超声在结缔组织病中的应用

风湿性疾病是一组以内科治疗为主的肌肉骨骼系统疾病,包括弥漫性结缔组织病及各种病因引起的关节和关节周围软组织,包括肌肉、肌腱、韧带等疾病.其中结缔组织病是风湿性疾病中的一大类,具有以下特点:①慢性病程;②以血管和结缔组织慢性炎症的病理改变为基础;③病变累及多个系统,包括肌肉、骨骼系统;④同一疾病,在不同患者的临床谱和预后差异甚大;⑤对糖皮质激素治疗有一定反应.其慢性病程和晚期累及多个器官损害造成诊断困难,只有早期诊断,并进行合理治疗,密切监测治疗效果,才能使患者得到良好预后. 目前超声在结缔组织病中的应用越来越广泛,对疾病的早期诊断、疗效判断及预后提供帮助.灰阶超声(GS)可以清晰地显示增厚的滑膜、关节腔积液、肌腱韧带炎症及表浅的骨侵蚀.能量多普勒(PD)可以显示血流信号,反映血管增生和急性期血管扩张的情况[1].     

来氟米特在常见风湿性疾病治疗中的进展

风湿性疾病是一组以内科治疗为主的肌肉骨骼系统疾病，包括弥漫性结缔组织病及各种病因引起的关节和关节周围软组织（肌肉、肌腱、韧带等）的疾病。常见的风湿性疾病有系统性红斑狼疮、类风湿关节炎、干燥综合征、多发性肌炎等。风湿性疾病是一类难治性疾病，传统运用非甾体抗炎药（NSAIDs）、糖皮质激素、慢性作用抗风湿药（DMARD）对风湿性疾病进行病情控制和改善症状，但副作用较大。     

重视弥漫性结缔组织病的肝损伤

弥漫性结缔组织病(CTD)是一种不仅累及骨、关节及筋膜、滑囊、肌腱等周围软组织,还可影响到全身各个系统、器官,且有多种自身抗体产生的自身免疫病.     

结缔组织疾病并发肺动脉高压的研究进展

结缔组织疾病是一类常常累及全身多个器官和系统的自身免疫性疾病。呼吸系统因具有丰富的胶原和血管等结缔组织常常被累及,可出现肺间质病变、肺动脉高压（pulmonary artery hypertension,PAH）、肺栓塞等,其中PAH是结缔组织疾病的严重并发症之一,其主要特征是肺动脉阻力进行性升高,最终导致患者右心衰竭而死亡。文献报道PAH患者平均自然存活时间短,预后差,现有治疗药物和治疗手段无法改变其自然病程,病因和发病机制研究一直是该领域的热点和难点。现将风湿性疾病并发PAH的研究进展综述如下。     

骨关节炎影像学研究进展

骨关节炎(osteoarthritis,OA)是最常见的一种关节疾病,以关节软骨损害为主,还可累及软骨下骨、滑膜、韧带、肌腱、关节囊等,甚至影响到整个关节,导致关节软骨发生退变、纤维化、断裂、溃疡等病变。0A主要影响老年人,是老年人肢体残疾和生活水平下降的主要原因。随着世界人口老龄化和肥胖率的增加,0A的患病率越来越高。     

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结缔组织病是风湿性疾病中的一大类,属非器官特异性的自身免疫疾病,可累及单个或多个系统出现不同的临床表现。其机制目前尚不明确,多数研究表明与自身抗体及T细胞介导有关,以血管和结缔组织的慢性炎症为病理基础。结缔组织病可累及大脑、脑膜、脊髓、颅神经及神经末梢等神经系统,有多种临床表现。多数结缔组织病经皮质类固醇及免疫抑制剂治疗后有效,但症状多不能完全缓解,新的治疗方案仍有待进一步研究。     

骨质疏松与强直性脊柱炎

AS 是一种与 HLA-B27相关的炎症性的脊柱及关节病变,主要侵犯中轴关节,几乎全部累及骶髂关节和不同程度累及外周关节,炎症可侵犯滑膜关节、关节软骨以及肌腱、韧带和韧带附着点,常引起纤维性和骨性强直[1]。骨质疏松（osteoporosis）作为 AS 的一个常见并发症,在疾病发病早期即可出现,如不予以重视,晚期即可出现骨折、关节畸形等严重并发症,其中脊柱骨折是 AS 晚期少见但却不可忽视的导致死亡的主要原因之一[2]。     

