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[Pregnant patient with rheumatoid arthritis treated with methotrexate and infliximab]. [甲氨蝶呤联合英夫利昔单抗治疗类风湿关节炎孕妇]。
Pub Date : 2016-01-01
Marija Bakula, Mislav Cerovec, Krešimir Rukavina, Nada Čikeš, Branimir Anić

Tumor necrosis factor-alpha inhibitors have become an established therapeutic regimen for patientswith rheumatoid arthritis. Regarding their harmful potential they are classified as category B medications. Animalreproduction studies have failed to demonstrate a risk to the fetus and there are no adequate and well-controlledstudies in pregnant women. Disease-modifying antirheumatic drugs (DMARDs) are often used in combination withbiological therapy and treatment with methotrexate has shown good results. This antimetabolite is classified as acategory X drug and its teratogenic effect is well known. The incidence of inflammatory rheumatic diseases is significantly higher in women. There are many reports on pregnant patients treated with biological therapy, oft en in combinationwith DMARDs. The effects of such a therapy on reproductive health is a theme of debate, with controversialviews on the matter. We present a patient with rheumatoid arthritis whose pregnancy was discovered at 31 weeks ofgestation. During that period she had been treated with methotrexate and infliximab, with no adverse effects.

肿瘤坏死因子- α抑制剂已成为类风湿关节炎患者的一种既定治疗方案。考虑到它们的潜在危害,它们被归类为B类药物。动物繁殖研究未能证明对胎儿有风险,也没有对孕妇进行充分和良好控制的研究。改善疾病的抗风湿药物(DMARDs)常与生物疗法联合使用,甲氨蝶呤治疗已显示出良好的效果。该抗代谢物被列为X类药物,其致畸作用是众所周知的。炎症性风湿病在妇女中的发病率明显较高。有许多关于孕妇接受生物治疗的报道,甚至经常与DMARDs联合使用。这种疗法对生殖健康的影响是一个争论的主题,在这个问题上有争议的观点。我们报告一位在妊娠31周时发现怀孕的类风湿关节炎患者。在此期间,她曾接受甲氨蝶呤和英夫利昔单抗治疗,无不良反应。
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引用次数: 0
[CLASSIFICATION AND DIAGNOSIS OF AXIAL SPONDYLOARTHRITIDES – HISTORY, PRESENT STATE, AND PERSPECTIVES]. [轴型脊柱关节炎的分类和诊断——病史、现状和观点]。
Pub Date : 2016-01-01
Simeon Grazio

Spondyloarthritis (SpA) is a group of inflammatory rheumatic diseases that share some common genetic,clinical, serological, radiological, and prognostic features. Since the early 1960s, several classification criteria forSpA have been proposed, and some of them were also used for diagnostic purposes. The ASAS international group ofexperts established a set of classification criteria for SpA, dividing them into axial or peripheral, according to predominantinvolvement. The paradigmatic entity of axial SpA is ankylosing spondylitis, which is diagnosed in clinicalpractice with significant delay. Therefore the ASAS classification introduced the term “non-radiographic axial SpA”,which refers to changes in the sacroiliac joints seen on MRI, but not on radiograph. Although the ASAS classificationhas been widely accepted in the professional community, recently initiatives were raised suggesting changes and aimingat improvements. In this paper these objections are discussed, as well as the responses of experts who consider thatthese changes are not necessary.

脊柱炎(SpA)是一组具有一些共同的遗传、临床、血清学、放射学和预后特征的炎性风湿病。自20世纪60年代初以来,已经提出了几种spa的分类标准,其中一些也用于诊断目的。ASAS国际专家组为SpA建立了一套分类标准,根据主要受累程度将其分为轴向性或周边性。轴向SpA的典型实体是强直性脊柱炎,在临床实践中诊断显着延迟。因此,ASAS分类引入了“非影像学轴向SpA”这一术语,指的是骶髂关节在MRI上而非x线片上的变化。尽管ASAS分类已被专业社区广泛接受,但最近提出了建议更改和改进的倡议。本文讨论了这些反对意见,以及认为这些变化没有必要的专家的回应。
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引用次数: 0
[DIAGNOSTIC ULTRASOUND OF MUSCULOSKELETAL SYSTEM IN RHEUMATOLOGY IN CROATIA – CURRENT STATUS AND NEW PERSPECTIVES]. [克罗地亚风湿病中肌肉骨骼系统的超声诊断-现状和新观点]。
Pub Date : 2016-01-01
Porin Perić, Nadica Laktašić-Žerjavić

In recent years, diagnostic ultrasound of the musculoskeletal system (MSUS) has become almost inevitablein everyday clinical practice in rheumatology. Due to the efforts of the European League Against Rheumatism(EULAR), the use of MSUS has significantly increased in Europe. Unfortunately, there are still certain open issues relatedto MSUS, primarily regarding different ways of interpretation of US findings, the standardization of MSUS findings,and the lack of clear criteria for assessing the competency of sonographers and their certification.

