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Prof. Theodor Dürrigl, MD, PhD (10th September 1926 – 20th July 2022) 西奥多·德里格教授,医学博士,博士(1926年9月10日- 2022年7月20日)
Pub Date : 2023-10-22 DOI: 10.33004/reumatizam-69-2-7
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引用次数: 0
Clinical presentation and treatment of adult-onset Still disease – a single-centre experience 成人发病Still病的临床表现和治疗——单中心经验
Pub Date : 2023-10-22 DOI: 10.33004/reumatizam-69-2-2
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引用次数: 0
Association between cutaneous manifestations and clinical features of IgA vasculitis IgA血管炎的皮肤表现与临床特征的关系
Pub Date : 2023-05-28 DOI: 10.33004/reumatizam-69-1-2
M. Šestan, Danica Grguric, N. Kifer, Ante vidović, M. Frković, M. Jelušić
Introduction: iga vasculitis (igav) is the most common systemic vasculitis in childhood. Purpuric rash is a man-datory criterion for diagnosing igav, it is mostly localized on the lower extremities and gluteal region, although it can also appear atypically affecting the face, trunk and upper extremities. in the most severe cases, ulcerations, necrosis and bullae can be present. Objectives: to evaluate the characteristics of cutaneous manifestations in patients with igav and to examine its association with clinical features. Subjects and methods: Retrospective analysis of data from patients with igav diagnosed and treated at the Referral centre for Paediatric and adolescent Rheumatology of the ministry of health of the Republic of croatia, in the period from january 2009 to December 2021. Results: igav was diagnosed in 234 patients, 124 boys and 110 girls with the median (range) age at the time of diagnosis of 6.5 (4.5–8.2) years. all patients had a purpuric rash, and in 127 of them (54.3%) igav began with a rash. cutaneous manifestations were most often presented in the form of palpable purpura and/or petechiae (87.2%) and in all patients were localized on the lower extremities. in 103 patients (44%) purpuric rash spread further to the upper extremities, trunk and/or face. at least one skin relapse occurred in 47 patients (20.1%). The most severe cutaneous manifestations which included ulcerations and necrosis developed in 11 patients (4.7%). Patients with cutaneous manifestations spread above the waist had a more statistically significant gastrointestinal involvement compared to patients with cutaneous manifestations affecting the lower extremities and gluteal region (50.5% vs. 36.6%, p=0.033), higher incidence of iga vasculitis nephritis (igavn) (31.1% vs. 19.8%, p=0.048) and were more frequently treated with systemic glucocorticoids (68% vs. 52.7%, p=0.018) and angiotensin-converting enzyme inhibitors (14.5% vs. 5.3%, p=0.016). almost all patients with ulcerations and necrosis required treatment with systemic glucocorticoids compared to the rest (90.9% vs. 57.8%,p=0.031). Conclusion: We observed that patients with purpuric rash spread above the waist have more frequently affected gastrointestinal system and a higher incidence of igavn. The prevalence of ulcerations and necrosis in igav is less common than the standard purpuric rash and this group of patients required systemic glucocorticoid therapy.
