自身免疫性结缔组织病中逆转录病毒蛋白抗体与临床表现及RNP抗体的关系

自身免疫性疾病的病因仍不清楚。病毒,特别是近来的逆转录病毒被提出是可能的病因。有人已经证明部分自身免疫性皮肤病患者含有可和人类逆转录病毒HTLV-Ⅰ和HIV-Ⅰ反应的抗体。此外,已经证明经逆转录     

抗核抗体在结缔组织病中的临床意义

正抗核抗体(antinuclear antibody,ANA)系以细胞成分为靶抗原的自身抗体的总称[1]。在结缔组织疾病中,细胞核的核仁、核内染色质、非组蛋白以及由RNA和相关蛋白构成的各种核糖核蛋白粒子都可以成为自身免疫反应攻击的靶子,继而产生ANA。迄今已有20余种抗核内各种不同成分的ANA被相继发现[2]。其中不少抗体对疾病的诊断有高度特异性,已成为诊断某一疾病的血清标记     

免疫印迹法检测结缔组织病抗ENA抗体

目前测定抗ＥＮＡ抗体的方法，国内常应用对流免疫电泳法（ＣＩＥ），国外则多采用双向免疫扩散法（ＤＩＤ）。以上两法虽简便，但需多种标准血清，且不能探明相应抗原的结构，限制了对抗原及其抗体的深化研究。我们采用免疫印迹法（ｉ。ｍｕｎｏｂｌｏｔｔｉｎｇ，ＩＢ）与ＣＩＥ法作对比检测了皮肌炎ＩＤＭ）、进行性系统性硬化症（ＰＳＳ）、Ｓｊ６ｇｒｅｎ综合征（ＳｊＳ）、类风湿性关节炎（ＲＡ）、重叠综合征（ＯＩ。Ｓ）和混合性结缔组织病（ＭＣＴＤ）等患者的血清抗ＥＮＡ抗体，现将检测结果报道如下。一、病例和方法（一）病例：…     

81例结缔组织病的血清抗核抗体分析

结缔组织病患者具有明显的体液免疫功能异常,有自身抗体 (特别是抗核抗体 )的存在。我们分析了自 1995年以来,我院收治的系统性红斑狼疮 (SLE)、皮肌炎和多发性肌炎 (DM/PM)、系统性硬皮病 (PSS)、干燥综合症 (SS)及混合性结缔组织病 (MCTD)81例。本组病例均为活动期患者,就其抗核抗体的检测情况进行了观察总结,现报告如下。 检测方法：抗 ds- DNA抗体的测定采用胶体金标记和斑点渗滤技术。 ENA多肽抗体的测定采用免疫印迹法,试剂盒均由广州万孚生物技术有限公司提供。 结果： 63例 SLE患者中抗核抗体阳性 52例 (82.54％ )…     

RNP抗原及其抗体

U1RNP抗原是可提取性抗原组成之一，与抗U1RNP抗体反应的主要是U1RNP中的3种多肽，U1－70K，U1A和U1C，目前认为抗RNP抗体是MCTD的标志性抗体，本文将主要介绍RNP抗原的组成，生物学功能和抗原性，以及抗RNP抗体与临床的相关性。     

RNP抗体的临床意义

作者对428例病人的临床和实验室资料进行了分析研究.根据惯用的诊断标准,符合SLE者91例,PSS 130例,RA 50例,PM/DM 31例,尚有126例不符合上述4种疾病的诊断标准,这126例中有53例有循环RNP抗体(用血球凝集试验检测RNP抗体),且都有相似的临床表现,其中有42例符合MCTD诊断标准.这42例主要表现有雷诺氏     

抗U1RNP抗体阳性新生儿红斑狼疮一例

患儿女,于生后20d出现左足底红斑,几天后面部、躯干出现类似红斑,耳后较多。一般情况尚可,无发热,无腹泻。其母为第3胎第1产,前两次为人工流产,此次妊娠体检时未见异常,孕40周,剖腹产一足月女婴。患儿体重3．75b,身长5lcm,呼吸40次,心率140次,Apgar评分在5min与10min均为10分。     

结缔组织病患者的血清抗ENA抗体检测

作者用对流免疫电泳法检测了127份分别来自CTD(93份),其它皮肤病(14份)及正常人(20份)血清中的抗ENA抗体,结果Sm抗体仅见于SLE者,且其出现与病情活动与否无关.4例MCTD者RNP均阳性,3例SCLE中2例Ro/La抗体阳性.ANA阴性的9例SLE中,6例Ro抗体阳性.故认为抗ENA抗体的检测对CTD的临床诊断,分型,判断预后和指导治疗均有重要意义.     