硬皮病合并肝结节病1例报告

<正>硬皮病是一种以皮肤局限性或弥漫性增厚和纤维化为特征的全身性结缔组织病,除皮肤外还可累及内脏。结节病是一种以非干酪肉芽肿为特征的系统性疾病,感染、药物、自身免疫等都可能是本病病因,肺部和淋巴结最常累及,肝脏是第三大常见的累及器官,临床表现缺乏特异性~([1-2])。两种疾病很少在同一患者身上被发现,易被漏诊、误诊。现分享1例硬皮病合并肝结节病患者的诊治过程,以加深临床医生对这两种疾病的认识。     

原发性干燥综合征和肾脏损害

干燥综合征(sjogren syndrome,SS)是一种影响外分泌腺的慢性炎症性、自身免疫性结缔组织疾病,临床上表现为干燥性角-结膜炎、口腔干燥.SS可分为原发性和继发性,在不伴有其他相关的系统性自身免疫性疾病时,称之为原发性干燥综合征(primary sjogren syndrome,pSS).pSS是一个系统性疾病,可以累及肺、肾脏、皮肤、血液、神经、淋巴等多个器官和系统,其中肾脏是最主要受累的器官之一[1].现对pSS的诊断标准和肾脏损害作一综述.     

关注结缔组织病相关间质性肺疾病

结缔组织病（connective tissues disease,CTD）是风湿性疾病中的一大类,常累及全身多个系统,表现为慢性炎症性自身免疫病,当侵犯呼吸系统时,可出现间质性肺疾病（interstitial lung disease,ILD）、胸膜炎和肺动脉高压等,见表1。CTD与ILD同时存在时,常称为结缔组织病相关间质性肺     

Biomechanics and aging

Starting from the physical basics of the biomechanics of connective tissues, organ structures and the corresponding age-related changes of skin, tendons, ligaments, cartilage, bones, blood vessels, and lungs are described. Finally, exogenous factors such as nutrition, hormones, immobilization, activity and adaptive mechanisms and their influence on the biomechanical properties of connective tissue are represented.     

Fibrodysplasia ossificants progressiva.

Two cases of fibrodysplasia ossificans progressiva are reported. Both patients were females and had suffered from the disease since birth. The characteristic anomalies of great toes and thumbs associated with multiple ectopic ossifications of the connective tissue are described. Fasciae, tendons, ligaments, and joint capsules of the proximal parts of the extremities and the dorsal aspect of the trunk were involved. The muscle atrophy was probably a secondary phenomenon.     

Detection of Luse bodies, spiralled collagen, dysplastic collagen, and intracellular collagen in rheumatoid connective tissues: an electron microscopic study.

BACKGROUND--Rheumatoid arthritis is a chronic inflammatory disease leading to alterations of the extracellular matrix in tendons, ligaments, and cartilage. The structural changes of the collagenous systems in rheumatoid connective tissues are largely unknown, however. METHODS--Thirty four samples of menisci, 36 cruciate ligaments, and four tendons were taken during joint surgery in patients with rheumatoid arthritis. Eighteen menisci, 35 ligaments, and 30 tendons obtained at necropsy served as a control group. The extracellular matrix in the two groups was analysed by the combined use of transmission and scanning electron microscopy, immunohistochemistry with monoclonal antibodies recognising collagen types IV and VI, and ultramorphometry. RESULTS--Normal tendons and ligaments predominantly showed a unidirectional fibril arrangement. Whereas type IV collagen showed a positive staining pattern along all basement membranes, type VI collagen formed fine, filaments aligned in parallel. In patients with rheumatoid arthritis a significant reduction of the mean diameter of the collagen fibrils was found owing to the presence of thin collagenous fibrils 20-60 nm in diameter. Most of these fibrils showed considerable changes in their arrangement with irregular courses (so-called interfibrillar dysplastic collagen). Up to 410 nm thick frayed fibrils with irregular outlines (spiralled collagen) and intracellular collagen forms were found in rheumatoid tissues. In addition, atypical thick collagenous structures with 41 nm periodicity (Luse bodies) were detected in the matrix. The upregulation of type IV collagen in rheumatoid arthritis was associated with an increase in the vascular density. The expression of type VI collagen was upregulated in fibrotic zones. CONCLUSIONS--The dramatic ultrastructural collagen changes lead to a structural and functional insufficiency of the extracellular matrix in rheumatoid connective tissues. The results suggest that collagen alterations may contribute to the development of tendon and ligament ruptures in rheumatoid arthritis.     