近年来,在风湿病学的日常临床实践中,肌肉骨骼系统(MSUS)的超声诊断几乎是不可避免的。由于欧洲抗风湿病联盟(EULAR)的努力,MSUS的使用在欧洲显著增加。不幸的是,MSUS仍然存在一些悬而未决的问题,主要涉及对超声检查结果的不同解释方式,MSUS检查结果的标准化,以及缺乏评估超声医师能力及其认证的明确标准。
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引用次数: 0
[JUVENILE IDIOPATHIC ARTHRITIS]. [幼年特发性关节炎]。
Pub Date : 2016-01-01
Lana Tambić Bukovac, Marija Perica

Juvenile idiopathic arthritis (JIA) is the most common rheumatic disorder in children and one of themost common causes of part-time or long-term disability. The term juvenile idiopathic arthritis defines the main characteristicsof the disease: joint inflammation of unknown origin manifested before the 16th birthday and lasting for morethan six weeks. JIA is very rare in infancy, with highest frequency in preschool age. It is not a single disease, but a groupof disorders with some common features of different immunopathogenesis and with different clinical manifestations. Accordingto the revised International League of Associations for Rheumatology (ILAR) criteria, JIA is classified into 8subtypes, but this classification is still a “work in progress“ because with new knowledge gained in genetics and immunology,the classification will obviously have to be changed and refined. New research of the disease pathogenesis is the basisfor the development of new and better treatments for JIA. The goal of such treatments is not just to relieve pain, but alsoto control inflammation and stop irreversible joint damage and long-term disability. Biological agents have significantlyimproved the disease prognosis.

青少年特发性关节炎(JIA)是儿童中最常见的风湿性疾病,也是导致部分或长期残疾的最常见原因之一。幼年特发性关节炎定义了该疾病的主要特征:在16岁生日之前出现不明原因的关节炎症,持续6周以上。JIA在婴儿期非常罕见,以学龄前发生率最高。它不是一种单一的疾病,而是一组具有不同免疫发病机制和不同临床表现的共同特征的疾病。根据修订后的国际风湿病协会联盟(ILAR)标准,JIA被分为8个亚型,但这种分类仍然是“正在进行的工作”,因为随着遗传学和免疫学方面的新知识的获得,这种分类显然必须改变和完善。对疾病发病机制的新研究是开发新的更好的JIA治疗方法的基础。这种治疗的目的不仅是缓解疼痛,而且还能控制炎症,阻止不可逆的关节损伤和长期残疾。生物制剂显著改善了疾病预后。
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引用次数: 0
[JUVENILE SPONDYLOARTHRITIS]. [青少年脊椎关节炎]
Pub Date : 2016-01-01
Lovro Lamot, Miroslav Harjaček

Juvenile spondyloartrhritis is a group of multifactorial diseases in which a disturbed interplay occursbetween the immune system and environmental factors on a predisposing genetic background, which leads to inflammationand structural damage of the target tissue. First symptoms of jSpA rarely involve the spine, while asymmetricaloligoarthritis of lower extremities, dactylitis, and peripheral enthesitis are much more common. There are many classificationcriteria for jSpA, but the majority of pediatric rheumatologists currently use the International League AgainstRheumatism (ILAR) criteria according to which most patients with jSpA are classified into the enthesitis-related arthritisgroup of juvenile idiopathic arthritis. To meet these criteria, a patient should have arthritis and/or enthesitis,with two or more symptoms such as sacroiliac joint tenderness and/or inflammatory back pain, HLAB27 genotype,HLA B27 genotype-associated disease in a first- or second-degree relative, uveitis, and male sex with eight or moreyears of age. Therefore, diagnosis is most oft en made only based on clinical examination and medical history. Anti-nuclear antibodies (ANA), rheumatoid factor (RF), and HLA testing with B27, B7, and DR4 alleles are preferred. Sincesubclinical gut inflammation is present in many patients, it is recommended to check fecal calprotectin levels. In patientswith signs of peripheral enthesitis it is warranted to perform power Doppler musculoskeletal ultrasound (PDUS),and in patients with signs of axial involvement radiographic and contrast-enhanced magnetic resonance imaging.Most patients are treated with nonsteroidal anti-inflammatory drugs (NSAIDs) and physical therapy, while in refractorycases with peripheral disease synthetic disease- modifying antirheumatic drugs (DMARDs), such as sulfasalazine,are used. In patients with axial involvement, biological DMARDs such as adalimumab, infliximab, and etanercept areobligatory. Although a number of studies gave us a good insight into the disease pathogenesis, the response to treatmentand prognosis are still difficult to predict.

