diagnosis in the era of the COVID-19 pandemic. Our case presentation adds symptoms of prolonged, spiking, unexplained, recurrent fever with a specific pattern for supporting systemic juvenile idiopathic arthritis diagnosis.

系统性青少年特发性关节炎(sJIA)的鉴别诊断往往是复杂的,因为临床表现的可变性和缺乏特异性体征。材料和方法:对2013-2022年PubMed/Medline和Scopus数据库的英文全文进行分析,使用以下关键词:“少年特发性关节炎”和“MIS-C”;“幼年特发性关节炎”和“川崎病”。作为问题的一个例子,病例描述的一个3岁的病人是提出。结果:第一步共识别167篇文献;然而,在排除重复文章和与主题无关的文章后,只有13篇被纳入分析。我们分析了描述sJIA和川崎病(KD)或儿童多系统炎症综合征(MIS-C)重叠临床特征的研究。我们讨论的主要问题是寻找将一种疾病与另一种疾病区分开来的具体特征。发热对静脉注射免疫球蛋白治疗的难治性是临床病程特征中最常见的指标。在其他临床症状中,长期反复发热、皮疹、不完全KD表型、高加索种族、脾肿大和复杂的巨噬细胞激活综合征也支持sJIA的诊断。在实验室测试中,高铁蛋白和血清白介素-18水平被发现对分化最有用。本病例表明,长时间的、不明原因的、具有特定模式的反复发热应是怀疑sJIA的原因。结论:新冠肺炎大流行时代sJIA与sars - cov -2相关MIS-C的重叠特征使诊断复杂化。我们的病例表现增加了长时间、尖峰、不明原因、反复发热的症状,具有支持系统性青少年特发性关节炎诊断的特定模式。
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引用次数: 2
Fibromyalgia - etiology, diagnosis and treatment including perioperative management in patients with fibromyalgia. 纤维肌痛 - 病因、诊断和治疗,包括纤维肌痛患者的围手术期管理。
IF 1.4 Q3 RHEUMATOLOGY Pub Date : 2023-01-01 Epub Date: 2023-05-10 DOI: 10.5114/reum/163094
Anna Dizner-Golab, Barbara Lisowska, Dariusz Kosson

Fibromyalgia (FM) is considered a multifactorial disorder/syndrome with not fully understood etiology. Chronic generalized pain is the main symptom. A broad spectrum of factors is proposed to explain the etiology. Its multifactorial nature is inherently associated with challenges in diagnosis and therapy. Various evidence of etiology has been evaluated with the aim of establishing a novel therapeutic approach. The main issue in the diagnosis and management is to focus on the evaluation of strict diagnostic criteria to minimize under- and overdiagnosis. Fibromyalgia is a challenge for perioperative management because of the increased risk of possible complications and poorer outcomes, including postoperative pain chronification. The authors have proposed an up-to-date evaluation of perioperative management considering the current guidelines. Multimodal analgesia combined with tailored perioperative care is the most appropriate assessment. Interdisciplinary research with special interest in pain management, including perioperative medicine, seems to be the main theme for the future.

纤维肌痛(FM)被认为是一种多因素疾病/综合征,病因尚未完全明了。慢性全身疼痛是其主要症状。人们提出了多种因素来解释其病因。其多因素的性质本身就给诊断和治疗带来了挑战。我们对各种病因证据进行了评估,目的是建立一种新的治疗方法。诊断和管理的主要问题是重点评估严格的诊断标准,以尽量减少漏诊和误诊。纤维肌痛是围手术期管理的一个挑战,因为可能出现并发症和较差结果的风险增加,包括术后疼痛慢性化。作者根据现行指南,提出了围手术期管理的最新评估方法。多模式镇痛与量身定制的围手术期护理相结合是最合适的评估方法。对疼痛管理(包括围手术期医学)特别感兴趣的跨学科研究似乎是未来的主题。
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引用次数: 0
Matrix metalloproteinases in rheumatoid arthritis and osteoarthritis: a state of the art review. 基质金属蛋白酶在类风湿关节炎和骨关节炎中的研究进展。
Q3 RHEUMATOLOGY Pub Date : 2023-01-01 DOI: 10.5114/reum/168503
Łukasz Pulik, Paweł Łęgosz, Gabriela Motyl