Combined high-resolution magnetic resonance imaging and histological examination to explore the role of ligaments and tendons in the phenotypic expression of early hand osteoarthritis

BACKGROUND: The pathogenesis of the early stages of hand osteoarthritis is poorly understood, but recent high-resolution magnetic resonance imaging (hrMRI) studies suggest that the joint ligaments have a major role in the phenotypic expression of the disease. OBJECTIVE: To combine hrMRI and cadaveric histological studies to better understand the mechanisms of damage, and especially the role of joint ligaments and tendons in disease expression. METHODS: hrMRI was carried out in the distal interphalangeal (DIP) and proximal interphalangeal (PIP) joints in 20 patients with osteoarthritis, with a disease duration < or = 12 months. Histological sections of the DIP and PIP joints were obtained from three dissecting-room specimens for comparative analysis. RESULTS: The collateral ligaments influenced the location of both hrMRI-determined bone oedema and bone erosion in early osteoarthritis. These changes were best understood in relation to the enthesis organ concept, whereby the interaction between ligament fibrocartilages leads to bone disease. Normal ligaments were commonly associated with microdamage at insertions corresponding to ligament thickening noted in early osteoarthritis. The ligaments also influenced the location of node formation in early osteoarthritis. The DIP extensor tendon insertions were associated with the development of a neoarticular surface. CONCLUSIONS: Small-joint collateral ligaments and tendons have a central role in the early stages of hand osteoarthritis, and determine the early expression of both the soft tissue and bony changes in disease.     

Overlapping syndromes, undifferentiated connective tissue disease, and other fibrosing conditions.

Many patients presenting with symptoms suggestive of a connective tissue disease do not fulfill criteria for a specific connective tissue disease at initial presentation. Some of these patients with undifferentiated connective tissue disease eventually develop a specific connective tissue disease. Recently, a large cohort of 213 patients from different centers with undifferentiated connective tissue disease of early onset were enrolled in a prospective protocoled study. Baseline characteristics, including antinuclear antibody profiles, were reported. The aim of this study was to define predictors for the development of specific organ-system involvement and connective tissue diseases in early undifferentiated connective tissue disease. Many patients show overlapping features of two or more connective tissue diseases. The presence of autoantibodies to U1-ribonucleoproteins has been associated with a particular overlap syndrome, mixed connective tissue disease. Most anti-U1-ribonucleoprotein-positive patients with undifferentiated connective tissue disease at presentation appear to develop mixed connective tissue disease over the course of disease. Although levels of anti-U1-ribonucleoprotein do not seem to be related to disease activity, this association suggests a pathogenetic relationship between anti-U1-ribonucleoprotein and mixed connective tissue disease. Genetic studies have shown that patients with antibodies against the 70-kD component of U1-ribonucleoprotein share a common epitope within the groove of their DR molecules at antigen-binding region, pointing to a particular antigen involved in the induction of these antibodies that may be relevant in the etiopathogenesis of associated disease.     

Connective tissue diseases and the liver

Connective tissue diseases such as systemic lupus erythematosus (SLE), rheumatoid arthritis, Sj?gren's syndrome, and scleroderma are systemic disorders that may have an autoimmune basis. The system manifestations vary, and there is frequent overlap among the syndromes. Liver involvement in patients with connective tissue diseases has been well documented but is generally considered rare. Although advanced liver disease with cirrhosis and liver failure is rare in patients with connective tissue diseases, clinical and biochemical evidence of associated liver abnormalities is common. Previous treatment with potentially hepatotoxic drugs or coincident viral hepatitis has usually been implicated as the main causes of liver disease in patients with connective tissue diseases. However, even after careful exclusion of these etiologies, the question remains whether to classify the patient as having a primary liver disease with associated autoimmune, clinical, and laboratory features or as having liver disease as a manifestation of generalized connective tissue disease. The main example of this pathogenetic dilemma is autoimmune hepatitis and SLE-associated hepatitis, which have been regarded as two different entities, although they have features in common of autoimmune syndromes. Several clinical and histopathologic features have been used to discriminate autoimmune hepatitis from SLE, a relevant diagnostic exercise because complications and therapy are quite different. Although hepatic steatosis and abnormal results on biochemical liver function tests are the most common hepatic abnormalities associated with connective tissue diseases, other less frequent abnormalities have been noted, such as nodular regenerative hyperplasia, portal vein obliteration and portal hypertension, features of primary biliary cirrhosis, and rarely portal fibrosis with abnormal lobular architecture. Vascular disorders of the liver also have been described, such as Budd-Chiari syndrome. Histologic assessment may reveal a variety of subclinical liver diseases. The aim of this contribution is to review the current published data regarding liver involvement in connective tissue diseases.     