青少年脊柱炎是一组多因素疾病,在易感的遗传背景下,免疫系统和环境因素之间的相互作用受到干扰,导致靶组织的炎症和结构损伤。jSpA的首发症状很少累及脊柱,而下肢不对称寡关节炎、指突炎和周围性膝炎则更为常见。jSpA有许多分类标准,但大多数儿科风湿病学家目前使用国际抗风湿病联盟(ILAR)标准,根据该标准,大多数jSpA患者被归类为青少年特发性关节炎的麻炎相关关节炎组。要满足这些标准,患者必须患有关节炎和/或关节炎,伴有两种或两种以上的症状,如骶髂关节压痛和/或炎症性背痛,HLAB27基因型,HLA B27基因型相关的一或二度亲属疾病,葡萄膜炎,年龄在8岁或以上的男性。因此,诊断往往仅根据临床检查和病史。首选抗核抗体(ANA)、类风湿因子(RF)和HLA检测B27、B7和DR4等位基因。由于亚临床肠道炎症存在于许多患者,建议检查粪便钙保护蛋白水平。对于有外周性炎症征象的患者,有必要进行功率多普勒肌肉骨骼超声检查(PDUS),以及有轴向受累影像学和增强磁共振成像征象的患者。大多数患者接受非甾体抗炎药(NSAIDs)和物理治疗,而在伴有外周疾病的难治性病例中,则使用合成疾病修饰抗风湿药物(DMARDs),如磺胺吡啶。对于轴向受累的患者,生物dmard如阿达木单抗、英夫利昔单抗和依那西普是强制性的。虽然许多研究使我们对疾病的发病机制有了很好的了解,但对治疗的反应和预后仍然难以预测。
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引用次数: 0
[RHEUMATOLOGY IN PEDIATRIC CARDIOLOGY FROM FETAL STAGE TO ADULTHOOD. THE ROLE OF FETAL ECHOCARDIOGRAPHY]. [从胎儿期到成年期儿科心脏病学中的风湿病学]。胎儿超声心动图的作用]。
Pub Date : 2016-01-01
Ivan Malčić

Even though in the last ten years pediatric cardiology and rheumatology have formally separated inCroatia as well, strong links still remain between them. They are no longer manifested through rheumatic fever, butthrough other rheumatic entities: systemic lupus erythematosus, antiphospholipid syndrome, systemic scleroderma,Kawasaki disease, polyarteritis nodosa, and some forms of granulomatous vasculitis. We take special note of the occurrenceof complete congenital atrioventricular block (CCAVB) in pregnant women who, due to systemic connectivetissue diseases, develop distinctive ANA antibodies (anti-SSA/Ro and/or anti SSB/La), but who are also likely to havethe same inflammation within the heart, leading to the development of restricted cardiomyopathy associated withCCAVC. Although rheumatology has thus been involved with early fetal age for some time now, there are also someissues relating to the embryonal stage (the association between methotrexate/folic acid and heart development in embryos),as well as to the late fetal stage (antiprostaglandin anti-infl ammatory agent impact on premature ductus arteriosusclosure). We gave special attention to the neonatal lupus syndrome, the most serious complication in the fetalperiod. Thus the multiple association between cardiology and rheumatology in all age groups, from the embryonal andfetal stage to adulthood, is being realized. The aim of this paper is to present some important links and thus furtheremphasize the new cooperation between pediatric cardiology and rheumatology from fetal stage to adulthood.