Although the pathological mechanisms involved in osteoarthritis (OA) and rheumatoid arthritis (RA) are different, the onset and progression of both diseases are associated with several analogous clinical manifestations, inflammation, and immune mechanisms. In both diseases, cartilage destruction is mediated by matrix metalloproteinases (MMPs) synthesized by chondrocytes and synovium fibroblasts. This review aims to summarize recent articles regarding the role of MMPs in OA and RA, as well as the possible methods of targeting MMPs to alleviate the degradation processes taking part in OA and RA. The novel experimental MMP-targeted treatments in OA and RA are MMP inhibitors eg. 3-B2, taraxasterol, and naringin, while other treatments aim to silence miRNAs, lncRNAs, or transcription factors. Additionally, other recent MMP-related developments include gene polymorphism of MMPs, which have been linked to OA susceptibility, and the MMP-generated neoepitope of CRP, which could serve as a biomarker of OA progression.

尽管骨关节炎(OA)和类风湿性关节炎(RA)的病理机制不同,但这两种疾病的发病和进展都与一些类似的临床表现、炎症和免疫机制有关。在这两种疾病中,软骨破坏都是由软骨细胞和滑膜成纤维细胞合成的基质金属蛋白酶介导的。本文综述了近年来关于MMPs在OA和RA中的作用的文章,以及针对MMPs缓解OA和RA中的降解过程的可能方法。新的实验性MMP靶向治疗OA和RA的方法是MMP抑制剂。3-B2, taraxasterol和naringin,而其他治疗旨在沉默mirna, lncrna或转录因子。此外,最近其他与mmp相关的进展包括与OA易感性相关的mmp基因多态性,以及mmp产生的CRP新表位,它可以作为OA进展的生物标志物。
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引用次数: 4
EULAR. European Congress of Rheumatology: Milan, 31 May-3 June 2023. 欧拉。欧洲风湿病大会:米兰,2023年5月31日至6月3日。
Q3 RHEUMATOLOGY Pub Date : 2023-01-01 Epub Date: 2023-07-02 DOI: 10.5114/reum/169417
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引用次数: 0
Obesity in rheumatological practice. 风湿病实践中的肥胖。
Q3 RHEUMATOLOGY Pub Date : 2023-01-01 Epub Date: 2023-08-31 DOI: 10.5114/reum/170401
Jarosław Kozakowski, Piotr Dudek, Wojciech Zgliczyński

Obesity is a chronic disease that leads to the development of secondary metabolic disturbances and diseases and strongly contributes to increased morbidity and mortality. On the other hand, musculoskeletal disorders are currently the main cause of disability and the second most frequent reason for visits to the doctor. Many studies clearly show that excessive body weight adversely affects the course of almost all musculoskeletal system diseases, from osteoarthritis, through metabolic, systemic connective tissue, to rarely diagnosed diseases. The impact of increased fat mass on the musculoskeletal system is presumably complex in nature and involves the influence of biomechanical, dietary, genetic, inflammatory and metabolic factors. Due to the epidemic nature of obesity and its serious health consequences, this disease requires energetic treatment. It is always based on lifestyle modification enriched, if necessary, by pharmacological and, in justified cases, surgical treatment.

肥胖是一种慢性疾病,会导致继发性代谢紊乱和疾病的发展,并严重增加发病率和死亡率。另一方面,肌肉骨骼疾病是目前导致残疾的主要原因,也是就诊的第二常见原因。许多研究清楚地表明,超重会对几乎所有肌肉骨骼系统疾病的病程产生不利影响,从骨关节炎到代谢性系统结缔组织,再到很少被诊断的疾病。脂肪量增加对肌肉骨骼系统的影响可能是复杂的,涉及生物力学、饮食、遗传、炎症和代谢因素的影响。由于肥胖的流行性及其严重的健康后果,这种疾病需要大力治疗。它总是以改变生活方式为基础,必要时通过药物治疗,在合理的情况下,通过手术治疗。
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引用次数: 0
The significance of prolactin in systemic connective tissue diseases. 催乳素在系统性结缔组织疾病中的意义。
Q3 RHEUMATOLOGY Pub Date : 2023-01-01 Epub Date: 2023-08-31 DOI: 10.5114/reum/170319
Michał Piotr Jakubaszek

Objectives: Does prolactin (PRL) level testing in the diagnosis of systemic connective tissue diseases make sense and should we test it in everyday practice?