引言:iga血管炎(igav)是儿童期最常见的系统性血管炎。Purpuric皮疹是诊断igav的一个重要标准,它主要局限于下肢和臀大区,尽管它也可能出现在面部、躯干和上肢的非典型影响。在最严重的病例中,可出现溃疡、坏死和大疱。目的:评价igav患者的皮肤表现特点,并探讨其与临床特征的关系。受试者和方法:对2009年1月至2021年12月期间在克罗地亚共和国卫生部儿科和青少年风湿病转诊中心诊断和治疗的igav患者的数据进行回顾性分析。结果:234名患者、124名男孩和110名女孩被诊断为igav,诊断时的中位(范围)年龄为6.5(4.5-8.2)岁。所有患者都有purpuric皮疹,其中127例(54.3%)igav以皮疹开始。皮肤表现最常见的是可触及的紫癜和/或瘀点(87.2%),并且所有患者都局限于下肢。103例(44%)purpuric皮疹进一步扩散到上肢、躯干和/或面部。47名患者(20.1%)至少出现一次皮肤复发。11名患者(4.7%)出现最严重的皮肤表现,包括溃疡和坏死。与影响下肢和臀大肌的皮肤表现相比,腰部以上皮肤表现的患者胃肠道受累更具统计学意义区域(50.5%对36.6%,p=0.033),iga血管炎肾炎(igavn)的发病率较高(31.1%vs.19.8%,p=0.048),并且更频繁地接受全身糖皮质激素治疗(68%vs.52.7%,p=0.018)和血管紧张素转换酶抑制剂治疗(14.5%vs.5.3%,p=0.016)。与其他患者相比,几乎所有溃疡和坏死患者都需要接受全身糖皮层治疗(90.9%vs.57.8%,p=0.031)。结论:我们观察到,扩散到腰部以上的purpuric皮疹患者更频繁地影响胃肠系统,igavn的发病率也更高。igav溃疡和坏死的发生率低于标准的purpuric皮疹,这组患者需要全身糖皮质激素治疗。
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引用次数: 0
COVID-19 COURSE AND OUTCOME IN PATIENTS WITH INFLAMMATORY RHEUMATIC DISEASES WHO ARE ON BIOLOGICAL OR TARGETED SYNTHETIC DISEASE-MODIFYING ANTIRHEUMATIC DRUGS – RESULTS FROM A SINGLE RHEUMATOLOGY CENTRE 使用生物或靶向合成抗风湿药物治疗的炎症性风湿病患者的COVID-19病程和结局——来自单一风湿病学中心的结果
Pub Date : 2023-05-28 DOI: 10.33004/reumatizam-69-1-4
Stipe Ćavar, F. Grubišić, H. S. Kavanagh, Ines Doko Vajdić, S. Grazio
The aim of this study was to investigate the course and outcome of COVID in patients with inflammatory rheumatic diseases (IRD) who are on biological disease-modifying antirheumatic drugs (bDMARDs) or targeted synthetic disease-modifying antirheumatic drugs (tsDMARDs). In this study, we used the data of patients with rheumatoid arthritis (RA), psoriatic arthritis (PsA) and axial spondyloarthritis (axSpA) who had been treated at the Department of Rheumatology, Physical and Rehabilitation Medicine of the Sestre milosrdnice University Hospital in Zagreb (Croatia) and in whose case the SARS-CoV-2 infection was proven in the period from February 2020 until the end of July 2021. In order to analyse this data, we have used the methods of descriptive statistics. Out of a total of 28 patients, 6 had a severe or critical case of COVID-19, but only one subject was hospitalized. All 6 patients were treated with bDMARDs before the onset of infection. Most of them (4/6) had moderate to high disease activity of IRD, as well as multiple comorbidities. No deaths were recorded in this cohort of patients. The results of this study suggest that the course of COVID-19 is associated with the disease activity of IRD and accompanying comorbidities, whereas the use of specific biological drugs might be associated with a more favourable outcome of the infection. Therefore, a better follow-up process and management of disease activity in patients with IRD should be implemented during the period of this pandemic, as well as the modification of specific therapy.
本研究的目的是探讨炎症性风湿病(IRD)患者使用生物减病抗风湿药物(bDMARDs)或靶向合成减病抗风湿药物(tsDMARDs)治疗后的病程和预后。在这项研究中,我们使用了在萨格勒布(克罗地亚)Sestre milosrdnice大学医院风湿病、物理和康复医学科接受治疗的类风湿性关节炎(RA)、银屑病关节炎(PsA)和轴性脊柱炎(axSpA)患者的数据,这些患者在2020年2月至2021年7月底期间被证实感染了SARS-CoV-2。为了分析这些数据,我们使用了描述性统计的方法。在总共28名患者中,有6名患者患有COVID-19重症或危重病例,但只有1名患者住院。6例患者均在感染发生前接受bdmard治疗。大多数(4/6)患者IRD疾病活动性中高,并伴有多种合并症。在这组患者中没有死亡记录。这项研究的结果表明,COVID-19的病程与IRD的疾病活动性和伴随的合并症有关,而使用特定的生物药物可能与更有利的感染结果有关。因此,在本次大流行期间,应实施更好的随访过程和对IRD患者疾病活动的管理,并修改特异性治疗方法。
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引用次数: 0
Gender differences in patients with Sjögren’s syndrome: A 10-year single centre experience 干燥综合征患者的性别差异:10年单中心经验
Pub Date : 2023-05-28 DOI: 10.33004/reumatizam-69-1-1
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引用次数: 0