尽管在过去的十年里,儿科心脏病学和风湿病学在克罗地亚也正式分离,但它们之间仍然保持着密切的联系。它们不再通过风湿热表现出来,而是通过其他风湿病实体表现出来:系统性红斑狼疮、抗磷脂综合征、系统性硬皮病、川崎病、结节性多动脉炎和某些形式的肉芽肿性血管炎。我们特别注意完全性先天性房室传导阻滞(CCAVB)在孕妇中的发生,由于全身性结缔组织疾病,产生独特的ANA抗体(抗ssa /Ro和/或抗SSB/La),但也可能在心脏内有相同的炎症,导致与ccavc相关的限制性心肌病的发展。虽然风湿病学已经与胎龄早期有关,但也存在一些与胚胎期有关的问题(甲氨蝶呤/叶酸与胚胎心脏发育之间的关系),以及与胎儿后期有关的问题(抗前列腺素抗炎药对动脉导管过早闭合的影响)。我们特别关注新生儿狼疮综合征,胎儿期最严重的并发症。因此,心脏病学和风湿病学在所有年龄组(从胚胎和胎儿阶段到成年)之间的多重关联正在实现。本文的目的是介绍一些重要环节,从而进一步强调从胎儿期到成年期儿科心脏病学与风湿病学之间的新合作。
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引用次数: 0
[EVALUATION OF PAIN AND LOCAL PHARMACOLOGICAL PAIN TREATMENT IN RHEUMATOLOGY]. [风湿病学中疼痛和局部药物治疗的评估]。
Pub Date : 2016-01-01
Tea Schnurrer-Luke-Vrbanić

The objective of this review is to present outcome measurement tools for chronic musculoskeletalpain in rheumatology patients and to provide an overview of local pharmacological pain treatment.Reliable and valid assessment of pain is fundamental for both clinical trials and effective pain management. Thecomplex nature of pain makes objective measurement impossible. Evaluation of chronic musculoskeletal pain and itsimpact on physical, emotional, and social functions requires multidimensional qualitative tools and health-relatedquality of life instruments. The main recommendation concerning outcome measurements of pain is that they shouldinclude an evaluation of pain, fatigue, disturbed sleep, physical functioning, emotional functioning, and patient globalratings of satisfaction and quality of life. Despite the growing field of new instruments and publications related to measuringthe various aspects of chronic pain, there is still little agreement on the topic among researchers and clinicalexperts and no unified approach has been adopted. There is still considerable need for the development of a core set ofmeasurement tools and response criteria regarding chronic pain management.It is well known that pain in articular joints and soft tissues of the musculoskeletal system represents the mostcommon symptom presenting to rheumatologists. Therefore, local pharmacological pain tretment has an importantrole in rheumatology treatment algorithms. Topical administration, as well as injection administration in joints andsoft tissue trigger points, can be done under the control of musculoskeletal ultrasound. The most frequently prescribeddrugs include NSARs and corticosteroids, with their effectiveness being well-proven in evidence-based practice.