Material and methods: Connective tissue diseases (CTDs) are a group of heterogeneous disorders, involving multiple body systems. Rheumatoid arthritis (RA) is one of the most common connective tissue diseases with a global prevalence of 0.3-1% and can be inherited. Less common are systemic lupus erythematosus (SLE), antiphospholipid syndrome (APS), systemic sclerosis (SSc), primary Sjögren's syndrome (pSS) and inflammatory myositis. Prolactin is responsible for lactation, breast growth and many other bodily processes, and is elevated in blood of woman who are pregnant or breastfeeding. Hyperprolactinemia is relatively common in women, so some rheumatic diseases may be caused by high prolactin levels, and it should be detected during diagnosis.

Results: Prolactin signals are found in arthritic joint tissues (chondrocytes and synovial fibroblasts) to inhibit cartilage degradation, synovitis and osteoclastogenesis. On the other hand, hyperprolactinemia also promotes the conversion of PRL to vasoinhibin, a fragment of PRL that directly stimulates and indirectly inhibits arthritis in a cell type-dependent manner. The role of the PRL/vasoinhibin axis in inflammatory arthritis should still be monitored and further research is needed to help elucidate the role of PRL in rheumatic diseases in order to ultimately develop new therapeutic interventions that can be tested in patients.

Conclusions: Nowadays, there are no clear indications for prolactin testing in the diagnosis of systemic connective tissue diseases. In the case of suspicion or confirmation of some systemic connective tissue diseases, such as rheumatoid arthritis or systemic lupus erythematosus, testing the prolactin level makes sense when severity of disease symptoms is observed.

目的:催乳素(PRL)水平检测在诊断系统性结缔组织疾病中有意义吗?我们应该在日常实践中进行检测吗?材料和方法:结缔组织疾病是一组异质性疾病,涉及多个身体系统。类风湿性关节炎(RA)是最常见的结缔组织疾病之一,全球患病率为0.3-1%,可遗传。不太常见的有系统性红斑狼疮(SLE)、抗磷脂综合征(APS)、系统性硬化症(SSc)、原发性干燥综合征(pSS)和炎性肌炎。催乳素负责哺乳、乳房生长和许多其他身体过程,并在怀孕或哺乳期妇女的血液中升高。高泌乳素血症在女性中相对常见,因此一些风湿性疾病可能是由高泌乳素水平引起的,应在诊断时进行检测。结果:关节炎关节组织(软骨细胞和滑膜成纤维细胞)中存在催乳素信号,可抑制软骨降解、滑膜炎和破骨细胞生成。另一方面,高泌乳素血症也促进PRL转化为血管抑制素,血管抑制素是PRL的一种片段,以细胞类型依赖的方式直接刺激和间接抑制关节炎。PRL/血管抑制素轴在炎症性关节炎中的作用仍需监测,需要进一步研究来帮助阐明PRL在风湿性疾病中的作用,以便最终开发出可在患者中测试的新的治疗干预措施。结论:目前,催乳素检测在诊断系统性结缔组织疾病方面没有明确的指征。在怀疑或确认某些系统性结缔组织疾病的情况下,如类风湿性关节炎或系统性红斑狼疮,当观察到疾病症状的严重程度时,检测催乳素水平是有意义的。
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引用次数: 0
Autoimmune polyendocrine syndromes associated with autoimmune rheumatic diseases. 与自身免疫性风湿性疾病相关的自身免疫性多内分泌综合征。
Q3 RHEUMATOLOGY Pub Date : 2023-01-01 Epub Date: 2023-08-31 DOI: 10.5114/reum/170266
Katarzyna Jankowska, Piotr Dudek, Małgorzata Stasiek, Katarzyna Suchta