009局限性硬皮病患者抗-U1RNP抗体的研究

抗ＵＩ核糖核蛋白 (抗 -Ｕ1ＲＮＰ)抗体可在多种自身免疫性疾病中存在 ,但存在于局限性硬皮病(ＬＳｃ)患者中未见报道。作者用免疫沉淀等方法首次发现在ＬＳｃ患者中具有抗 -Ｕ 1ＲＮＰ抗体。病例和方法 :血清标本来自 70例ＬＳｃ患者 ,30例系统性硬皮病 (ＳＳｃ)患者和 30名健康对照者。按临床类型ＬＳｃ患者分为 3组 :①泛发性硬斑病组 (ｎ=2 2 ) ;②线状硬皮病组 (ｎ =2 8) ;③硬斑病组 (ｎ =2 0 )。既有硬斑病又有线状损害则归到泛发性硬斑病组。用从ＨｅＬａ细胞中提取的Ｕ小核ＲＮＡｓ进行免疫沉淀检测所有血清标本中抗 -Ｕ 1ＲＮＰ抗…     

Anti-SL-Ki antibody in a patient with fatal connective tissue overlap disease

A patient with photosensitivity is described who presented with an overlap syndrome combining features of systemic lupus erythematosus with a mild polymyositis and scleroderma and with symptoms of the sicca syndrome. In addition, he was anti-SSA/Ro positive and had a constellation of antibodies directed to ENA including the anti-SL-Ki antibody. The condition was fatal and he died from heart failure and respiratory insufficiency.     

Nutrition and connective tissue disease

Despite an incomplete overall understanding, nutrition plays an important role in connective tissue disease. Assessment of patients with connective tissue disease for nutritional status and metabolic disturbances may significantly contribute to patient outcomes. Several studies have indicated the multifactorial role of macronutrients, micronutrients, and supplements in the setting of connective tissue disease. There is additional evidence regarding the roles of weight, obesity, and malnutrition. This contribution reviews a growing body of data regarding nutrition in the development and treatment of various connective tissue diseases, including systemic lupus erythematosus, dermatomyositis, and systemic sclerosis.     

Mixed connective tissue disease

Mixed connective tissue disease (MCTD) was first described by Gordon Sharp,¹ in 1972, as a distinct connective tissue disorder characterized by overlapping features of systemic lupus erythematosus (SLE), progressive systemic sclerosis (PSS), and dermato/polymyositis (DP/PM). This can be distinguished from other overlap syndromes of the connective tissue diseases by the presence of high titers of an antinuclear antibody with specificity for ribonuclear protein (RNP).^1,2 Initial reports described the following additional characteristics of MCTD patients: a good therapeutic response to systemic corticosteroid therapy, a very low incidence of significant renal involvement, and a better prognosis than for SLE or PSS.^1–4 Currentopinion is divided, but in general MCTD is considered to be an overlapping clinical expression in the evolution of a more classic connective tissue disease, usually PSS. The prognosis is less favorable than once thought, particularly when the MCTD is dominated by features of PSS.^5–8     

Panniculitis in connective tissue disease

Specific and nonspecific panniculitis occur in connective tissue diseases. Microscopic studies of the epidermis and dermis, immunofluorescent microscopy, and extensive serologic testing may aid in the diagnosis and classification of these unusual syndromes. Connective tissue panniculitis and focal lipoatrophy may be forms of immunoreactive panniculitis or connective tissue disease (or both) in which typical microscopic, serologic, or other events related to classic connective tissue disease have not occurred.     

Mixed connective tissue disease.

Three patients with mixed connective tissue disease (MCTD) had clinical features that included a high incidence of Raynaud phenomenon, arthritis, myositis, and swollen hands. The diagnostic laboratory test result was the presence of high titers of antibody to extractable nuclear antigen. These antibody titers are notably reduced or abolished in patients with MCTD when the tanned red blood cells that are used in the test are pretreated with ribonuclease. Speckled antinuclear antibodies were present in all patients. Patients with MCTD have a low incidence of renal disease, are responsive to treatment with prednisone, and have a good prognosis.     

Mixed connective tissue disease syndrome.

Fifteen patients with epidermal nuclear staining on direct immunofluorescence of normal skin and high titer serum antibody to ribonuclease-sensitive extractable nuclear antigen (ENA) had diffuse nonscarring and focal alopecia, abnormal pigmentation, swollen hands with sclerodactyly, and chronic cutaneous lupus erythematosus (LE) as the most common dermatologic features. Direct immunofluorescence of normal, unexposed skin revealed a particulate ('speckled') epidermal nuclear staining pattern in all 15 patients and subepidermal immunoglobulin deposits in 5. Ribonucleoprotein antibodies in high titer are associated with this characteristic type of epidermal nuclear staining. These findings provide easily detectable markers for a less aggressive subset of LE characterized by distinctive clinical and laboratory features consistent with mixed connective tissue disease.     

Undifferentiated connective tissue disease and its cutaneous manifestations

Undifferentiated connective tissue disease (UCTD, also named UCT syndrome, latent lupus or incomplete lupus) is regarded as an autoimmune disorder in which signs and symptoms are widely variable and evocative for connectivitis but not sufficiently evolved to fulfil any of the accepted classification criteria for the defined connective tissue diseases. In this paper we describe the case of a 47-year-old woman affected by UCTD according to the preliminary classification criteria supplied by Mosca et al. in 1999.