本综述的目的是提供风湿病患者慢性肌肉骨骼疼痛的结果测量工具,并提供局部药物治疗疼痛的概述。可靠和有效的疼痛评估是临床试验和有效的疼痛管理的基础。疼痛的复杂性使得客观测量成为不可能。评估慢性肌肉骨骼疼痛及其对身体、情感和社会功能的影响需要多维的定性工具和与健康相关的生活质量仪器。关于疼痛结果测量的主要建议是,它们应该包括对疼痛、疲劳、睡眠紊乱、身体功能、情绪功能和患者满意度和生活质量的总体评分的评估。尽管有越来越多的新仪器和出版物与测量慢性疼痛的各个方面有关,但研究人员和临床专家在这一主题上仍然没有达成一致意见,也没有采用统一的方法。关于慢性疼痛管理,仍然需要开发一套核心的测量工具和反应标准。众所周知,关节和肌肉骨骼系统软组织的疼痛是风湿病学家最常见的症状。因此,局部疼痛药物治疗在风湿病治疗算法中占有重要地位。局部给药,以及关节和软组织触发点的注射给药,可以在肌肉骨骼超声的控制下完成。最常用的处方药包括NSARs和皮质类固醇,它们的有效性在循证实践中得到了很好的证明。
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引用次数: 0
[JUVENILE SPONDYLOARTHRITIS]. [青少年脊椎关节炎]
Pub Date : 2016-01-01 DOI: 10.5772/39149
Lovro Lamot, Miroslav Harjaček
Juvenile spondyloartrhritis is a group of multifactorial diseases in which a disturbed interplay occurs between the immune system and environmental factors on a predisposing genetic background, which leads to inflammation and structural damage of the target tissue. First symptoms of jSpA rarely involve the spine, while asymmetrical oligoarthritis of lower extremities, dactylitis, and peripheral enthesitis are much more common. There are many classification criteria for jSpA, but the majority of pediatric rheumatologists currently use the International League Against Rheumatism (ILAR) criteria according to which most patients with jSpA are classified into the enthesitis-related arthritis group of juvenile idiopathic arthritis. To meet these criteria, a patient should have arthritis and/or enthesitis, with two or more symptoms such as sacroiliac joint tenderness and/or inflammatory back pain, HLAB27 genotype, HLA B27 genotype-associated disease in a first- or second-degree relative, uveitis, and male sex with eight or more years of age. Therefore, diagnosis is most oft en made only based on clinical examination and medical history. Anti- nuclear antibodies (ANA), rheumatoid factor (RF), and HLA testing with B27, B7, and DR4 alleles are preferred. Since subclinical gut inflammation is present in many patients, it is recommended to check fecal calprotectin levels. In patients with signs of peripheral enthesitis it is warranted to perform power Doppler musculoskeletal ultrasound (PDUS), and in patients with signs of axial involvement radiographic and contrast-enhanced magnetic resonance imaging. Most patients are treated with nonsteroidal anti-inflammatory drugs (NSAIDs) and physical therapy, while in refractory cases with peripheral disease synthetic disease- modifying antirheumatic drugs (DMARDs), such as sulfasalazine, are used. In patients with axial involvement, biological DMARDs such as adalimumab, infliximab, and etanercept are obligatory. Although a number of studies gave us a good insight into the disease pathogenesis, the response to treatment and prognosis are still difficult to predict.
青少年脊柱炎是一组多因素疾病,在易感的遗传背景下,免疫系统和环境因素之间的相互作用受到干扰,导致靶组织的炎症和结构损伤。jSpA的首发症状很少累及脊柱,而下肢不对称寡关节炎、指突炎和周围性膝炎则更为常见。jSpA有许多分类标准,但大多数儿科风湿病学家目前使用国际抗风湿病联盟(ILAR)标准,根据该标准,大多数jSpA患者被归类为青少年特发性关节炎的麻炎相关关节炎组。要满足这些标准,患者必须患有关节炎和/或关节炎,伴有两种或两种以上的症状,如骶髂关节压痛和/或炎症性背痛,HLAB27基因型,HLA B27基因型相关的一或二度亲属疾病,葡萄膜炎,年龄在8岁或以上的男性。因此,诊断往往仅根据临床检查和病史。首选抗核抗体(ANA)、类风湿因子(RF)和HLA检测B27、B7和DR4等位基因。由于亚临床肠道炎症存在于许多患者,建议检查粪便钙保护蛋白水平。对于有外周性炎症征象的患者,有必要进行功率多普勒肌肉骨骼超声检查(PDUS),以及有轴向受累影像学和增强磁共振成像征象的患者。大多数患者接受非甾体抗炎药(NSAIDs)和物理治疗,而在伴有外周疾病的难治性病例中,则使用合成疾病修饰抗风湿药物(DMARDs),如磺胺吡啶。对于轴向受累的患者,生物dmard如阿达木单抗、英夫利昔单抗和依那西普是强制性的。虽然许多研究使我们对疾病的发病机制有了很好的了解,但对治疗的反应和预后仍然难以预测。
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引用次数: 15
[VAGAL NERVE STIMULATION IN THE TREATMENT OF PATIENTS WITH RHEUMATOID ARTHRITIS – RESULTS THROUGH DAY 84 OBTAINED AT THE CROATIAN CENTER OF AN INTERNATIONAL PILOT STUDY]. [迷走神经刺激在类风湿关节炎患者治疗中的应用——克罗地亚国际试点研究中心84天的研究结果]。
Pub Date : 2016-01-01
Ines Doko, Simeon Grazio, Frane Grubišić, Ralph Zitnik

Objective: Electrical stimulation of the vagus has proven effective in various inflammatory conditions in animalmodels. The aim of this study is to show the effect of vagal nerve neurostimulation on clinical and laboratory parameters in two patients with active rheumatoid arthritis (RA) and an inadequate response to methotrexate.