Autoimmune polyendocrine syndromes (APSs), also called autoimmune polyglandular syndromes, are a group of autoimmune diseases characterized by the co-occurrence of dysfunctions of several (at least two) endocrine glands. They develop under the influence of environmental factors in genetically predisposed people. Autoimmune polyendocrine syndromes may accompany autoimmune rheumatic diseases and worsen their course - APS-2 and APS-3 are the most common. The APS-2 includes the coexistence of, e.g. Hashimoto's disease, celiac disease and rheumatoid arthritis (RA). In APS-3, rheumatic diseases such as RA, systemic lupus erythematosus, and Sjögren's syndrome may coexist with Hashimoto's disease, type 1 diabetes and hypogonadism or other endocrinopathies. Undiagnosed endocrine diseases may be the reason for the intensification of metabolic disorders observed in the course of rheumatic diseases, cause the ineffectiveness of rheumatological treatment and also increase the frequency of bone fractures due to osteoporosis, cardiovascular complications and even miscarriages when coexistent, e.g. Hashimoto's disease with hypothyroiditis, which increases the risk of pregnancy loss. It is important to be able to conduct an extensive interview, paying attention to the symptoms of possible endocrinopathy as well as the features of other autoimmune disorders in the physical examination (e.g. vitiligo or darkening of the skin in Addison's disease). Depending on the history and physical examination, screening for various APSs is advised.

自身免疫多内分泌综合征(APS),也称为自身免疫性多腺体综合征,是一组自身免疫性疾病,其特征是同时出现几个(至少两个)内分泌腺的功能障碍。它们是在遗传易感人群的环境因素的影响下发展起来的。自身免疫性多内分泌综合征可能伴随自身免疫性风湿性疾病,并使其病程恶化——APS-2和APS-3是最常见的。APS-2包括桥本病、乳糜泻和类风湿性关节炎(RA)的共存。在APS-3中,风湿性疾病如RA、系统性红斑狼疮和干燥综合征可能与桥本病、1型糖尿病和性腺功能减退症或其他内分泌疾病共存。未确诊的内分泌疾病可能是风湿性疾病过程中观察到的代谢紊乱加剧的原因,导致风湿病治疗无效,同时也增加了骨质疏松、心血管并发症甚至流产导致骨折的频率,例如患有甲状腺功能减退的桥本病,这增加了流产的风险。重要的是能够进行广泛的访谈,在体检中注意可能的内分泌疾病的症状以及其他自身免疫性疾病的特征(例如艾迪生病中的白癜风或皮肤变黑)。根据病史和体检情况,建议对各种APSs进行筛查。
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引用次数: 0
Pregnancy- and lactation-related osteoporosis: an important topic also for rheumatologists. 妊娠期和哺乳期相关的骨质疏松症:也是风湿病学家的一个重要话题。
Q3 RHEUMATOLOGY Pub Date : 2023-01-01 Epub Date: 2023-08-31 DOI: 10.5114/reum/171597
Waldemar Misiorowski
ObjectivesMaterial and methodsResultsConclusions
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引用次数: 0
Radiosynovectomy of the hip joint - preliminary experience. 髋关节放射滑膜切除术的初步经验。
Q3 RHEUMATOLOGY Pub Date : 2023-01-01 DOI: 10.5114/reum/166608
Marek Marcin Chojnowski, Danuta Owczarczak, Maria Teresa Płazińska, Marek Dedecjus, Leszek Królicki

Introduction: Radiosynovectomy (RSV) is a minimally invasive method of treating and controlling joint inflammation refractory to conventional pharmacotherapy. It consist in intraarticular injection of radioactive colloids which irradiate the inflamed synovial membrane to cause its subsequent involution. Despite the fact that hip joint involvement is quite common in systemic inflammatory arthropathies, hip joint RSVs are rarely performed. The aim of this paper is to assess to safety and efficacy of hip joint radioisotope treatment.