Diagnosis and classification criteria of Sjögren’s syndrome 干燥综合征的诊断和分类标准
Pub Date : 2023-05-28 DOI: 10.33004/reumatizam-69-1-5
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引用次数: 0
Association of biological therapy and malignancy in inflammatory rheumatic diseases 风湿性炎性疾病的生物学治疗与恶性肿瘤的关系
Pub Date : 2023-05-28 DOI: 10.33004/reumatizam-69-1-6
Martina Jambrović, M. Cerovec, I. Padjen, B. Anić
inflammatory rheumatic diseases are chronic, progressive autoimmune diseases which affect the musculoskeletal system and other organ systems. nowadays, a large number of patients is treated with biological therapy. although biological drugs selectively affect specific molecules of the immune system, they weaken the overall immune system of the body. Therefore, the patients are more susceptible to infections and other diseases such as lymphomas, breast and skin cancers and melanomas. Chronic inflammation which occurs due to autoimmune disease is also a risk factor for malignant development. so far, studies have not proven direct correlation between biological therapy and solid or haematologic tumours. on the other hand, the increased risk for developing skin cancer in patients on tumour necrosis factor alpha inhibitors has been described. in this review paper we analysed the available medical literature on the risks for malignant disease development in patients with rheumatic diseases who are on biological disease – modifying anti-rheumatic drugs.
炎性风湿性疾病是影响肌肉骨骼系统和其他器官系统的慢性进行性自身免疫性疾病。目前,大量患者接受生物治疗。尽管生物药物选择性地影响免疫系统的特定分子,但它们会削弱身体的整体免疫系统。因此,患者更容易感染和其他疾病,如淋巴瘤、乳腺癌、皮肤癌和黑色素瘤。由自身免疫性疾病引起的慢性炎症也是恶性发展的危险因素。到目前为止,研究还没有证实生物治疗与实体瘤或血液肿瘤之间的直接相关性。另一方面,已经描述了使用肿瘤坏死因子α抑制剂的患者患皮肤癌症的风险增加。在这篇综述中,我们分析了现有的医学文献,这些文献涉及服用生物性疾病改良抗风湿药物的风湿性疾病患者发生恶性疾病的风险。
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引用次数: 0
Incidence of thromboembolic events in patients with a systemic form of vasculitis and cutaneous vasculitis 系统性血管炎和皮肤血管炎患者血栓栓塞事件的发生率
Pub Date : 2023-05-28 DOI: 10.33004/reumatizam-69-1-3
ana Šimac, Željka Kardum, Jasmin Ahić, Ana Marija Masle, K. K. stranski, V. Prus
Objectives: vasculitis is a rare disease characterized by inflammation and necrosis of blood vessels. inflammation-induced thrombosis is a hallmark of several autoimmune diseases, such as systemic lupus erythematosus (sle), rheumatoid arthritis (ra), sjögren’s syndrome (ss)
目的:血管炎是一种以血管炎症和坏死为特征的罕见疾病。炎症引起的血栓形成是一些自身免疫性疾病的标志,如系统性红斑狼疮(sle)、类风湿性关节炎(ra)、sjögren综合征(ss)。
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引用次数: 0
THERAPEUTIC APPROACH TO A PATIENT WITH ANTISYNTHETASE SYNDROME – A CASE REPORT 一例抗合成酶综合征患者的治疗方法&附1例报告
Pub Date : 2022-12-05 DOI: 10.33004/reumatizam-68-2-5
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引用次数: 0
Indolent extranodal B-NHL CD20 positive MALT lymphoma in a patient with primary Sjögren’s syndrome with a good response to rituximab treatment 原发性Sjögren综合征患者的无痛结外B-NHL CD20阳性MALT淋巴瘤,对美罗华治疗反应良好
Pub Date : 2022-12-05 DOI: 10.33004/reumatizam-68-2-3
sjögren’s syndrome (ss) is a chronic autoimmune disease in which chronic lymphocytic infiltration leads to progressive destruction of the exocrine glands and consequently causes the dry syndrome. The most commonly affected are the lacrimal and salivary glands. less often, ss presents with the symptoms of exocrine glands of other localisation, arthritis, myositis, vasculitis involving the skin and the nervous system, interstitial lung disease
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Reumatizam
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