The specificity of capillaroscopic pattern in connective autoimmune diseases. A comparison with microvascular changes in diseases of social importance: arterial hypertension and diabetes mellitus

Capillaroscopy is a method with substantial value for diagnosis and differentiation of primary and secondary Raynaud’s phenomenon in rheumatic diseases. The most specific finding is in systemic sclerosis—the so-called “scleroderma pattern.” which is characterized by the presence of dilated capillaries, hemorrhages, avascular areas, and neoangiogenesis. Similar changes are found in patients with dermatomyositis, overlap syndromes, and others and are termed “scleroderma-like pattern.” For the development of these patterns, the most specific finding in the early phase is appearance of dilated capillaries. Capillaroscopic changes in connective autoimmune diseases are specific and differ significantly from those of that can be found in other diseases. Diseases of social importance such as diabetes mellitus and arterial hypertension often present as comorbidity in patients with rheumatic diseases. In diabetes mellitus, the capillaroscopic examination does not show dilated capillaries until the advanced stages of the disease. In the late stages of connective tissue disease, a loss of capillaries is typical. In addition, in diabetes mellitus, the diabetic stiff-hand syndrome and sclerodactyly are common complications, which have to be differentiated from similar signs in rheumatic diseases, and capillaroscopic examination appears to be useful in these situations. In arterial hypertension, a reduced capillary density in different body regions has been observed in patients with established disease as well as in preclinical stages. Analogous phenomenon of reduction in the nail-fold area has also been observed in a group of patients with essential hypertension, none of whom previously received hypertensive drugs.     

The specificity of capillaroscopic pattern in connective autoimmune diseases. A comparison with microvascular changes in diseases of social importance: arterial hypertension and diabetes mellitus

Abstract

Capillaroscopy is a method with substantial value for diagnosis and differentiation of primary and secondary Raynaud’s phenomenon in rheumatic diseases. The most specific finding is in systemic sclerosis—the so-called “scleroderma pattern.” which is characterized by the presence of dilated capillaries, hemorrhages, avascular areas, and neoangiogenesis. Similar changes are found in patients with dermatomyositis, overlap syndromes, and others and are termed “scleroderma-like pattern.” For the development of these patterns, the most specific finding in the early phase is appearance of dilated capillaries. Capillaroscopic changes in connective autoimmune diseases are specific and differ significantly from those of that can be found in other diseases. Diseases of social importance such as diabetes mellitus and arterial hypertension often present as comorbidity in patients with rheumatic diseases. In diabetes mellitus, the capillaroscopic examination does not show dilated capillaries until the advanced stages of the disease. In the late stages of connective tissue disease, a loss of capillaries is typical. In addition, in diabetes mellitus, the diabetic stiff-hand syndrome and sclerodactyly are common complications, which have to be differentiated from similar signs in rheumatic diseases, and capillaroscopic examination appears to be useful in these situations. In arterial hypertension, a reduced capillary density in different body regions has been observed in patients with established disease as well as in preclinical stages. Analogous phenomenon of reduction in the nail-fold area has also been observed in a group of patients with essential hypertension, none of whom previously received hypertensive drugs.     

胃肠外疾病患者幽门螺杆菌感染分析

目的检测幽门螺杆菌（Helicobacterpylori，H．pylori）在胃肠外疾病患者中的感染率及其分布特征，探讨其与胃肠外疾病发病的相关性，以期在疾病预防及临床治疗中发挥指导作用。方法对1530例特发性血小板减少性紫癜、糖尿病、结缔组织病、肝硬化、过敏性紫癜及其他胃肠外疾病患者进行14C-尿素呼气试验检测。结果1530例胃肠外疾病患者中有1140例14C-UBT结果阳性，感染率74．5％。特发性血小板减少性紫癜、糖尿病、结缔组织病、过敏性紫癜组H．pylori感染率均高于健康人（P〈0．05），肝硬化患者H．pylori感染率与健康人无明显差异。特发性血小板减少性紫癜、糖尿病、结缔组织病等合并慢性胃炎或消化性溃疡的病例H．pylori感染率较单纯慢性胃炎或消化性溃疡患者高（P〈0．05），而肝硬化及过敏性紫癜组则无明显差异。在特发性血小板减少性紫癜等胃肠外疾病患者中联合抗日．pylori治疗疗效优于单纯治疗原发病。结论H．pylori感染可能在消化道以外疾病的发生发展过程中起重要作用，但是否为相关胃肠外疾病发病的独立危险因素需要进一步证实。