Material and methods: We retrospectively analyzed the effects of 21 hip joint RSVs performed in 14 patients (10 female, 4 male; aged 8 to 79; mean age 48 years). Before the RSV, all the patients underwent clinical and ultrasound examination. The radiosynovectomies were performed using rhenium-186 sulfide under ultrasound guidance. Each patient underwent post-therapeutic scintigraphy to assess intraarticular distribution of the radiopharmaceutical. The effects of the treatment were assessed clinically and ultrasonographically during at least 2 follow-up visits 3 and 6 months after the RSV.

Results: In 9 cases, we observed complete resolution of symptoms 3 and 6 months after the RSV. Four patients had only a partial response and required repeated treatment, and all responded well to the second RSV. In 4 patients the treatment had no significant effect, and no repeated treatment attempt was made. All the responders suffered from inflammatory arthropathies; the non-responders had osteoarthritis, with no history of systemic diseases. In all the patients, no significant adverse effects were observed; in particular there were no radiation burns or infections. All post-therapeutic scintigrams showed proper, intraarticular distribution of the radiopharmaceutical.

Conclusions: Radiosynovectomy of the hip joint in systemic joint diseases, especially performed using ultrasound-guidance, is a safe and effective treatment modality.

简介:放射滑膜切除术(RSV)是一种微创治疗和控制常规药物治疗难治性关节炎症的方法。它存在于关节内注射放射性胶体,照射发炎的滑膜,导致其随后的复旧。尽管髋关节受累在全身性炎性关节病中很常见,但髋关节rsv很少进行。目的是评价髋关节放射性同位素治疗的安全性和有效性。材料和方法:我们回顾性分析了14例患者(女性10例,男性4例;8至79岁;平均年龄48岁)。RSV发病前,所有患者均行临床及超声检查。超声引导下用硫化铼-186行放射滑膜切除术。每位患者均接受治疗后显像检查以评估放射性药物在关节内的分布。在RSV发生后3个月和6个月的至少2次随访期间进行临床和超声检查评估治疗效果。结果:我们观察到9例RSV感染后3个月和6个月症状完全缓解。4例患者只有部分反应,需要重复治疗,所有患者对第二次RSV反应良好。4例患者治疗无明显效果,无重复治疗尝试。所有应答者均患有炎性关节病;无应答者患有骨关节炎,无全身性疾病史。所有患者均未观察到明显不良反应;特别是没有辐射烧伤或感染。所有治疗后的闪烁图显示放射药物在关节内的正确分布。结论:髋关节放射线滑膜切除术治疗全身性关节疾病,尤其是超声引导下行放射线滑膜切除术是一种安全有效的治疗方式。
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引用次数: 0
Successful use of tadalafil in oligohydramnios associated with lupus pregnancy. 他达拉非在狼疮妊娠羊水过少的成功应用。
Q3 RHEUMATOLOGY Pub Date : 2023-01-01 DOI: 10.5114/reum/167432
Kushagra Gupta, Meenakshi Bansal, Kamal Kishore, Divya Goyal
, We present the case of a 26-year-old female patient, who came to us with complaints of generalized swelling in the legs and a malar rash on her face, during the 17 th week of gestation. The patient’s complaints had begun only 1 month before prior to which she was fine. On general examination mild pallor and pedal edema were observed. The initial vital state was stable. Investigation showed mild anemia with a hemoglobin level of 7.4 mg/ dl. Initial investigations are presented in Table I. Serum albumin levels were low (2.5 g/dl) and urine examination showed presence of proteinuria. 24-hour urine protein estimation was 8 g/day. Other significant results: ANA positivity (1 : 2560, homogenous) and raised anti-dsDNA levels (450 IU/ml) as well as positive direct Coombs test. The ENA was positive for anti-Sm, anti-SSA and anti-SSB antibodies and C3 (31 mg/dl) and C4 levels (8 mg/dl) were also low, suggestive of lupus flare. Echocardiography showed mild pericardial effusion. The patient was diagnosed with lupus nephritis with autoimmune hemolytic anemia and serositis. Ultra-sound abdomen assessment done at baseline showed a normal healthy fetus appropriate for gestational age
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引用次数: 0
期刊
Reumatologia
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