Patients and methods: The research was conducted as part of an international pilot study. Patients were implanted with the Cyberonics system for electrical stimulation of the vagus. After an initial in-clinic stimulation, the patientsperformed the stimulations at home for 42 days, when the device was inactivated. On day 56 the stimulations were reinitiated. The following parameters were evaluated: tender and swollen joint count, physician’s (PGA) and patient’s (PtGA) global score, intensity of pain, disease activity (DAS28), functional ability (HAQ), serum CRP level, and EULAR response.

Results: In the period from the screening visit to the day 42 visit, both patients experienced an improvement ofDAS28 (7.00 and 6.22 vs. 4.03 and 2.13), PGA (70 and 53 vs. 27 and 16), PtGA (48 and 43 vs. 15 and 14), tender joint count (26 and 28 vs. 4 and 0), swollen joint count (24 and 14 vs. 8 and 2), intensity of pain (72 and 87 vs 21 and 7), HAQ score (2.25 and 2.25 vs. 1.5 and 1.375), and CRP levels (23.8 and 5.58 vs. 13 and 4.61). After the device deactivation, DAS28 and VAS pain worsened in both patients.

Conclusion: Vagal neural stimulation in the treatment of patients with active RA and an inadequate response tomethotrexate is effective in reducing clinical symptoms and parameters of inflammation. Our results are in accordance with the results obtained in other centers. Research on a larger number of subjects is necessary for a better evaluation of the effect of this new approach to the treatment of patients with rheumatoid arthritis.

目的:在动物模型中,电刺激迷走神经已被证明对各种炎症情况有效。本研究的目的是显示迷走神经刺激对两例活动性类风湿关节炎(RA)患者临床和实验室参数的影响,并对甲氨蝶呤反应不足。患者和方法:该研究是一项国际试点研究的一部分。病人被植入了控制论系统来电刺激迷走神经。在最初的临床刺激之后,患者在家中进行了42天的刺激,此时设备已停用。在第56天,重新开始刺激。评估以下参数:压痛和肿胀关节计数、医生(PGA)和患者(PtGA)总体评分、疼痛强度、疾病活动性(DAS28)、功能能力(HAQ)、血清CRP水平和EULAR反应。结果:在筛选访问42天的访问中,两个病人经历了改进ofDAS28(7.00和6.22比4.03和2.13),PGA(70 - 53和27岁和16岁),PtGA(48岁和43岁和15 - 14),温柔的关节数(26和28比4和0),关节肿胀数(24日和14日与8和2),疼痛强度(72年和87年对21和7),哈克得分(2.25和2.25和1.5和1.375)和c反应蛋白水平(23.8和5.58和13,4.61)。装置停用后,两例患者的DAS28和VAS疼痛加重。结论:迷走神经刺激治疗对甲氨蝶呤反应不足的活动性RA患者可有效减轻临床症状和炎症参数。我们的结果与其他中心的结果一致。为了更好地评估这种治疗类风湿关节炎的新方法的效果,有必要对更多的受试者进行研究。
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引用次数: 0
[AUTOIMMUNITY AND IMMUNODEFICIENCIES]. [自身免疫和免疫缺陷]。
Pub Date : 2016-01-01
Alenka Gagro

Autoimmune diseases and primary immunodeficiencies share a common pathogenesis characterizedby dysregulation of immunity. Although most autoimmune diseases show a polygenic inheritance pattern, it has beenshown that monogenic defects of various immune system components could lead to autoimmunity as well. These findingshave opened a new pathway for understanding the development of autoimmune diseases and the overlap betweenimmunodeficiency and autoimmunity. Th e mechanism of how a single gene defect leads to autoimmunity is not completelyknown. The purpose of this clinically-oriented review is to describe the incidence, clinical presentation, andpossible mechanisms of autoimmunity in patients with primary immunodeficiencies relevant to rheumatologists.

自身免疫性疾病和原发性免疫缺陷有一个共同的发病机制,其特征是免疫失调。虽然大多数自身免疫性疾病表现为多基因遗传模式,但已有研究表明,各种免疫系统成分的单基因缺陷也可能导致自身免疫。这些发现为理解自身免疫性疾病的发展以及免疫缺陷和自身免疫之间的重叠开辟了新的途径。单个基因缺陷导致自身免疫的机制尚不完全清楚。本临床综述的目的是描述与风湿病学家相关的原发性免疫缺陷患者自身免疫的发生率、临床表现和可能的机制。
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引用次数: 0
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Reumatizam
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