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以肺动脉高压为主的混合性结缔组织病报告并文献复 习2014.03.14
目的:探讨混合性结缔组织病并发肺动脉高压的诊断与治疗,从而提高对该病的认识。方法:报道1例以肺动脉高压为首诊的混合性结缔组织病临床资料并复习国内相关报道。结果:国内之前报道8例,包括本例共9例,全为女性;首发主要症状是胸闷、心悸、气喘;临床表现为雷诺现象...
Maternal Mixed Connective Tissue Disease and Offspring with Chondrodysplasia Punctata2014.03.14
Objectives: To describe the case of a mother with mixed connective tissue disease (MCTD) whose male and female offspring from 2 successive pregnancies had chondrodysplasia punctata (CDP) in the absence of identifiable biochemical or genetic abnormali...
Cardiac involvement in mixed connective tissue disease: A systematic review2014.03.14
Objective: To report the clinical characteristic of cardiac disease in patients with mixed connective tissue disease (MCTD).Method: We identified published case series that reported cardiac manifestations of patients with MCTD by searching the PubMed...
Anti B-cell therapy against refractory thrombocytopenia in SLE and MCTD patients: long-term follow-up and review of the literature2014.03.14
Objective: The objective of this study was to retrospectively evaluate the clinical and immunological effects of anti-B cell treatment in patients with systemic lupus erythematosus (SLE) and mixed connective-tissue disease (MCTD) with autoimmune thro...
Mixed connective tissue disease: An overview of clinical manifestations, diagnosis and treatment2014.03.14
混合性结缔组织病:一则关于临床表现、诊断和治疗的综述
混合性结缔组织病最常见的临床表现有雷诺现象、关节痛、关节肿胀、食道功能障碍、肌肉无力和手指外观香肠状,这些连同抗核糖核蛋白(RNP)抗体存在。然而,器官受累比已经报道的第一描述更为广泛。本病可出现严重的肺、肾、心血管、胃肠道和中枢神经系统症状。预后差、死...
混合性结缔组织病的诊治2014.03.14
混合性结缔组织病发病并不少见,本病为临床上具有系统性红斑狼疮、系统性硬化症、多发性肌炎/皮肌炎及类风湿性关节炎等疾病混合表现,血清中有高滴度RNP抗体为特征。本文对混合性结缔组织病的发病机制、临床表现、诊断标 准、治疗等予以介绍。
Severe ischemia following treatment with rituximab in a patient with mixed connective tissue disease: an unusual complication2014.03.14
Rituximab is a monoclonal antibody that depletes B cells. It is increasingly used for difficult- to-treat auto-immune diseases, including systemic lupus erythematosus,1 Sjo¨ gren syndrome,2 scleroderma,3 Raynaud phenomenon,4 and mixed connective...
Neuropsychological assessment in mixed connective tissue disease: comparison with systemic lupus erythematosus2014.03.14
Objective: The aims of the study were to assess cognitive functions (CF) in patients with mixed connective tissue disease (MCTD) and to compare MCTD patients with systemic lupus erythematosus patients with and without neuropsychiatric manifestations ...
Epstein-Barr Virus-Negative, CD5-Positive Diffuse Large B-Cell Lymphoma Developing after Treatment with Oral Tacrolimus for Mixed Connective Tissue Disease : A Case Report and Review of the Literature2014.03.14
A 69-year-old woman, who had been diagnosed as having Sjögren's syndrome at 37 years old and mixed connective tissue disease at 42 years old, was under treatment with oral prednisolone. In 2009, she was diagnosed as having active systemic lu...
Efficacy of bosentan in the treatment of a patient with mixed connective tissue disease complicated by pulmonary arterial hypertension2014.03.14
波生坦治疗混合性结缔组织病并发肺动脉高压症患者的疗效
本研究旨在探讨一位患有抗磷脂综合征相关的混合性结缔组织病(MCTD)的年轻女性,使用波生坦治疗严重肺动脉高压的疗效。一名27岁的女性突然呼吸困难。在此之前,她从没有经历过任何呼吸困难的现象,她被确诊为患有MCTD。实验室测试表明抗核抗体、抗RNP抗体、抗心磷脂抗体...
Fluctuation of Anti-Endothelial Cell Antibody Titers in Mixed Connective Tissue Disease2014.03.14
Background: Antibodies directed against endothelial cell surface antigens have been described in many disorders andhave been associated with disease activity. Since the most prominent histopathologic feature in mixed connective tissue disease (MCTD) ...
Dysbalance of angiogenic and angiostatic mediators in patients with mixed connective tissue disease2014.03.14
Objective Vascular disease is common in mixed connective tissue disease (MCTD). The aim of the present study was to investigate, whether dysbalance of angiogenic and angiostatic factors occurs in MCTD.Methods In all, 38 patients with MCTD, and 40 pat...
Distinct phenotypes in mixed connective tissue disease: subgroups and survival2014.03.14
The aim of the present study was to assess the autoantibody profile, dominant clinical symptoms and cluster characteristics of different Mixed connective tissue disease (MCTD phenotypes. Two-hundred-and-one patients with MCTD were followed-up longitu...
Differential immunoglobulin class-mediated responses to components of the U1 small nuclear ribonucleoprotein particle in systemic lupus erythematosus and mixed connective tissue disease2014.03.14
The objective of this paper is to determine whether patients with systemic lupus erythematosus (SLE) and mixed connective tissue disease (MCTD) possess differential IgM- and IgG-specific reactivity against peptides from theU1small nuclear ribonucleop...
Prevalence and severity of interstitial lung disease in mixed connective tissue disease: a nationwide, cross-sectional study2014.03.14
混合性结缔组织病的间质性肺病的患病率和严重性:一个全国性、横断面的研究
研究背景 混合性结缔组织病(MCTD)是一种免疫介导的、原因不明的全身性疾病。Objective Toassess the prevalence, pattern and severity of interstitial lung disease (ILD)in a crosssectional study of the nationwide, Norwegian MCTD cohort. 研究目的挪威MCTD队列...
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